_id
stringlengths 7
16
| description
stringlengths 55
95.2k
|
|---|---|
pmc-6163764-2
|
Case 2: Theophylline
The patient is a 22-year-old female who was admitted to your hospital for an asthma exacerbation. She reports to you that she has not had her prescriptions refilled and ran out of her medications 1 week ago. Pharmacy consult included assessing adherence/compliance, determining dosage for bolus and maintenance theophylline, and discussion with patient about plans for discharge and reducing exacerbations.
|
pmc-6163764-3
|
Case 3: Tobramycin
The patient is a 24-year old female who resides in a long-term care facility secondary to quadriplegia. She was hospitalized for a urinary tract infection and the urine culture grew Pseudomonas aeruginosa. Home meds included oxybutynin, docusate sodium, and, baclofen with pharmacy to dose tobramycin.
|
pmc-6163764-4
|
Case 4: Phenytoin
The patient is a 30-year-old male who is admitted for a closed head trauma and has developed generalized tonic-clonic seizures. He was initially placed on lamotrigine but has not responded well to that. He was started on phenytoin a couple of days ago, but his seizure frequency increased on the second day of therapy. There are lab profiles and a consult for pharmacy to dose phenytoin.
|
pmc-6163764-5
|
Case 5: Vancomycin + gentamicin (Spring 2017)
The patient is a 10-year-old female admitted for osteomyelitis. Consult for pharmacy to dose vancomycin and gentamicin. Pharmacy and nursing collaboration with family to plan for discharge and monitor long-term side effects.
|
pmc-6163764-6
|
Case 6: Lidocaine (Spring 2018)
The patient is a 70-year-old female who resides at a local long-term care facility and is admitted for ventricular tachycardia. Consult for pharmacy to dose lidocaine to a steady-state concentration of 2 mg/L. The patient also had suspected C. difficile infection.
Pharmacy students then entered the simulated healthcare environment and communicated with nurses on duty. Each patient case has a team comprised of nursing and pharmacy students. They gathered the necessary information on patient status from the nursing students as well as the chart, and then conferred to calculate empiric or adjusted doses of the aforementioned medications for the patient. Once the dose was calculated, the pharmacy students communicated the planned new dosing regimen (including administration and monitoring parameters) to the nursing students using SBAR. Necessary collaborations with additional health professionals (MD, NP, or PA) were noted. Nursing and pharmacy students both participated in the debrief session including collaborative “rounds” where each discipline identified their top priority problems related to the patient case identified during the simulation. They discussed roles and responsibilities, patient-centered values, communication processes, and criticality of teamwork. Complexity in the simulation included error detection, verification of patient data, appreciation of changing status, and dosage parameters associated with age, body weight, and organ function. Both the simulation and debrief session were facilitated by nursing and pharmacy faculty, providing assistance as needed on content and the collaborative process. In the debrief, any within-profession jargon was addressed and clarified. Lack of actual patients and the absence of a physician in the consultation limited communication, however, and form the basis for our future plans.
In addition to basic case components, several cases had embedded errors or risk that students were expected to identify. For example, nursing students noted to pharmacy that potassium was still being administered even though potassium levels had reached normal range. In another example a mistake was detected when Zosyn (piperacillin/tazobactam) was substituted for Zofran (ondansetron) in the medications available for administration, even though the patient had a penicillin allergy. These safety “near misses” further strengthened the values, teamwork, and communication needed to correct medication errors by both nursing and pharmacy professions.
|
pmc-6163809-1
|
A 35-year-old woman with a history of having lived near a pigeon farm, with erosive esophagitis, anorexia nervosa, malnutrition (IMC = 17.08 Kg/m2) (reference IMC = 18.5–24.99 Kg/m2) and a family history of rheumatoid arthritis, was admitted to our hospital complaining of one month of progressive neurological symptoms: holocranial headache, vomiting, blurred vision, bradypsychia, vertigo, aphasia, gait instability and right-sided paresthesia and weakness. During the admission, the patient was somnolent, febrile and presented a generalized tonic-clonic seizure. Physical examination showed deep tendon reflex preserved, isoreactive pupils, bilateral horizontal nystagmus, severe right-sided paresis, bilateral ataxia with right-sided predominance and meningeal stiffness.
A lumbar puncture (LP) showed an elevated open pressure at 35 cm H2O (reference range, <20 cm). Cerebrospinal fluid (CSF) analysis revealed 271/mm3 cells (reference range, 0 to 10/mm3), with 95% mononuclear cell, which were predominantly lymphocytes. The glucose level was 7 mg/dL (reference range, 40 to 70 mg/dL) and protein level was 168 mg/dL (reference range, 15 to 45 mg/dL). Polymerase chain reaction (PCR) for Herpes simplex, Epstein barr, Varicella, Enterovirus, Tuberculosis and atypical Mycobacteria were negative. India ink preparation was positive and cryptococcal antigen showed positive results at 1/100. In order to rule out a CNS lymphoma as a cause of predominant lymphocytes seen in the CSF, we performed flow cytometry which did not show clonality. Treatment was initiated with liposomal amphotericin B 0.63 mg/kg/day, flucytosine 100 mg/kg levetiracetam 500 mg bid and medprednisone 1 mg/kg/day. Five LPs evacuations were made due to neurological deterioration. Mycological culture was positive for Cryptococcus neoformans variant.
An extensive analysis to rule out immunocompromised status was performed. We aimed to discard hepatic insufficiency, chronic renal disease, immunodeficiency associated with pregnancy, IgA deficiency, HIV, idiopathic deficiency of CD4, autoimmune rheumatic diseases, lymphoma and other neoplasias. All the results were normal. Cancer screening was done with a pan-tomographic exam (Hospital Italiano, Buenos Aires, Argentina) and serologic exams (Hospital Italiano, Buenos Aires, Argentina): Alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA) and CA-125, 19.9, 15.3 and 6.2, which also were negative.
Magnetic resonance imaging (MRI, Hospital Italiano, Buenos Aires, Argentina) showed hydrocephalus, bilateral cerebellar lesions with increased signal in T2-weighted and fluid attenuation inversion recovery (FLAIR) sequences. Also, we observed a focal lesion on the right-side gyrus of the cingulum in relation to the peak of the corpus callosum (). Cerebellar lesions showed an evolution in time of ischemic vascular type, which could correspond to vasculitis.
Digital subtraction angiography (Hospital Italiano, Buenos Aires, Argentina) was performed and showed changes in the caliber of the vessels of the right antero-inferior cerebellar artery as well as the tonsillar branch of the right postero-inferior cerebellar artery (). Similar findings were seen in the right parietal region in cortical branches. This procedure confirmed CNS vasculitis.
The patient started with diplopia and important visual deficit. Funduscopic exam revealed both pink papillae with net borders. Visual evoked potential showed prolonged latency of the p100 wave. It was interpreted as bilateral optic neuropathy and intracranial hypertension secondary to cryptococcal infection. After 30 days of treatment with amphotericin B and flucytosine, it was changed for fluconazole 400 mg for 10 weeks more, with good clinical response.
After a one-year follow-up, the patient presented an expressive improvement of laboratory and the physical examination showed mild ataxia, tinnitus and mild right hypoacusia. However, the patient presented diminished bilateral visual acuity and hydrocephalus. Vestibular myogenic evoked potentials showed involvement of the vestibular-spinal pathway with right-sided predominance. The patient is now on vestibular rehabilitation and is being evaluated for a cochlear implant.
|
pmc-6164009-1
|
A 33-year-old man presenting with multiple red-blue to black papules on his right hip and penis was referred to our clinic. Physical examination revealed multiple warty, keratotic, red-blue to black papules with a diameter of 2–5 mm on the areas mentioned above ().
HH-RCM (VivaScope 3000, Caliber, United States, distributed in Europe by Mavig, Munich, Germany) showed an acanthotic epidermis and hypo-reflective oval areas in the dermis separated by fine septa with hyper- and medium-reflective cells floating inside. These findings were highly suggestive for dilated vascular spaces containing blood cells and supported the diagnose of multiple AKs ().
Slit-lamp bio-microscopy examination of the cornea showed whorl-like lines in the inferior cornea of both eyes. With a suspicion of FD, HH-RCM examination was performed after local anesthesia in order to find a corneal overload. RCM revealed the presence of intracellular hyper-reflective inclusions in most of the epithelial cells (), which can possibly be related to deposition and accumulation of glycosphingolipids.
The demonstration of deficient α-Gal A enzyme activity in white blood cells confirmed the diagnosis of FD and the patient started the enzyme replacement therapy (Agalsidase beta).
|
pmc-6164050-1
|
A two-year-old, intact female Scottish Terrier dog presented with erosive and ulcerative lesions affecting the nasal planum (). These lesions were neither painful nor pruritic and had been present since the dog was four months old. There was no familial history of a similar condition affecting the parents or the littermates.
The dog had initially been treated by the referring veterinarian with short courses of amoxicillin-clavulanic acid (Synulox; Zoetis, Rome, Italy) 25 mg/kg orally twice daily combined with prednisone (Deltacortene; Bruno Farmaceutici, Rome, Italy) 0.5 mg/kg orally once daily, leading to a temporary improvement, followed by progressive worsening of the condition. Pseudomonas aeruginosa, which was sensitive to gentamycin and quinolones, was isolated from the nasal tissues by a bacterial culture performed by the referring veterinarian when the dog was nine months old. Marbofloxacin (Aristos; ATI, Ozzano nell’Emilia, Italy) 2 mg/kg orally once daily was administered for one month without improvement. The referring veterinarian then administered methylprednisolone acetate (DepoMedrol Vet; Zoetis, Rome, Italy) 1 mg/kg intramuscularly, leading to clinical improvement of the lesions and abnormal scarring and stenosis of the nostrils. Three weeks later, erosions and ulcerations were progressively worsening, and the dog was referred for dermatological consultation. The dog was regularly vaccinated, dewormed, on heartworm prevention, and fed a commercial dry food. A fipronil-S-methoprene spot-on product (Frontline Combo; Merial, Milano, Italy) was applied monthly for flea and tick prevention. The patient had no history of previous diseases. On general examination, the dog appeared to be in good health. The rectal temperature was normal and respiratory and heart rates were within normal limits. Lymph nodes were normal in size. On dermatological examination, wide and deep ulcers were apparent, with complete destruction of the nasal planum, philtrum, and nostrils as well as the central part of the upper lips. The ulcerative process also affected the gum, in correspondence with the upper central incisors. These lesions were neither painful nor pruritic, and the dog could eat normally (). Differential diagnoses considered for this presentation included nasal planum vasculopathy of the Scottish Terrier dog, dermal arteritis of the nasal philtrum, discoid lupus erythematosus, pemphigus complex, squamous cell carcinoma, and leishmaniosis. The latter was unlikely because the dog lived in a nonendemic area in Northern Italy and Immunofluorescence Antibody Test titer was negative. Cytological examination of cutaneous lesions revealed sparse cocci, rare neutrophils, and many erythrocytes and was therefore considered inconclusive. Biopsy specimens were collected by the referring veterinarian under general anesthesia with a 5 mm biopsy punch from erosions and ulcers. Histological examination of haematoxylin and eosin-stained sections revealed an irregularly hyperplastic epidermis, erosions, ulcerations, and granulation tissue, and it was not diagnostic. Even if the primary pathognomonic lesions were not observed, the clinical presentation and response to therapy were consistent with the diagnosis of a dermatosis previously described as vasculopathy of the nasal planum of the Scottish Terrier dog. The dog was treated with oral prednisolone, 1 mg/kg once daily (Vetsolone; Bayer, Milano, Italy) and oral ciclosporin, 5 mg/kg once daily (Atoplus; Elanco, Sesto Fiorentino, Italy) for three weeks. Prednisolone was slowly tapered and discontinued after 20 weeks of therapy, while ciclosporin was continued at the same dosage. Two stents were placed in the nostrils in order to prevent abnormal scarring and subsequent stenosis (). Stents consisted of two sections of silicone drainage tubes measuring 1.5 cm in length and 0.4 cm diameter. These were inserted in the nares rostrally and fixed to the dorsolateral walls of the nasal cavities with a monofilament EP 3.5 suture material composed of polyglycolide-poly (e-caprolactone) copolymer (Monofil; Assut Europe, Rome, Italy) under general anesthesia. Stents were well tolerated by the dog and were removed and replaced twice, every two months, due to dissolution of the suture material. Stents were definitely removed after six months from the first application.
During the treatment, no adverse effects were observed except for polyuria and mild polydipsia. Haematological and biochemical profiles were performed once monthly. No relevant haematologic abnormalities were recorded. Biochemistry panel showed moderate elevation of alkaline phosphatase (ALP) consistent with glucocorticoid administration. All these abnormalities resolved when prednisolone was discontinued. During the first month of therapy, the dog’s condition showed improvement at the weekly rechecks. Ulcers, although still present, markedly decreased in size and depth. Erosive and ulcerative lesions completely regressed within five months after stents removal (). There was complete re-epithelialization of the muzzle, although with severe scarring, and regrowth of hair at the periphery of the affected area. The nasal planum and the central part of the upper lip were absent, and two remnants of the nares were present; however, the dog could breathe and eat normally. More than one and a half years after the start of the medications, the dog is still on treatment with 5 mg/kg ciclosporin orally once daily, breathes and eats normally, and the quality of life is excellent.
|
pmc-6164094-1
|
Susan is a 51-year-old woman. As an adolescent, she developed scoliosis. Working as a hairstylist when she was 18 or 19, she began to have severe pain in her hands, making her work very difficult. She had carpal tunnel surgery on her right hand which provided temporary relief. Her symptoms slowly returned and she continued to have intense pain and difficulty with her daily activities. Seeking relief from her symptoms, she received treatment from chiropractors and Chinese medicine practitioners with no significant benefit. She also tried yoga and swimming but depending on the yoga movements or swimming strokes, she began to have more pain.
When she was in her early forties, she began to have weakness in the lower half of her body. Her legs would frequently give out on the stairs, causing her to fall. Control of her bladder and bowel function also began to deteriorate. She had an MRI, and based on the results, she was scheduled for emergency surgery to stabilize her cervical spine and relieve spinal cord compression. She was informed that it would be an outpatient surgery and she would be home the same day.
In May 2011, Susan had a 6-level cervical spine fusion to stabilize her spine and relieve pressure on her spinal cord. When she woke up, she realized something was wrong. The entire right side of her body was paralyzed. What was supposed to be an outpatient surgery with a return home the same day ended up being an 8-week hospital and rehabilitation stay to learn how to walk again.
In August 2012, a second surgery was performed with the goal of pain relief. However, she reported her pain was significantly worse afterwards. She would receive weekly injections for pain relief that she reports would only marginally decrease her pain for about one week. She was also taking high doses of medication for nerve pain relief which she also reports provided marginal benefit. She had areas of heightened sensitivity on her legs where a bed sheet or even a gentle breeze would cause intense pain. Exposure to hot water would feel cold, and cold water would feel hot. She was unable to walk more than one block and remained in bed for over ten hours per day. She suffered extensive personal and professional quality of life losses at this time.
Looking for relief, she tried medical yoga and received temporary relief. She also practiced traditional Tai Chi which provided minimal to no relief. She continued to have intense pain which had a significant impact on her quality of life and ability to perform daily tasks, including walking. In 2014, she tried a Qigong class and reported feeling that there was something very different about this class. Almost immediately, she felt a strong sense of relaxation that she had reportedly not found in many years. She was unable to attend many classes, so she purchased a video of the movements. Over the next year, she began to practice the exercises consistently until she could attend formal classes.
Susan credits the practice of qigong with saving her life. She says it gave her a reason to get out of bed and socialize in the very early days of her practice. She is now able to walk with no limitations and her pain has improved by approximately 90%. Within three months of beginning qigong, she was able to stop all medications and injections she was receiving for pain relief. She continues to have some weakness in her arms and her hands, but it does not interfere with her ability to complete her daily activities. Despite her extensive cervical spine surgery, she reports full mobility in her shoulders and has nearly recovered full mobility in her neck. Her energy has also greatly improved and she routinely teaches three Qigong classes per week. In addition to this, she teaches at special events, including a Qigong class at a large yoga festival with over 1000 people in attendance. Recently, she started her first full time job in over six years.
James is a 70-year-old male. Over ten years ago, he was diagnosed with multi-level degenerative disc disease (DDD) in his lumbar spine, as well as severe central stenosis or narrowing of his spinal column around his spinal cord at L3-4, L4-5, and L5-S1. In 2011, he had a CT scan and was told by his physician that it would not be long until he must rely on a wheelchair for all mobility. Surgery was presented as an option, but he was informed that the success rate was less than 10%.
He decided to forgo surgery and take his chances. Gradually, his legs became weaker and he would fall spontaneously. He worked as a salesman and as he was talking to clients, his legs would give out without warning and he would fall to the ground. To help his situation, he would park as close as he could to the entrance of stores or other destinations and would walk with carts or holding onto shelves or furniture. This would only help for so long before he would fall again.
He began looking for other options to help manage his condition. In 2012, he tried yoga. While it helped to temporarily control his pain, it had no effect on the weakness in his legs and he would continue to fall. One year later, at the suggestion of his wife, he tried a Qigong class. Due to his family’s personal schedules, he was unable to attend another class for two months. He decided to purchase a video of the movements he had learned and practiced them each day for 4–5 months until he was able to return to normal classes.
Since participating in Qigong classes, he has not fallen even once, and has no reports of pain. He stated that “Qigong gave me my life back.” He has no other medical problems to mention and takes no medications. He revealed that the improvements he has experienced have gone far beyond what he expected. Where he used to fall often and without notice, he has not fallen since he began qigong and reports that he now even has a “spring to his step, and a spring in his heart.” Not too long ago, he sustained a left rotator cuff tear. After continuing to practice qigong, he had a full return of strength and movement with no pain or difficulty with his routine daily activities, all within six months.
In his professional life, he felt like he was burning out as an IT programmer but practicing qigong has reinvigorated him. He was able to complete many projects (some complex) that he never would have thought possible. He has since become certified to teach Qigong and tells anyone who will listen about his story. He has also witnessed many others gain significant benefit from the practice of Qigong and is thrilled that he gets to share this with others.
|
pmc-6164177-1
|
A 64-year-old Native American man presented with worsening lower back pain, and numbness and tingling radiating from his belly button down both legs. At the time of admission, he reported gradually increasing weakness in both legs for 3 days that led to an inability to walk. His past medical history is significant for hepatitis C for many years, which led to liver cirrhosis. His past surgical history is significant for a previously repaired umbilical hernia. His family history included breast cancer (sister) and lung cancer (mother). He smoked cigarettes for 1–2 years in the 1980s, but it is unknown how many cigarettes he smoked per day. In addition, he was a former heroin abuser. He never consumed alcohol. He worked as a manager in the laundry department in a hospital. Family members deny any exposure to asbestos. An ultrasound of his liver 1 year prior to the current presentation reported coarse echotexture, suggestive of underlying cirrhosis. Several years earlier, he had not responded to interferon and ribavirin treatment. However, 1 year before presentation, he did respond to ledipasvir/sofosbubir (Harvoni) treatment. Although he cut the treatment short to just 5 weeks, a recent hepatitis viral test detected no hepatitis C ribonucleic acid (RNA). He had hepatitis C virus (HCV) RNA genotype 1a. He was a prior intravenous drug user and was in a methadone program. Home medications were as follows: nadolol, spironolactone, bumetanide, and methadone. On admission, his blood pressure (BP) was 109/67 mm Hg, heart rate (HR) 57 beats per minute, and temperature 36.6 °C. A physical examination had the following results: no jugular venous distention, his lungs were clear to percussion and auscultation, his heart sounded normal, there were no murmurs, his abdomen was slightly distended, his spleen and liver were not palpable, and some spider angioma was noted on his skin. On neurological examination: he was alert and awake; he was oriented to time, his name, and his location; and his cranial nerves were grossly intact. While no gait disturbance was observed, marked weakness of his lower extremities and swelling over the T9 area of his spine were found. He had a blood urea nitrogen (BUN) of 66 mg/dL, creatinine of 2.8 mg/dL, alkaline phosphatase of 505 U/L, aspartate aminotransferase of 210 U/L, alanine aminotransferase of 66 U/L, and total bilirubin of 1.5 mg/dL. Magnetic resonance imaging (MRI) of his thoracic and lumbar spine revealed a pathologic fracture at T11 with retropulsion and severe cord compression (Fig. ) and right chest wall and thoracic spine mass with tumor invasion into the spinal canal and thoracic cord compression at T6 (Fig. ). In addition, numerous metastatic lesions in his thoracic and lumbar spine were noted. A MRI scan of his chest/abdomen and pelvis without contrast was performed and revealed a large right liver mass and multiple lesions in his ribs, spine, and mediastinum, suggestive of metastatic disease. He was started on intravenously administered steroids. Surgical spinal cord decompression and stabilization/fusion of his spine was performed. Pathology results of an intervertebral disc and the T9 vertebral body reported metastatic carcinoma favoring HCC (Fig. ). Tumor cells were positive for Hep Par-1 and glypican-3 (Fig. ), and negative for cytokeratin (CK) 7, CK20, thyroid transcription factor 1 (TTF-1), inhibin, OCT3/4, prostate-specific antigen (PSA), prostatic specific acid phosphatase (PSAP), renal carcinoma marker (RCC), and PAX8. Subsequently, he was treated with radiation to the T11 spine lesion and was scheduled to begin radioembolization with yttrium-90, but his condition deteriorated, and he died 2 months after diagnosis. An autopsy was not performed.
|
pmc-6164177-2
|
The second case involved a 70-year-old Native American man presenting with upper back pain and numbness of his right foot for approximately 10 days. The symptoms had worsened, and he noticed some difficulty with walking. He did not have any past medical or surgical history. He was a former tobacco smoker and stopped smoking approximately 20 years ago, but it is unknown how many packs or cigarettes per day he smoked. He drank alcohol very rarely and not significantly. He did not have family history of any significance. He was never on medications until he was diagnosed as having HCC. He worked at a warehouse in the past. His job position was unknown. He has no known environmental or drug allergies. On admission, his BP was 166/119 mm Hg, HR was 97 beats per minute, and temperature was 36.7 °C. His physical examination had the following results: he was normocephalic, he had a non-traumatic skull, he had normal hearing, he had no nasal discharge, his chest wall movement was symmetric, his breath sounds were clear, he had no rales/wheezing, his HR was within the normal limit and had regular rhythm with no murmurs or thrills, his abdomen was soft with no distension, there was no palpable mass, there was no hepatomegaly or splenomegaly, a bilateral pedal pulse was present, there was no visible joint swelling, his skin was warm to the touch, he had normal color, and he had no rash/ulcers. A neurological examination had the following results: he was alert and awake; he was oriented to time, his name, and his location; his cranial nerves were grossly intact; he had no gait disturbance or motor deficits; his superficial reflexes were intact; a slight decrease in sensation over his right lower extremity was noted. Abnormal laboratory results were as follows: aspartate aminotransferase level of 104 U/L and alanine aminotransferase level of 90 U/L. CT of his chest and abdomen revealed a 10.0 × 8.3 × 7.4 cm soft tissue mass with associated osseous destruction involving the posterior right fourth, fifth, and sixth ribs and adjacent thoracic vertebral bodies, with significant soft tissue extension into the spinal canal and evidence of spinal cord compression. An additional lesion of the left iliac wing measuring 3.6 cm was noted. There were numerous enhancing lesions throughout his liver that were suspicious for primary versus metastatic disease (Fig. ). No signs of cirrhosis were detected on CT images. Further laboratory tests showed alpha-fetoprotein (AFP) levels of 98,884.0 ng/mL. Carcinoembryonic antigen (CEA) and cancer antigen (CA) 19-9 levels were slightly elevated. Hepatitis C RNA genotype 1a was detected. Hepatitis C RNA was 6.73 log IU/mL by reverse transcriptase polymerase chain reaction (RT-PCR).
An MRI of his thoracic spine was performed and revealed a large, posterior, left chest wall mass measuring 10.0 × 6.7 × 9.5 cm, with associated osseous destruction of the underlying right posterior fourth through seventh ribs and adjacent vertebral bodies, with complete obstruction of the right foramen and significant involvement of his spinal canal, causing spinal cord compression (Fig. ). A biopsy of the soft tissue mass was performed and showed a metastatic, poorly differentiated carcinoma favoring hepatocellular origin (Fig. ). Tumor cells were positive for hepatocyte, glypican-3, and pan-CK (Fig. ), and focally positive for CK20 and CEA but were negative for CK7 and TTF-1, CA 19-9, p63, CDX2, and OCT3/4. Because of the poorly differentiated histology and atypical presentation, he was treated with oxaliplatin plus fluorouracil/leucovorin []. After radiation of T5–T7 and a subsequent decrease in AFP levels, he eventually refused further therapy and was placed on hospice care. He died 6 months after diagnosis. An autopsy was not performed.
|
pmc-6164201-1
|
A 14-year-old girl came to Thammasat University Hospital for renal replacement therapy. She presented with end-stage renal disease (oliguria, anemia, and failure to thrive). She was the first child of healthy nonconsanguineous Thai parents. She was born at term to a 23-year-old mother and a 25-year-old father. Her prenatal and perinatal periods were uncomplicated. Her development was unremarkable until six months of age. She clawed by using her knees and elbows, instead of hands, at the age of 8 months. She was unable to walk on her feet but moved on her knees during her childhood period. She came to our hospital at the age of 12 years. Her weight was 25.4 kilograms (below the 3rd percentile) and her sitting height was 70 cm (below the 3rd percentile). Her vitals were normal, except high blood pressure at 120/80 mmHg (above 99th percentile for her age, sex, and height). She had pallor. Her distinctive facial features were the cloudy cornea, exophthalmos, underdeveloped ala nasi, maxillary hypoplasia, and micrognathia (). Upper extremity deformities included shortening of arms and forearms, flexion contracture of elbows, distorted wrists, and shortening of all fingers. Deformities of lower extremities were short thighs, short-bowed legs, and flexion contracture of knee. Distorted and restricted in motion of ankles, and deformities of feet were observed. Her heart, lungs, abdomen, and neurological examination were unremarkable. Her cognitive was appropriated with age.
Her initial investigations demonstrated blood urea nitrogen 120 mg/dL, serum creatinine 8.3 mg/dL, Na 134, K 4.5, Cl 95, HCO3 11 mmol/L, phosphate 2.6, calcium 3.8 mg/dL, albumin 0.6 g/dL, alkaline phosphatase (ALP) 13.5 U/L, with parathyroid hormone (PTH) level at 94 pg/ml. Her radiography of the upper extremities showed absence of carpal bones, osteolytic lesions of metacarpal, and distal ends of ulna and radial bones (). The radiography of the lower extremities demonstrated absence of tarsal bones, osteolytic lesions of metatarsal bones, and distal end of fibula (). In addition, severe cortical thinning of all bones indicating osteopenia was observed (Figures and ).
Ultrasonography showed small size of both kidneys and echocardiography demonstrated left ventricular hypertrophy. The diagnosis of end-stage renal disease was made with estimated glomerular filtration rate (GFR) of 7 mL/min/1.73 m2. All clinical presentation and investigations were compatible with the clinical diagnosis of MCTO. She received hemodialysis followed by continuous ambulatory peritoneal dialysis and medications including erythropoietin, ferrous fumarate, 0.50 µg of calcitriol, and 1400 mg of elemental calcium. Six months later, she developed generalized tonic-clonic seizure from hypercalcemia (12.4 mg/dL). Her serum chemistry demonstrated normal serum phosphorus level (3.9 mg/dL) and low serum alkaline phosphatase (4 U/L), with normal serum PTH level at 103 pg/mL. These findings indicated a marked reduction in the bone uptake of calcium after a period of calcium supplement. Calcium and calcitriol were discontinued until serum calcium returned to normal level, then only 720 mg of elemental calcium was reintroduced. Two years later, she had a slightly high serum calcium level (10.8 mg/dL), normal serum phosphorus level (3.5 mg/dL), and normal PTH level (113 pg/ml). Interestingly, during the past 2 years, we observed persistently low serum ALP levels (2.6–4.9 U/L) [], indicating poor osteoblastic activity and limited bone formation. Thus, impaired bone mineralization from dysregulation of osteoblast and osteoclast was suspected and MAFB mutation might be responsible for MCTO disease in this patient.
We performed a genetic testing, MAFB sequencing on her and her parental blood. The genomic were isolated from peripheral lymphocytes using Puregene DNA extraction kit (Qiagen, Valencia, CA). A short region of the amino-terminal transcriptional activation domain of the MAFB gene, containing mutation hotspots, was amplified by polymerase chain reaction (PCR). We designed primers by using software (Primer3Plus). The forward and reverse primer sequences for PCR amplification were 5′-GCTCAAGT TCGACGTGAAGA-3′ and 5′-GTAGTTGCTCGCCATCCAGT-3′, respectively. PCR products were visualized on a 2% agarose gel and purified using DyeEx 2.0 spin kit (Qiagen, Valencia, CA). The products were then sequenced by capillary electrophoresis. This study was approved by the ethic committee of the Faculty of Medicine, Thammasat University, Thailand (MTU-EC-PE-1-005/59).
We identified a de novo heterozygous missense mutation at nucleotide 197 from C to G (NM_005461.4; c.197C > G; ), predicting the change of amino acid at codon 66 from serine to cysteine (p.Ser66Cys). There was negative in both mother and father's result. This missense mutation occurred within the transactivation domain of MafB protein. This serine at the codon 66 is evolutionarily conserved among species, and in silico prediction of pathogenicity programs classify this variant as deleterious (SIFT) and probably damaging (PolyPhen).
|
pmc-6164205-1
|
A 76-year-old woman with malignant melanoma of the upper gingiva underwent subtotal maxillectomy and neck dissection of the right side. Six months after tumor resection, two zygomatic implants were inserted into bilateral zygomatic bones. After another 6 months, second-stage surgery was performed and two dental implants were placed in the anterior region of the maxilla. However, the position and depth of the dental implants were inappropriate for the final prosthesis. Therefore, the two anterior implants could not be used for support. The zygomatic implants and prosthesis have remained stable for 3 years since functional loading (Figures –).
|
pmc-6164205-2
|
An 81-year-old man was diagnosed with squamous cell carcinoma of the left maxillary gingiva and underwent partial maxillectomy. Two years after tumor resection, two dental implants in the anterior maxillary region and one zygomatic implant into the right side zygomatic bone were placed. After another 6 months, second-stage surgery was performed; however, one dental implant in the anterior region had to be explanted due to loss of osseointegration. Subsequently, the implants and prosthesis have remained stable for 1 year and 6 months since functional loading (Figures –).
|
pmc-6164205-3
|
An 83-year-old woman had a chief complaint of difficulty in eating due to severe instability of her upper removable denture. Fifteen years ago, she had been diagnosed with malignant melanoma of the maxillary gingiva. After preoperative superselective arterial injection chemotherapy, bilateral partial maxillectomy and postoperative concurrent chemoradiotherapy were performed. Thirteen years after tumor resection, two dental implants and two zygomatic implants were placed on each side of the zygomatic bones. Two years after functional loading, the left abutment with magnetic attachments was fractured. A new abutment with magnetic attachments was fabricated, and the prosthesis is currently being used without any complications (Figures –).
|
pmc-6164207-1
|
A 76-year-old female presented to the emergency department with complaints of the left thigh and hip pain and swelling for five days. She reported having a history of chronic left leg sciatic pain that contributed to a fall two days prior to the onset of these symptoms. Her past medical history was significant for colon cancer requiring a low anterior resection, which is eight years ago. The patient was noted to be confused and tachycardic. She was afebrile but had leukocytosis of 14,000. On physical examination, she was noted to have a significant crepitus to the left thigh and knee. Radiographs of the left leg confirmed subcutaneous emphysema consistent with necrotizing fasciitis (). Prior to surgical consultation, the patient also received a pelvic computed tomography (CT) scan to evaluate for hip fractures. This further confirmed the necrotizing fasciitis (Figures and ) but also identified a collection in the presacral space () that communicated to the left leg through the left sciatic notch, which is consistent with an AL. The patient was immediately taken to the operating room for debridement of the thigh and diverting colostomy.
An exploratory laparotomy with diverting colostomy was created to control ongoing contamination of the leg. Intra-abdominally, there were no abnormal findings, which is consistent with the extraperitoneal nature of the disease process. The decision, at this point, was to access the extraperitoneal collection through interventional radiology so as to minimize intra-abdominal contamination. After the colostomy was completed, the left thigh and hip were incised revealing a significant amount of feculent and purulent drainage. Necrotic, nonviable tissue was debrided down towards the knee, and the wound was left open and dressed. The patient was septic during the procedure and remained septic postoperatively. After an initial discussion with the patient's family, the plan was to perform percutaneous drainage of the presacral abscess postoperatively and obtain an orthopedic consultation as the hip joint was actively infected from the AL.
Recommendations by orthopedic and trauma consultants were that the patient would initially need an above the knee amputation due to the significant soft tissue loss and function from the extensive debridement. Furthermore, their concern was that this patient may ultimately need disarticulation of the left hip with potential hemipelvectomy if severe and recurrent osteomyelitis developed.
The patient's family ultimately decided to withdraw care, and the patient died in the hospital on day three.
|
pmc-6164210-1
|
A healthy female Caucasian patient (M.M, 46 years old) with an overall good oral hygiene attitude presented at our private dental practice in 2008; following a preliminary full-mouth dental bleaching and direct conservative therapies (i.e., restorations at elements 1.6–1.7) at the right maxillary quadrant, a decision was made to replace an old metal-ceramic prosthetic crown of tooth 1.5. Lateral and occlusal views of the preexisitng restoration are shown in Figures and . Esthetic reasons guided the replacement, in order to achieve a new optimal integration with adjacent bleached teeth. At start of the new restorative cycle, informed consent was obtained.
|
pmc-6164221-1
|
This is a 60-year-old man who presented with gum pain of one-month’s duration. His pain emanated from an area of exposed jaw bone in the left lower posterior gum. Two months earlier, he had noticed a blister in the same area, which became an abscess and was subsequently drained. He also had bad dentition that required several recent visits to his dentist. He had multiple recent teeth extractions and several artificial crowns. In addition, he had 20 pounds of weight loss as well as night sweats for the six months prior to presentation. He had no fever, neck mass or external neck draining ulcers.
His past medical history included type 2 diabetes mellitus, hypertension, hyperlipidemia and chronic obstructive pulmonary disease. He had a history of penicillin allergy (rash). He had been recently prescribed oral clindamycin for one month for his oral lesion with no improvement.
His vital signs were normal. Mouth examination showed exposed bone around the root sites of teeth #18 and 19 (see arrow), with artificial crowns over several teeth in the lower jaw (). There were no enlarged cervical lymph nodes and examination of other systems was unremarkable.
Biopsy of the left mandibular bone around the root sites of teeth #18 and 19 was obtained and sent for histopathology, aerobic and anaerobic bacterial, fungal and mycobacterial cultures. Bacterial culture grew alpha hemolytic streptococcus, Eikenella corrodens and Micrococcus spp. Fungal and mycobacterial cultures were negative.
Laboratory blood work including complete blood count, electrolytes and kidney function were completely unremarkable.
Computerized tomography maxillofacial imaging showed a lytic lesion in left ramus of the mandible with loss of bone matrix (). Chest radiograph was completely normal. Differential diagnoses considered in addition to actinomycosis included nocardiosis, tuberculosis, osteosarcoma of the mandible and endemic fungal infections.
Histopathological examination of the mandibular bone showed osteonecrosis, sulfur granules and embedded organisms on hematoxylin and eosin (H&E) stain ( and ), which were better characterized on Gomori-Grocott methenamine silver stain (GMS) as multiple branching organisms (). The official histopathology report read, “acute and chronic osteomyelitis with Actinomyces-like organisms”. A diagnosis of actinomycosis was made, following which the patient was desensitized and treated with intravenous penicillin G for two weeks, followed by oral penicillin VK for six months. He made a complete recovery at the end of therapy with total resolution of symptoms and closure of the exposed bone.
|
pmc-6164221-2
|
This describes a 70-year-old woman who presented with left upper jaw pain and mastication difficulties of several weeks’ duration. She had undergone complete dental extraction three months earlier. There were no other significant complaints. Her past medical history was significant for multiple myeloma treated with pomalidomide and 20 mg weekly oral dexamethasone for nine years prior to presentation. She also had history of type 2 diabetes mellitus, hypertension and chronic kidney disease stage 3.
Vital signs were within normal limits. Her physical examination was also unremarkable except for the oral examination, which showed she was completely edentulous. In addition, she had an area of sequestrum with overlying calculus noted in the left maxilla bone corresponding to the extraction sites of teeth #11, 12 and 13. There were no surrounding lymph node enlargements or other significant examination findings.
Routine laboratory blood analysis, including complete blood count and complete metabolic profile, was unremarkable.
The piece of sequestrum was removed leaving a “hole” in the upper jaw and was subsequently sent for histopathology. The tissue sections revealed osteonecrosis, osteolytic changes with acute inflammation. The osteolytic spaces were filled with Actinomyces-like organisms and a few fragments of foreign material consistent with vegetables.
The clinical impression of acute osteomyelitis caused by actinomycosis was made. A possible differential that was also considered was medication-related osteonecrosis of the jaw (MRONJ), since she had been on chronic steroids and pomalidomide therapy for several years. Unfortunately, microbiologic cultures were not sent on the specimen obtained from the upper jaw.
The patient was started on a six-month regimen of penicillin VK 500 mg orally four times daily in addition to oral hygiene measures. At her clinic follow-up one month later, her symptoms had completely resolved, and the oral defect was beginning to close. At the end of her six-month therapy, she had made a complete recovery.
|
pmc-6164362-1
|
The patient reported is a 56-year-old woman who was diagnosed with acute myeloid leukemia (AML) with cytogenetic abnormality of inversion 16 in 2013. She achieved a complete remission (CR) after standard induction chemotherapy with 7 + 3 regimen consisting of ara-C and daunorubicin followed by consolidation with high-dose ara-C (HiDAC). She relapsed a year later and was re-induced with a salvage chemotherapy regimen MEC (mitoxantrone, etoposide, and cytarabine) achieving a second CR, which was followed by a matched unrelated allogeneic stem cell transplant (allo-SCT). Her posttransplant course was uneventful without significant graft versus host disease and prolonged requirement for immunosuppression. Two years after allo-SCT, she had a central nervous system (CNS) relapse of her original leukemia and presented with an infiltrating lesion in the lumbosacral spine; her CSF cytology was positive for myeloblasts. She was admitted to the hospital to receive reinduction chemotherapy; her vitals upon admission were as follows: temperature 37.7 °C, blood pressure (BP) 129/65 mmHg, heart rate (HR) 72/min, and respiratory rate (RR) 14/min. She was started on intrathecal chemotherapy with ara-C and systemic chemotherapy with the salvage chemotherapy regimen FLAG-IDA (fludarabine, ara-C, and idarubicin). The day chemotherapy started for the patient was noted as day 1. On day 10, the patient developed neutropenic fever, and the white blood count (WBC) noted to be <0.1 × 109/L with absolute neutrophil count (ANC) of 0. She was started on intravenous (IV) cefepime 2 g every 8 hour after evaluation for underlying infectious etiology was done. The work up did not isolate any organism and included blood culture, urine culture, and chest X-ray. On day 16, the patient developed left upper quadrant abdominal pain. Vital signs then were as follows: maximum temperature (Tmax) 37.5°C, along with HR of 80–94/min, RR 16–18/min, and BP SBP 105–126/DBP 55–71 mmHg. Her blood tests then were as follows: WBC <0.1 × 109/L, ANC 0, hemoglobin 8.0 g/dl, platelet 12 × 109/L, and serum blood chemistry and liver function tests were noted to be without significant derangements. A CT scan of the abdomen was performed that showed diffuse thickening of stomach wall (), concerning for infectious or infiltrative malignant process. Her absolute neutrophil count had been at the nadir for 10 days prior to this development. Her antimicrobial coverage was increased to include anaerobic coverage by changing her antibiotic regimen from IV cefepime 2 g every 8 hour to IV piperacillin/tazobactam 3.375 g every 6 hour, leading to short-lived symptomatic improvement for roughly two weeks. Upon symptom recurrence, the patient was noted to be febrile with Tmax of 39.5°C, along with HR of 109–139/min, RR 18–20/min, and BP SBP 94–124/DBP 55–71. Two sets of peripheral blood cultures were drawn which did not show growth of any organism after 5 days of incubation. The patient continued to remain hemodynamically stable.
An upper gastrointestinal (GI) endoscopy was performed which showed a large ulcerative lesion with purulent discharge and inflammatory changes (). Citrobacter freundii, Enterococcus faecalis, and Bacillus cereus were isolated from culture on gastric biopsies. Imaging, endoscopic, and microbiological findings were consistent with phlegmonous gastritis.
Infectious disease service was consulted, IV piperacillin/tazobactam 3.375 g every 6 hour was stopped, and the antibiotic regimen was changed to IV vancomycin (managed per pharmacy protocol based on weight and renal function) and IV meropenem 1 g IV every 8 hour. The recommendation for broad coverage of microorganisms was made by infectious disease service, given the high risk of mortality associated with phlegmonous gastritis. Prophylactic antifungal and antiviral for neutropenia were continued. Organism susceptibilities were carried out for Citrobacter freundii and Enterococcus faecalis, which revealed Citrobacter freundii to be resistant to ampicillin, cefazolin, and cefuroxime, while Enterococcus faecalis was noted to be pansensitive. Per organism susceptibility and with the help of infectious disease service, her antibiotic regimen was changed to IV cefepime 2 g every 8 hour, IV metronidazole 500 mg IV every 8 hour, and IV vancomycin per pharmacy protocol. This antibiotic regimen was continued for a total of two weeks. The patient's gastrointestinal symptoms resolved quickly, and she was able to resume normal diet. Follow-up CT scan a month later showed marked improvement in gastric thickening (). She is currently doing well with her AML in remission and no recurrence of her GI symptoms.
|
pmc-6164439-1
|
Case 1 involved a 3-day-old male Boxer puppy that presented at the Clinic for Obstetrics, Gynecology and Andrology of Large and Small Animals with Ambulatory Service of the Justus-Liebig University in Germany. The owner reported that the puppy was very active and continuously suckling. Nonetheless, he did not gain weight, and the owner frequently observed nasal discharge after suckling.
On examination, the puppy exhibited a good general condition and was very lively. Intraoral examination revealed CP (). The CP was characterised by a midline defect in the hard palate and a caudally divergent defect in the soft palate. The defect was moderately wide, and no asymmetrical facial growth could be detected. CP was classified as “- - H S H - -“ according to the LAHSAL classification []. No other congenital diseases were detected, and the puppy showed no signs of pneumonia. When milk replacer (Babydog Milk®, Royal Canin, Köln, Germany) was offered, the puppy suckled immediately. Although he showed nasal discharge and occasional sneezing, he demonstrated a good appetite. He required a long time to consume the amount of milk necessary for gaining body weight. Because of his good general condition, an attempt at raising him was made and the puppy was assigned to the clinic.
|
pmc-6164847-1
|
The patient is a 61-year-old Caucasian female, height 168 cm, weight 58 kg, with an uneventful previous history until age 55, when she developed precordial pressure after exposure to psychosocial stress after the unexpected death of her father. ECG showed left anterior hemiblock, missing R-progression until V4, and flat T-waves in III, aVL, and V1. Echocardiography revealed moderately reduced systolic function, dyskinesia of the interventricular septum, and regional wall motion abnormalities, indicative of TTS. Coronary angiography was normal but ventriculography was indicative of TTS (). Cardiac MRI (cMRI) revealed a reduced systolic function with a left ventricular ejection fraction (EF) of 40%. Stress testing revealed reduced physical capacity. TTS resolved after a few days without therapy. The family history was positive for sudden death of her brother at age 66 and her grandmother from the mother’s side at age 77. Her mother, aged 85 suffered from heart failure.
At age 57 tachycardious atrial fibrillation (AF) and isolated ventricular ectopic beats were recorded, which resolved spontaneously. ProBNP was 866 ng/L (n, 0–247 ng/L). The EF on cMRI had slightly improved (48%) compared to the previous cMRI. After initiation of a neurohumoral therapy with carvedilol, angiotensin-converting enzyme inhibitors (ACEI), and a statin, systolic function improved, stress test became normal, and proBNP declined to 152 ng/L. At age 59 the EF deteriorated again to 48% and the LVEDD to 59 mm. Despite re-establishing β-blockers, the EF further decreased to 40%, the LVEDD increased to 68 mm, and the proBNP to 1058 ng/L. ECG showed stable sinus rhythm but there was easy fatigability upon psychosocial stress.
After pneumonia at age 60, severe heart failure developed with an EF of 18%. Echocardiography showed mitral insufficiency and pulmonary hypertension. Coronary angiography was normal again. Myocarditis was excluded upon cMRI (). Levosimendan was given once, followed by sacubitril and valsartan in combination and ivabradine. The latter had to be discontinued after two months because of a suspected arrhythmogenic effect. Since the patient initially refused implantation of an implantable cardioverter defibrillator (ICD), a LifeVest® was prescribed. Already one day after dismissal, the LifeVest® delivered an appropriate shock because of ventricular fibrillation. After admission, three further episodes of ventricular fibrillation occurred, which were all terminated by adequate LifeVest® shocks. Because of a suspected pro-arrhythmogenic effect, procoralan was discontinued and a therapy with amiodarone begun. Additionally, an ICD was implanted, genetic investigations initiated, and the patient was scheduled for heart transplantation (HTX).
Genetic testing by means of a gene panel covering 40 genes associated with dilated CMP (dCMP) revealed the heterozygous mutation c.1489G > T (p. E497X) in exon 9 of the titin gene. The neurological history was noteworthy for intense myalgias during gripal infections since years and sore muscles during one month with pneumonia. Since clinical neurologic exam and creatine-kinase (CK) were normal, no further invasive work-up for myopathy was conducted. At discharge she was on a therapy with sacubitril (97 mg/d), valsartan (103 mg/d), nebivolol (1,25 mg/d), amiodarone (50 mg/d), spironolactone (25 mg/d), furosemide (40 mg/d), and duloxetine (30 mg/d).
|
pmc-6165110-1
|
On 13 May 2016, a 30-year-old male presented with fever (39–40 °C) and headache followed by severe arthralgia and arthritis of both wrists, metacarpal, and phalangeal joints bilaterally, and the right ankle. Around five weeks later, the patient remained with persistent fever. And, amoxicillin-clavulanate was started to treat a lower respiratory tract infection with remission of symptoms although arthralgia/arthritis remained. Early July, the patient evolved again with recurrent fever and severe arthralgia/arthritis associated with loss of joint function without response to non-steroidal anti-inflammatory drugs (NSAIDs), being admitted to the hospital five days post new symptom onset (). Informed written consent was obtained from the participant after approval by the Ethics in Research Committee of Hospital Universitário Clementino Fraga Filho, Federal University of Rio de Janeiro (reference CAAE 02920212.8.3001.5279).
The patient was diagnosed with systemic lupus erythematosus (SLE) at the age of 9; he was diagnosed with Nephritis class III/IV in 2007 and with a common variable immunodeficiency, in 2014. Despite previous irregular treatment with immunosuppressive and immunomodulatory therapy such as Rituximab (2008–2010), the patient was using Prednisone, Hydroxychloroquine and Cyclosporine in addition to Colchicine and Prophylactic Azithromycin at hospital admission ().
Physical examination showed a temperature of 36.5 °C, redness, warmth, and swelling of left and right wrists and right ankle. Although there were no clinical manifestations of nephropathy, laboratory results showed a slight renal impairment. In addition, he presented anemia, leukocytosis with neutrophilia, lymphopenia and elevated inflammatory markers, such as C-reactive protein (CRP) ().
Because septic arthritis by gonococcal infection was initially suspected, ceftriaxone and azithromycin were used in addition to prednisone dose reduction and cyclosporine withdrawal (). All hemocultures were negative and synovial liquid (SL) was not accessible by needle aspiration. Ultrasonography showed severe inflammatory joint disease ( and ). Because the right ankle also presented high inflammatory response, a synovial biopsy was indicated. Tissue fragment showed fibro-adipose overgrowth, hypervascularization and granulation tissue with mono and polymorphonuclear infiltrate and fibrin deposition, still suggesting infectious arthritis. No bacterial, fungal or Mycobacterium tuberculosis infections were detectable by direct examination or culture. However, viral etiology could not be excluded yet.
Arbovirus co-transmission increased markedly in Rio de Janeiro in 2016, and the patient’s mother and sister had a history of non-laboratory proved Chikungunya virus (CHIKV) and Zika virus (ZIKV) infection, respectively. Arthritogenic arboviruses was then investigated. Molecular diagnosis and ZIKV and CHIKV isolation in cells culture were performed in serum, urine and/or tissue samples. Viral RNA of serum, urine, and tissue were extracted from 250 μL of clinical samples using TRIzol LS Reagent (Ambion Inc, Foster City, CA, USA) according to the manufacturer’s recommendations. cDNA synthesis and real-time RT-PCR for detection of CHIKV and ZIKV RNA were performed by GoTaq 1-Step Probe RT-qPCR System (Promega Corporation, Madison, WI, USA) according to the manufacturer’s recommendations. The real-time RT-PCR protocol included specific primers and probe sets previously describe [,]. Biological samples showing amplification by real-time RT-PCR underwent culturing for viral isolation in cells of different lineage, such as Macaca mulatta kidney (LLC-MK2) and African green monkey kidney epithelial cells (VERO and MA-104). Commercial kits Anti-CHIKV IgM and IgG ELISA (Euroimmun Corporation, Luebeck, Germany) were used for detection of anti-CHIKV IgM, IgG, and the Dengue IgM Capture DxSelect, Dengue IgG DxSelect and Dengue NS1 antigen DxSelect (Focus Diagnostics, Diasorin Molecular LLCCypress, CA, USA) were used for the detection of IgM and IgG anti-DENV and NS1 antigens. CHIKV RNA amplification in the patient serum was detected on day 62 and low level of IgG anti-DENV was also demonstrated ( and ).
The renal function declined progressively, methylprednisolone pulse therapy and intravenous immunoglobulin (IVIG) were introduced along with mycophenolate. Renal function improved to pre-admission level in addition to a modest clinical and ultrasonographic improvement of the arthropathy ( and ).
After hospital discharge, the patient had another admission and needed drug adjustment after drug-induced untoward effects. In addition, recurrent arthralgia and joint immobilization correlated with active inflammatory response confirmed by bone scintigraphy, USG and MRI ( and ).
Both SLE and/or viral-induced disease were investigated. After 275 days post symptoms onset, the patient still presented ZIKV RNA in both blood and urinary compartments (). The patient died 28 days later of renal failure and pulmonary sepsis.
|
pmc-6165585-1
|
A 40-year-old male originally from the Netherlands presented to the clinic with an acute onset of unilateral left cervical adenopathy that began approximately three months prior. The lymphadenopathy was uncomfortable but not reported as painful. Prior medical history includes several kidney stones, and the patient as a child underwent tonsillectomy and tympanostomy tubes. The patient had never smoked, and a review of the family history did not reveal any instances of cancer. No other signs or symptoms were present to suggest lymphoma or leukemia, and the patient denied fever, chills, night sweats, or weight loss. The patient denied headache, seizures, or any other neurological deficits that would have indicated extra nodal central nervous system involvement []. Spontaneous reduction of lymphadenopathy was observed on a subsequent visit three weeks later. A long-term follow-up was discussed and arranged with the patient to follow the course of the disease.
CT imaging demonstrated a 3 cm lymph node in the left level II region as well as several 1 cm left jugulodigastric nodes. Additionally, a PET scan of the torso (from skull base to thigh) was performed after the injection of 17.4 mCi of fluorine-labeled fludeoxyglucose and revealed the left level II and jugulodigastric nodes with a maximum standardized uptake value of 3.0. No other significant metabolic activities or lymphatic involvement was seen in the chest, abdomen, or pelvis.
The patient was referred for excisional biopsy, and the tissue was reviewed by a team of hematopathologists. The excised lymph nodes measured 3.3 × 2 × 1.3 cm and 1 × 0.7 × 0.2 cm. Histological section revealed native architecture distortion by excessive numbers of large histiocytes with abundant foamy cytoplasm (). No histiocytic atypia was observed, and acid-fast staining and GMS fungal staining were negative. Reed-Sternberg cells and well-defined granulomas were absent. The nodes were stained with a S100 protein monoclonal 4C4.9 antibody at a dilution of 1 : 50 which revealed numerous S100-positive histiocytes. The nodes were free of signs of malignancy. Emperipolesis, one of the few definitive diagnostic criteria for the disease, was evident on microscopy ().
Laboratory results of the collected complete blood count, complete metabolic panel, and lactate dehydrogenase labs were all within normal limits. Serum protein electrophoresis detected IgG kappa monoclonal proteins of low concentration.
|
pmc-6165590-1
|
In February 2013, a 60-year-old Caucasian woman was admitted to the V. A. Nasonova Research Institute of Rheumatology with complaints of weakness, weight loss of 25 kg over 2 years, dryness of the eyes and mouth, and enlargement of the cervical and axillary lymph nodes. Her medical history was consistent with a 20-year course of SS. Since May 2011, she had experienced numbness in her feet followed by the development of Raynaud's syndrome and recurrent purpura on the shins. Increasing and decreasing lymphadenopathy involving the neck and axillae was observed over 2 years. Within 3 months before admission, she was taking 4 mg of methylprednisolone every other day.
At the time of admission, peripheral blood counts were as follows: hemoglobin 13.6 g/dl, platelets 224 × 109/L, and white blood cells 6.7 × 109/L, with 85% neutrophils, 8% lymphocytes, and 7% of monocytes. Laboratory data showed that electrolytes and renal and liver function were within the normal range. The serum lactate dehydrogenase level was elevated to 593 IU/L (normal <378). Ophthalmic examination revealed that the patient had keratoconjunctivitis sicca (with filamentous keratitis), with a Schirmer's test score of <1 mm/5 min and tear break-up time of 3-4 sec (OD) and 8 sec (OS). Dental examination of the submandibular and parotid salivary glands showed no salivation (sialometry: 0 mL). A labial salivary gland biopsy showed marked focal lymphocytic sialadenitis with focus score of 4 foci per 4 mm2. Anti-SSA/Ro antibody level exceeded 200 U/ml (normal < 25), antinuclear antibody level was 1:640 (normal < 1:160) with homogeneous and speckled patterns, and rheumatoid factor level was 885 IU/mL (normal < 20). Based on the clinical, serological, and pathological features, the diagnosis of SS was confirmed.
Protein electrophoresis and immunofixation of the patient's serum showed monoclonal immunoglobulin (Ig) M kappa (1.5 g/L) and a decrease in polyclonal IgG and IgA levels. The monoclonal Ig had the property of cryoglobulin. Urine protein electrophoresis and immunofixation assays detected the presence of monoclonal free kappa type light chains (0.06 g/24 h). Increased β2-microglobulin and C-reactive protein levels were noted to be as high as 5.99 mg/L (normal < 2.4) and 47.7 mg/L (normal < 6.0), respectively. In addition, the C4 complement level was reduced to 0.02 g/L (normal range 0.1–0.4). A full staging computerized tomography (CT) scan showed multiple enlarged lymph nodes in the neck, axillary, mediastinal, lung hilar, abdominal, and retroperitoneal spaces, with a fusion of the conglomerates (maximum size of 4.1 × 2.4 cm) and mild splenomegaly (measuring 8.2 × 13.3 × 15.2 cm). Additionally, bilateral lymphangitis was found in the lung parenchyma, and the patient had developed moderate bilateral hydrothorax with compression of basal segments of the lower lobes.
Histologic examination of the right axillary lymph node revealed a completely effaced architecture (). The sinus system was open and filled with pleomorphic histiocytic cells, some of which showed emperipolesis of peripheral blood cells (). Immunohistochemical staining showed the expression of CD68 () and CD163 in these histiocytic cells accompanied by coexpression of S100 (), while the staining result was negative for CD1a and Langerin. The lymph node tissue showed an infiltrate consisting of partly nodular and partly diffused pattern of CD20-positive and CD10-negative B-lymphocytes and extensive plasma cell infiltration (). The plasma cells showed positive staining for light kappa chain (), IgM heavy chain (), and MUM1 (). However, they did not stain positive for the expression of CD138, VS38c, CD56, and Cyclin-D1. Ki-67, a marker of cell proliferation, was expressed in 5–7% of the cells of the sinuses and up to 50% of the cells within the intervening cords of the lymph node (). We failed to detect Human herpesvirus-8 by immunohistochemistry and EBV-encoded RNA (EBER) in situ hybridization (EBER-ISH), but the DNA of the EBV was detected in the sample by the polymerase chain reaction (PCR). Further, sequence analysis of lymph node tissue showed no mutations within the codon 265 of the MYD88 gene and somatic mutations in KRAS and MAP2K1. Histological and cytological analysis revealed no infiltration of bone marrow by lymphocytes and plasma cells. Thus, the patient was diagnosed with concomitant RDD and plasmacytic marginal zone lymphoma. After six cycles of MDB (melphalan [Alkeran] + dexamethasone + bortezomib), a partial response was achieved according to the data of the CT scan. As follow-up analyses, electrophoresis and immunofixation of serum proteins were performed that failed to detect any presence of monoclonal IgM kappa. Electrophoresis followed by immunofixation of concentrated urine proteins showed secretion of the monoclonal free kappa type light chains in trace amounts. In November 2013, a month after the end of chemotherapy, a fast-growing firm mass developed in the left mandibular fossa spreading anteriorly to the parotid area. Soon, peripheral paralysis of the facial nerve occurred (). Histological examination of this mass revealed a necrotic tissue interspersed with foci of small lymphoid cells with scarce large tumor cells, mainly with round and oval nuclei containing nucleoli and pyroninophilic cytoplasm. Large tumor cells were also present as perivascular and perineural clusters (). Small lymphoid cells were predominantly T lymphocytes (CD3+) and expressed either CD4 or CD8, approximately in equal proportions. Large lymphoid cells expressed CD20 (), CD79α, CD30 (), light kappa chain (), IgM heavy chain (), PAX5, and MUM1 (). Expression of Ki-67 was observed in nearly 60% of tumor cells. The EBER-ISH demonstrated the presence of EBV-positive large lymphocytes (). EBV-positive diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS) was diagnosed.
Multiplex BIOMED-2 PCR fragment assays were used to study rearrangements of the Ig heavy chain (IGH) gene in NMZL and DLBCL samples. IGH framework 1, 2, and 3 assays (Tube A, Tube B, and Tube C) were used to detect Vh-Jh rearrangements. Identical clonal patterns between the NMZL and DLBCL were demonstrated (). The resulting condition was considered a transformation of NMZL into an EBV-positive DLBCL, NOS. R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) immunochemotherapy was prescribed to the patient. Unfortunately, a few days following the completion of the first cycle, the patient suddenly lost consciousness and died. No postmortem study was performed.
|
pmc-6165595-1
|
A 51-year-old male, with a remote previous history of L4-L5 spinal decompression and fusion, presented in our outpatient clinic with worsening lower back pain. Physical examination showed lumbar radiculopathy and neurogenic claudication, while a magnetic resonance imaging (MRI) scan of the lumbar spine revealed disc protrusions and high-grade spinal canal stenosis at the L2-L3 and L3-L4 levels. Consequently, he underwent elective spinal decompression revision surgery, with an extension of instrumented fusion from L2-L5.
On experiencing new onset persistent headaches on the second postoperative day, a computerized tomography (CT) myelogram was performed, and showed CSF leakage from a dural tear at the L3-L4 level (). Soon after the CT myelogram, image-guided lumbar drain placement was performed, and 8cc of fibrin glue was injected at the site of the leak.
On the fourth postoperative day, an urgent unenhanced CT scan of the head was performed after the patient developed altered mental status, confusion, disorientation, and slurred speech. The CT scan revealed areas of acute haemorrhage in both cerebellar hemispheres, with mass effect on the fourth ventricle and the brainstem and mild obstructive hydrocephalus (). Subsequent review of nursing charts revealed excess CSF drainage over the previous night; thus, immediate clamping of the lumbar drain was performed, and an external ventricular drain (EVD) was placed by the neurosurgery team. MRI scanning of the brain, with and without contrast, revealed evidence of intracranial hypotension ().
On the fourteenth postoperative day, the patient had an open surgical dural repair using direct suture closure, along with DuraGen® (a synthetic dural allograft), and fibrin glue. Additionally, the lumbar drain was successfully removed.
The patient's subsequent hospital course was complicated by deep venous thrombosis and respiratory failure, and he was ultimately discharged to the rehabilitation unit after EVD removal, ten days after the open dural repair surgery. No residual neurological deficits were present at the time of discharge.
|
pmc-6165602-1
|
A 67-year-old man was brought to the emergency department by ambulance after being found lying on the floor of his home. He reported a fall onto his right hip with a long lie for an estimated 24 hours. His medical history was significant for opioid use disorder, gout, hypertension, and osteoarthritis. He had undergone prosthetic joint replacement of the left shoulder nine years before this presentation. He had chronic bilateral knee pain but reported acute worsening of pain in his right knee starting one day before the fall. He endorsed a history of intramuscular injection of opioids to his buttocks and upper arms but denied injection to his hip or knee.
On examination, he was found to have a swollen right knee with no erythema or signs of trauma. The X-ray of the right knee is shown in . His initial blood pressure was 240/120 mmHg. His serum creatinine of 159 µmol/L was significantly increased from baseline, but creatine kinase was not markedly elevated (447 U/L). Laboratory testing also revealed leukocytosis (20.2 × 109 cells/L) with neutrophil predominance (18.2 × 109 cells/L) as well as elevated C-reactive protein (292 mg/L). Serological tests for HIV, hepatitis B, and hepatitis C were negative.
After admission to hospital, the patient's kidney injury resolved promptly with volume repletion, and his hypertension responded to antihypertensive medication. On his third day in hospital, the patient reported urinary urgency without dysuria or gross hematuria, which prompted investigation though he later denied having had urinary symptoms. Urine dipstick showed positive blood (250 ery/µL), positive leukocyte esterase, and positive nitrite; according to local laboratory protocol, microscopy of urine sediment and Gram stain were not performed. Urine culture showed >100 × 106 CFU/L yellow-grey colonies and nonlactose fermenting colonies on blood agar and MacConkey agar, respectively, after 24 hours of incubation. Oxidase test was positive. It was identified as B. vietnamiensis, a B. cepacia complex organism, by BD™ Bruker MALDI Biotyper™.
During his admission, the patient's right knee became more swollen and painful until he could no longer mobilize. Treatment for gout was ineffective in relieving his symptoms, so a joint aspiration of the right knee was performed. The knee joint aspirate was frankly purulent fluid with a nucleated cell count of 26 766 × 106 cells/L, of which 95% were neutrophils, and microscopy revealed intracellular calcium pyrophosphate crystals. Culture grew >100 × 106 CFU/L grey colonies on blood and chocolate agar plates after 24 hours of incubation. The Gram stain from the growth showed Gram-negative rods, which were also identified as B. vietnamiensis by MALDI. For the synovial fluid isolate, susceptibility testing by E-Test® indicated minimum inhibitory concentration values in the susceptible range for meropenem, levofloxacin, trimethoprim/sulfamethoxazole, ceftazidime, and minocycline (0.5 µg/mL, 1 µg/mL, 0.75–1 µg/mL, 0.75–1 µg/mL, and 0.38–0.5 µg/mL, respectively) according to Clinical and Laboratory Standards Institute breakpoints []. Antibiotic susceptibility profile for the urine culture isolate using Vitek 2® was similar to the results from the knee joint aspirate. Blood cultures had no growth of bacteria. Transthoracic echocardiogram showed no evidence of endocarditis.
The patient was treated with three days of empiric meropenem, which was subsequently switched to ceftazidime with a plan to complete six weeks of therapy. Irrigation and debridement of the knee joint was performed four days after the diagnostic aspiration. His knee pain improved significantly, and after twenty-eight days of antibiotic therapy, the patient left the hospital against medical advice. By the time of discharge, the patient's leukocyte and C-reactive protein levels had normalized.
|
pmc-6165604-1
|
A 48-year-old healthy male presented with a hemoglobin level of 21 mg/dl and an elevated hematocrit (63%). The patient had an increased hematocrit (64%) 12 years ago, leading to clinical suspicion of polycythemia, although the patient was quickly lost to follow-up. At that time, JAK2 mutational testing was negative. At the current presentation, the patient reported fatigue, headache, blurred vision, and excessive sweating. He declined both a history of living at high altitude and smoking. Exogenous EPO use was also excluded. Past medical history includes atrioventricular block requiring pacemaker insertion, hypertension controlled with lisinopril, mild depression managed with citalopram, and erectile dysfunction treated with sildenafil. On physical examination, the patient's vital signs were as follows: blood pressure: 140/100 mmHg; heart rate: 97; respiratory rate: 19 (with excessive redness in the face); BMI: 25.85 kg/m2; SpO2: 100%.
WBC: 3.6 x 109/L HGB: 19.4 g/dL Hct: 63 % MCV: 85 fl/cell MCH: 26 pg MCHC: 31 g/dL RDW: 25.7 % PLT: 132 x 109/L Creatinine: 1.2 mg/dl (normal range: 0.6-1.2) Carboxyhemoglobin: 1.5% (normal range: 0-1.4%) EPO: 687 mU/ml (normal range: < 29.5)
CT scans of the chest and abdomen showed no evidence of malignancy, while MRI of the head was negative. Subsequent genetic/biochemical testing ruled out congenital polycythemias.
Due to the polycythemia having no obvious etiology, a bone marrow aspiration and biopsy were performed (Figures and ). The core biopsy showing a markedly decreased myeloid: erythroid ratio with CD71-positive erythroid precursors comprising ~80% of marrow cellularity () and MPO-positive myeloid cells comprising < 10% of cells (). Surprisingly, the touch prep revealed increased plasma cells (16%, ), and CD138 immunohistochemical staining highlighted the increased plasma cells arranged in clusters in the core biopsy (~15% of cellularity, ). Flow cytometry demonstrated the bone marrow aspirate contained a monotypic lambda-restricted CD38/CD138-positive plasma cell population (). Karyotype analysis of the aspirate revealed a normal male 46,XY karyotype, while FISH revealed an IGH/CCND1 rearrangement (t(11;14)), solidifying a diagnosis of plasma cell myeloma. Subsequent serum studies showed increased serum lambda light chains (41.58 mg/dl; normal range: 0.57-2.63 mg/dl) and a kappa: lambda ratio of 0.02. Immunofixation revealed an IgD lambda paraprotein that was too small to quantitate.
The patient was phlebotomized until Hct dropped below 50, which led to the resolution of symptoms. The patient has not been treated for the smouldering PCM.
|
pmc-6165612-1
|
The patient was a 71-year-old male with metastatic CRPC. At age of 62, he presented with a Gleason score 4+4 adenocarcinoma (cT3aN0M0) and was treated with ADT plus external beam radiation therapy following radical prostatectomy. At age 67, he was diagnosed with CRPC, after PSA levels increased to 11.8 ng/ml and received docetaxel 70 mg/m2 with prednisone 10 mg daily. After 16 cycles of docetaxel, the patient presented with biochemical failure, indicated by an elevated PSA level of 40.2 ng/ml. At age 71, abiraterone was given at the standard dose of 1000 mg once daily with prednisone 5 mg twice daily. Two weeks after treatment with abiraterone was initiated, the patient was transferred to Kinki University Hospital, Osaka, Japan, with a chief complaint of convulsive seizures. His blood pressure level was 90/65 and no abnormalities were noted on brain CT. Routine laboratory and endocrinology tests revealed mild liver dysfunction (AST 57 IU/L, ALT 68 IU/L) and decreased levels of potassium 2.1 mEq/l and cortisol 3.0pg/ml (). The levels of serum potassium before abiraterone therapy were 4.5mEq/l. We determined that the convulsive seizure occurred as a result of hypokalemia associated with abiraterone therapy. He received potassium supplementation and increased the dose of prednisone to 25 mg/d following discontinuation of abiraterone. Furthermore, furosemide, which was used for a prolonged period because of protracted lower extremity edema, was also interrupted. Seven days after the supplementation therapy, the levels of serum potassium and plasma cortisol were normalized (5.0 mEq/l and 7.5 pg/ml, respectively). He was discharged two weeks after being admitted and was prescribed oral prednisone (20 mg/d).
|
pmc-6165612-2
|
The patient was a 68-year-old male with metastatic CRPC. At age of 66, he presented with a Gleason score 5+4 adenocarcinoma (cT4N1M1) and was treated with MAB. After 11 months of treatment with MAB, the patient presented a biochemical failure, revealed by an increased PSA value to 10.2 ng/ml. He received docetaxel 70mg/m2 with prednisone 10mg daily. However, the treatment was interrupted after 10 months because of severe general fatigue and abiraterone 1000 mg/d with prednisone 5 mg twice daily was initiated. One month after treatment with abiraterone, the patient consulted our hospital with a chief complaint of severe lethargy. His blood pressure was 110/73 and laboratory and endocrinology findings revealed decreased levels of potassium 1.7 mEq/l and cortisol 2.9 pg/ml and elevated levels of ACTH 61.4 pg/ml (Figures and ). Plasma levels of aldosterone were within normal range. The serum level of potassium before abiraterone therapy was 3.2 mEq/l. We established that severe lethargic was caused by hypokalemia associated with abiraterone. This patient also received furosemide for the treatment of chronic heart failure. He received potassium supplementation and increase in prednisone (25 mg daily) following withdrawal of abiraterone and furosemide. Seven days after potassium supplementation therapy, the levels of plasma ACTH and serum potassium were all normalized; however cortisol was still at reference value or lower. At 14 days, plasma cortisol was also normalized and at 20 days after being admitted, the patient was discharged with the use of oral prednisone, 20 mg daily.
|
pmc-6165619-1
|
An 18-year-old male with no past medical history presented as an outside hospital transfer for acute respiratory failure. Approximately 2 months before, he began to have a cough associated with intermittent hemoptysis. He was evaluated at an urgent care clinic and received antibiotics without resolution of his symptoms. At that time, his renal function and urinalysis were within the normal limits. He denied history of joint pain or subjective joint swelling. Dyspnea progressed, and he was taken to his local hospital. On transfer, patient was intubated and on mechanical ventilation.
On physical examination, he was intubated and sedated. Lungs were with coarse breath sound bilaterally. There was bilateral swelling of his knees.
Laboratory studies showed hemoglobin of 8.4 g/dL, WBC count of 5.6/L with a lymphopenia, platelet count of 126/L, creatinine of 1.6 mg/dL, INR of 1, and PTT of 26.6 seconds. Urinalysis was with 41 red blood cells, and protein to creatinine ratio was 0.94. Anti-nuclear antibody (ANA) was greater than 12, anti-dsDNA was 507 IU/mL, C3 was 63 mg/dL, C4 was 8 mg/dL, and anti-cardiolipin IgM was 15.8 mpl.
His anti-smith,anti-MPO,anti-PR3,anti-cardiolipin IgG, anti-beta 2 glycoproteins IgG/IgM, anti-GBM, lupus anticoagulant, and cryoglobulins were negative.
Blood and sputum cultures were negative. Chest X-ray showed bibasilar airspace disease (Figure ). Bronchoalveolar lavage showed progressively hemorrhagic aliquots (Figure ).
He was treated with pulse dose methylprednisolone for three days, one dose of intravenous cyclophosphamide, and five days of plasmapheresis. Shortly after, he was extubated and weaned to room air. His hemoptysis resolved with stabilization of his hemoglobin. He was discharged in the stable condition.
|
pmc-6165620-1
|
A 46-year-old previously healthy female developed an insidious onset severe persistent headache, most prominent in the occipital region lasting for 10 days. Six days after the onset, she experienced dysarthria and a difficulty in moving her tongue within the mouth with a difficulty in eating and drinking. She did not complain of nasal regurgitation of food or nasal quality of speech. After admission, she was found to have a high-grade fever. She was otherwise healthy and denied symptoms of cough, decreased appetite, weight loss, or past history of tuberculosis. On admission, she was found to be ill with elicitable neck stiffness. Neurological examination revealed bilateral hypoglossal nerve palsy with marked tongue atrophy, more prominent in the left side () with tongue fasciculations and without other cranial nerve palsies or pyramidal weakness. Her eye movements were saccadic with a broad-based ataxic gait without other signs of cerebellar involvement.
Her blood tests revealed a haemoglobin of 12.5g/dl with a neutrophil leukocytosis (19,000/µL; 92.2% of neutrophils) with elevated ESR (100 1st Hr) and CRP (195 u/L). Her blood cultures were negative. Noncontrast CT brain did not reveal any abnormality. Cerebrospinal fluid (CSF) biochemistry revealed significant elevation of protein (111 mg/dL) with 59 polymorphs and 8 lymphocytes per cubic millimetre with reduced CSF glucose (29 mg/dL). CSF for GeneXpert for tuberculosis and staining for acid-fast bacillus (AFB) and fungal and atypical cells were negative. Pyogenic, mycobacterial, and fungal CSF cultures were negative and CSF for Meningococcus, Haemophilus, and Pneumococcus antigens were also negative. Her chest radiograph did not reveal any changes suggestive of pulmonary tuberculosis or sarcoidosis. Syphilis (VDRL & THPA), HIV serology, and autoimmune markers for vasculitis (rheumatoid factor, ANA (IF), and p & c-ANCA) were negative.
We initiated her on empirical treatment as for pyogenic meningitis with ceftriaxone and vancomycin for which she had a gradual improvement of general status with improvement of fever, meningism, gaze, and gait abnormalities while tongue weakness and atrophy persisted. Since we considered tuberculous meningitis as a possibility, we deferred treatment with steroids. Her rapid recovery in the absence of steroids or antituberculous drugs further supported our presumed diagnosis of pyogenic meningitis. Subsequently, she underwent MRI of brain and brainstem, which revealed a posteromedial infarction in the lower part of the medulla oblongata without leptomeningeal enhancement and did not show a significant cerebral oedema (). At the end of three weeks of antibiotics, inflammatory markers and repeat CSF analysis reached normal levels. After discharge, we reviewed her at one month and three & six months and she was free of fever with good general condition and had normal inflammatory markers. However, she had persistent tongue atrophy with difficult speech from which she was gradually recovering with the help of physiotherapy.
|
pmc-6165621-1
|
A 60-year-old female with osteoarthritis of the right knee underwent Mako/Stryker robotic-assisted total knee arthroplasty. The patient's past medical history consisted of hypertension. No medical conditions predisposing the patient to a fracture were noted. A midvastus approach was used, and trackers were placed with unicortically inserted pins in the tibial shaft and femoral shaft for the robotic tracking system. The operation was performed without complication, and the tracking pins were removed manually at the conclusion of the case. Postoperative radiology reports noted proper positioning of prosthesis with no acute fracture.
Physical therapy was begun on postoperative day 1 with partial weight bearing with a front wheel walker with PT assistance and was able to walk the entire hospital corridor two times. Patient was discharged on postoperative day 2 with outpatient physical therapy arrangements. Patient had a satisfactory postoperative course.
The patient had an uncomplicated recovery until 6 weeks postoperatively when she was at work and sustained a ground level fall due to acute pain of the right thigh and her leg “giving out.” Patient was unable to bear weight on the extremity. She presented to the emergency department where she was found to have a complete, oblique fracture through the midshaft of the right femur (). On radiography, the femoral tracking pin site is clearly visible at the location of the fracture in the distal segment (). The patient was taken to the OR and underwent intramedullary nailing of the right femur with retention of right knee prosthesis hardware (). CT scan of right lower extremity confirmed evidence of a fracture through the prior surgery pin tracks. Following intramedullary nail placement, the patient had a normal postoperative course. Final films at 6 months showed a healed fracture with stable orthopedic hardware (). At this time, the patient was pain free, ambulating without issue, and elected to proceed with an as needed follow-up.
|
pmc-6165622-1
|
A 74-year-old male complained of dysphagia and abdominal pain lasting for two months. Esophagogastroduodenoscopy revealed esophageal candida infection that was treated with nystatin, a drug that has not been reported as causing hepatic injury. He had a history of arterial hypertension, prostatic hypertrophy, yet unspecified myopathy causing walking-disabilities for 40 years, and diabetes mellitus type 2 diagnosed four years prior to debut of the gastrointestinal symptoms. For the past 55 years he had smoked 20 cigarettes a day and had an alcohol consumption of 21 units (252 g) a week. Four and a half and one and a half years earlier, he had undergone surgical removal of a malignant melanoma Clark's level 2 from his left cheek and a basal cell carcinoma from his back. Two months earlier, a benign inguinal lymph node as well as nine colonic hyperplastic polyps had been removed. The lymph node was detected by physical examination as part of the follow-up program for malignant melanoma and was also seen at positron-emission tomography (PET).
After treatment of the esophageal infection, his condition got worse and he developed jaundice as well as anemia. Dysphagia and abdominal pain continued and his appetite decreased. Furthermore, he developed weight loss, light-colored stools, dark-colored urine, diarrhea, and fatigue. An abdominal ultrasound showed gallbladder sludge, a poorly outlined and hypoechoic pancreas, and a dilated common bile duct, 8.7 mm in diameter. These findings aroused suspicion of gallstone-related cholecystitis. Additionally, abdominal contrast-enhanced computed tomography (c-CT) showed that the CBD had a diameter of 11 mm, intrahepatic cholestasis with stenosis at the hepatic duct bifurcation, a liver cyst located to segment 8, a right-sided renal tumor classified as Bosniak 3, pancreatic calcifications, and a presumed benign cyst located to the pancreatic neck. Serological tests revealed C-reactive protein (CRP) 79 mg/L [< 6 mg/L], hemoglobin 6.6 mmol/L [8,3-10,5 mmol/L], elevated alanine aminotransferase (ALT) 164 U/L [10-70 U/L], bilirubin 252 μmol/L [5-25 μmol/L], and alkaline phosphatase (AP) 500 U/L [35-105 U/L)]. The amylase was not elevated. The elevation of CRP was due to a urinary tract infection, and E. coli was isolated from the peripheral blood. He was treated with antibiotics, whereafter the CRP normalized and the hemoglobin almost normalized (7.4 mmol/L). After two failed attempts of endoscopic retrograde cholangiopancreaticography (ERCP), a magnetic resonance cholangiopancreaticography (MRCP) revealed a normal main pancreatic duct, intrahepatic cholestasis, and bile duct changes suspicious of a bile duct carcinoma Bismuth-Corlette type IV in the hilar region, involving both the right and left hepatic bile ducts. By magnetic resonance imaging (MRI), no hepatic tumors or pseudotumors but a benign cyst were observed ().
One month later, an ERCP showed multiple strictures of the small intrahepatic bile ducts in several liver segments, dilation of the CBD (12 mm), and a stenosis of the bifurcation (). Unfortunately, it was not possible to perform ERCP guided biopsy, for technical reasons. A stent was placed, and one week later, liver enzymes had improved: ALT 56 U/L, bilirubin 134 μmol/L, and AP 343 U/L. Serum cancer associated antigen 19-9 (CA 19-9) was strongly elevated to 3003 kU/L [0-37 kU/L] but dropped to 165 kU/L after stent placement. Hereafter, either a bile duct carcinoma or PSC was suspected. Bile duct brush cytology revealed inflammation and atypical cells. Additional blood tests showed strongly elevated IgG4 (12.9 g/L, [0.052-1.40 g/L]), elevated IgG (25.69 g/L, [6.1-15.7 g/L]), negative cytoplasmic and perinuclear neutrophil cytoplasmic antibodies (c-ANCA and p-ANCA), and positive IgM rheumatoid factor. Serologic markers concerning viral hepatitis as well as anti-smooth-muscle antibodies, anti-liver-kidney microsome type 1 (LKM1) antibodies, anti-mitochondrial antibodies (AMA), glomerular basement membrane antibodies (GBM), liver cytosol specific antibody type 1 (anti-LC1), and anti-nuclear antibodies were all negative. He had a normal glomerular filtration rate (GFR).
To further investigate the possibility of an autoimmune etiology, particularly of IgG4-SC, a liver CNB was obtained (). The liver biopsy had a length of 60 mm and contained 32 portal tracts. Twenty-four of the portal tracts showed chronic (mainly lymphoplasmacytic) inflammation, some of them with weak or moderate interphase activity. A portal-based, expansile inflammatory nodule (IN) measuring 6 mm in largest dimension, leading to expansion of a portal tract due to storiform fibrosis and lymphoplasmacytic infiltration, was identified (Figures and ). Numerous myofibroblasts, immunohistochemically positive for smooth-muscle antigen, were observed in the IN (data not shown). No accumulation of neutrophilic granulocytes was found, and granulomas and multinucleated giant cells were lacking. In the bile duct located inside the IN, strong infiltration with lymphocytes and plasma cells was observed (). The bile duct mucosa was intact, without erosion or ulceration. The epithelium was mainly monolayered, but focally with slight hyperplasia. The epithelial cells were cylindric, and only focally slight variation of nuclear size was observed. Additionally, at some sites, a notable degree of obstructive cholestasis was found. Obliterative phlebitis was identified (Figures and ) and, in addition, venolitis. Immunohistochemically, there was diffuse infiltration with IgG4-positive plasma cells (). Hot spots revealing 339 IgG4-positive and 468 IgG-positive plasma cells per high power field (HPF, 0.2 mm2) were found, corresponding to an IgG4/IgG ratio of 72% (Figures and ). Moreover, microscopy showed several portal tracts with moderate periductal fibrosis and inflammatory infiltrates dominated by lymphocytes and plasma cells and associated with eosinophilic granulocytes (in hot spots up to 13 /HPF). In the light of these findings, the diagnosis of IgG4-SC involving the extra- and intrahepatic small bile ducts was suggested. At this time, it was speculated whether the patient also had IgG4-positive autoimmune pancreatitis (AIP) type 1, another manifestation of IgG4-RD that is often associated with IgG4-SC. However, the main pancreatic duct was unremarkable by MRCP, the amylase was not elevated, no focal or diffuse enlargement of the pancreas or delayed enhancement was found at c-CT, and calcifications and cysts are usually not a feature of AIP. Hence, the International Consensus Diagnostic Criteria (ICDC) for AIP were not fulfilled []. Other lesions frequently associated with IgG4-SC are IgG4-related retroperitoneal fibrosis and IgG4-related thyroiditis, but these manifestations were not present in our patient [, ].
The inguinal lymph node that was surgically removed two months before debut of the gastrointestinal symptoms was initially classified as reactive with nodular lymphoid hyperplasia and smaller areas with nonnecrotic granulomatous inflammation. The lymph node was retrieved from the archive and showed expanded interfollicular zones as well as follicular hyperplasia with activated germinal centers (). Interfollicular zones as well as follicles were infiltrated by an increased number of plasma cells (). In several foci, the germinal centers were penetrated by small venules. Immunohistochemically, hot spots revealing 593 IgG4-positive and 646 IgG-positive plasma cells per high power field (HPF, 0.197-0.199 mm2) were found, corresponding to an IgG4/IgG ratio of 92% (Figures and ). Based on the above, it was assumed that the patient had IgG4-related lymphadenopathy associated with IgG4-SC. Interestingly, there were a few smaller areas with a characteristic granulomatous inflammation. These granulomatous foci were arranged in a ring-like fashion around lymphoid follicles, a feature also called perifollicular granuloma (). Epitheloid granulomas usually make a diagnosis of IgG4-RD unlikely []. However, perifollicular granulomas are an exception, as they have been reported in a number of cases of IgG4-related lymphadenopathy [–]. shows that the perifollicular granulomas were accompanied by numerous IgG4-positive cells. Of note, perifollicular granulomas are not specific for IgG4-related lymphadenopathy and can also be observed in, for example, nodular lymphocyte predominance Hodgkin lymphoma or reactive lymph nodes of unknown etiology [].
The patient began combined treatment with a daily dose of 100 mg azathioprine and 37.5 mg prednisolone. Within one month, the daily dose of prednisolone was tapered to 12.5 mg. Liver enzymes and AP decreased further after initiation of medical treatment. The dose of prednisolone was whatsoever not tapered continuously due to his muscle disease that seemed to improve due to steroids. The patient stopped with prednisolone 16 months after initiation of the immunosuppressive treatment and now, 26 months later, he is stable without recurrence, taking 150 mg azathioprine daily. However, at present, it is considered to increase his insulin dose, as the blood glucose levels are suboptimal. Unfortunately, no follow-up imaging was performed. Instead, AP, ALT, and immunoglobulins were checked regularly and are currently normal.
|
pmc-6165623-1
|
A 20-year-old woman, gravida 7 para 0060, at 14+3/7 weeks gestation by 8-week ultrasonography with history of depression and bipolar disorder presented to the emergency department (ED) complaining of unremitting nausea and vomiting, exacerbated by oral intake, for the past several weeks. She also reported intermittent hematemesis, epigastric abdominal pain, and mild diarrhea. However, she denied headache, fever, chills, chest pain, shortness of breath, palpitations, or dizziness. She also denied vaginal bleeding and discharge, vaginal bleeding, leakage of fluid, contractions, suprapubic or pelvic pain. She was given IV hydration, ondansetron, famotidine, and metoclopramide but continued to vomit without visible blood. She had mild hypokalemia, which was replaced with intravenous (IV) and oral potassium. She slowly improved throughout her hospital course and was able to tolerate oral intake by hospital day 6. The patient had a two-year history of multiple ED visits for the same issue, for which she was diagnosed with hyperemesis gravidarum and managed with hydration and antiemetics.
During the following month, the patient returned several times with the same complaints. She was treated again with IV hydration and antiemetics (oral ondansetron and promethazine suppositories), which resolved her symptoms. She was instructed to continue her home doxylamine succinate-pyridoxine hydrochloride and prescribed ondansetron and promethazine prophylactically.
At 22+4/7 weeks gestation, the patient presented to labor and delivery again with complaint of nausea and vomiting. She reported that she remained free of symptoms for approximately a month with home medications but began vomiting again with inability to keep fluids or solids down for the past 12 hours following consumption of contaminated food. She denied any fever, chills, diarrhea, headache, or blurred vision. She reported good fetal movement and no vaginal bleeding or discharge or contractions. While on the labor and delivery, she continued to vomit with antiemetics (IV ondansetron and promethazine). On the hospital day 2, patient was found in the shower and reported that warm showers are the only relieving factor for nausea and vomiting—concerning for CHS. Urine drug screening (UDS) was performed and was positive for cannabinoids. The patient was informed that this was possibly related to her cannabis exposure. She remained abstinent throughout the hospital stay and was continued on IV fluids and antiemetics. On the hospital day 3, she noted vast improvement and was able to tolerate regular diet. Patient was discharged home with promethazine, ondansetron, and doxylamine succinate-pyridoxine hydrochloride. Patient was counseled on completely discontinuing all exposure to cannabis and voiced understanding. UDS remained negative at subsequent prenatal visits.
At 40+1/7 weeks gestation, the patient delivered vaginally a live female infant weighing 3.19 kg with APGAR score of 8/9 without any complications. The mother and baby were discharged home on the second postpartum day.
|
pmc-6165624-1
|
A 71-year-old African American male with past medical history significant for liver cirrhosis secondary to chronic hepatitis C infection status after orthotopic liver transplantation, end-stage renal disease secondary to hypertensive nephropathy, peripheral vascular disease status after right above-knee amputation, and seizure disorder presented to the emergency department with altered mental status. Prior to change in mental status, the patient was fully alert and oriented. Altered mental status was described as psychomotor retardation and absence of verbal response to questions. Nursing home records showed that the patient had a recent onset of shingles and was started on valacyclovir by his primary care physician three days prior to presentation. He missed his usual hemodialysis session on the day of presentation in view of change in mental status. There was no reported fever, headache, or convulsions. There was no history of illicit drug or alcohol use. Except for valacyclovir, he has not been receiving any new prescription or over-the-counter medication.
The patient was afebrile on admission with the normal vital signs, except for elevated blood pressure of 176/85 mmHg. On neurologic examination, the patient appeared alert and disoriented. There were no meningeal signs or focal neurologic deficits. Skin examination showed crusted vesicles on an erythematous base over the lower back in S1 dermatomal distribution, consistent with the reported history of shingles (). Cardiovascular, respiratory, and abdominal examinations were within the normal limits.
Complete blood count revealed hemoglobin concentration of 10.0 g/dL, white blood cell count of 2,770/µL, and platelet count of 201,000/µL. Liver function tests were within the normal limits, while serum blood urea nitrogen and creatinine were 37 mg/dL and 6.3 mg/dL, respectively. Serum ammonia 30.8 µmol/L (normal range: 18.0–72.0 µmol/L). There were no significant electrolyte derangements or metabolic acidosis (). CT head with and without contrast and MRI brain showed no acute intracranial abnormalities.
Further review of nursing home records showed that the patient was prescribed valacyclovir at 1 g three times a day, which is significantly higher than the recommended dose of 500 mg daily in the presence of end-stage renal disease. At this point of time, valacyclovir was discontinued. Electroencephalogram (EEG) was performed, showing generalized slowing consistent with toxic-metabolic insult (). Lumbar puncture was deferred as there was low suspicion for central nervous system infection, considering the absence of suggestive clinical features or imaging findings. The patient received two consecutive sessions of hemodialysis, and his mental status returned to baseline within three days of hospital admission. Together, these findings suggested valacyclovir neurotoxicity as the underlying etiology for the current presentation.
|
pmc-6166289-1
|
A 62-year-old Asian man with a medical history of diabetes mellitus and pancreatitis due to alcohol experienced speech disturbance. At the age of 58 years, he was treated with insulin for his diabetes mellitus. After that, his blood sugar level was well controlled by diet therapy. There were no relatives with intracranial aneurysms. His symptom was transient and had completely improved when he presented to our institution. He had no neurological abnormalities when he underwent radiological examinations. Magnetic resonance images showed no abnormality in his brain including hemorrhage or cerebral infarction. Magnetic resonance angiography (MRA) revealed a left DMCA that originated from the ICA distal to the anterior choroidal artery (Fig. ). An aneurysm at the M1/M2 junction of the DMCA was found (Fig. ). Three-dimensional computed tomographic angiography (CTA) also demonstrated the left DMCA associated with aneurysms at the M1/M2 junction and left ICA top (Fig. ). In our patient, the aneurysm was located on the DMCA, and not the main trunk of the MCA. The diameter of the DMCA was almost the same as that of the main MCA. The diameters of the DMCA and ICA top aneurysms were both less than 5 mm. Therefore, the aneurysms were not surgically treated, and periodic examinations by magnetic resonance images (MRI) and MRA were planned.
|
pmc-6166300-1
|
A 61-year-old homeless man with a past medical history significant for benign
spindle-cell thymoma presented with acute hypoxic respiratory failure. Two months
prior, he was treated for Bordetella bronchiseptica pneumonia and
empyema with intravenous (IV) antibiotics and right pleural decortication.
Evaluation during the first hospitalization was negative for HIV, hepatitis B
infection, syphilis, blastomycosis, and coccidioidomycosis. He was discharged but
was subsequently rehospitalized within 1 week with recurrent pneumonia. During this
second hospitalization, he was treated with another 2-week course of antibiotics
with some improvement in symptoms and was discharged home. Two days after being
discharged, he presented to our institute with hypoxic respiratory failure requiring
endotracheal intubation. Pertinent findings on physical examination were fever,
hypoxia, and tachycardia. Oral candidiasis was noted. Lung auscultation revealed
coarse and mechanical breath sounds bilaterally. Chest radiographic findings showed
bilateral patchy airspace opacities (). Computed tomography scan of the
chest showed a stable, large anterior mediastinal mass, multiple cavitary lesions,
and diffuse ground-glass opacities (). The patient was started on broad-spectrum IV antibiotics
with cefepime and vancomycin. Examination of the bronchoalveolar lavage revealed
B bronchiseptica and Pneumocystis jirovecii.
The patient’s antibiotics regimen was changed to piperacillin/tazobactam,
sulfamethoxazole-trimethoprim with prednisone, and fluconazole. Repeat HIV serology
was negative. Laboratory results showed panhypogammaglobulinemia and low total B-
and CD4 T-cells (). IV immunoglobulin (IG) treatment (400 mg/kg every 3-4 weeks) was
initiated. He was evaluated for possible thymectomy but was not a surgical candidate
due to his clinical condition. His clinical status continued to deteriorate, and he
subsequently suffered cardiac arrest and death.
|
pmc-6166301-1
|
A 56-year-old woman with a past medical history of scleroderma, chronic constipation,
and hypertension presented to the emergency room with generalized abdominal pain
associated with multiple episodes of vomiting. Pain was described as 5/10 in
intensity, localized in the right lower quadrant with no exacerbating or relieving
factors. Vomiting was nonbloody and nonbilious. She has also been constipated more
than usual for the past week. On presentation, vital signs were normal except for
mild tachycardia of 104. Physical examination is significant for right lower
quadrant tenderness and decreased bowel sounds. Initial blood count and basic
metabolic panel were normal but the lactic acid on presentation was 4.4 mmol/L.
A computed tomography (CT) scan revealed multiple loops of large bowel positioned
between the liver and the right diaphragm indicative of Chilaiditi syndrome, cecal
wall thickening (),
multiloculated pelvic abscess with droplets of air suggestive of peritonitis, and
segmental distension of several loops of distal small bowel concerning for ileus or
partial obstruction. The patient underwent CT-guided drainage of the pelvic abscess
with return of 600 cc of purulent material after which the patient was started on
intravenous vancomycin, piperacillin/tazobactam, and metronidazole. Over the next 2
days, drain output was increased gradually along with spike in white blood cell
count. Repeat CT scan showed worsening of the pelvic fluid collection as well as
development of new distant fluid collections in the anterior and outer left abdomen.
The patient underwent exploratory laparotomy with abdominal washout and right
hemicolectomy. Operative findings included feculent peritonitis and necrotic cecum
with perforations (). Pathology of the specimen reported moderately differentiated
adenocarcinoma with invasion into pericolonic adipose tissue. Perforation in the
cecum was likely related to a combination of factors. There was a segment of normal
intervening colonic mucosa between the nonobstructing cecal mass and the cecal
perforation. The lumen was widely patent at the time of perforation and therefore we
believe that compromised wall integrity from underlying scleroderma and luminal
compression from coexisting Chilaiditi syndrome played a role in the perforation in
the cecum. There were no tumor elements at the site of perforation on histology. The
patient was discharged with an end ileostomy on day 7 and was later seen in surgery
clinic in 2 weeks with improvement in symptoms. The patient is scheduled to
follow-up with hematology oncology.
|
pmc-6166303-1
|
A 56-year-old man, HIV serology positive for 8 years, presented to the emergency
department with progressive worsening of shortness of breath for 2 days. He
experienced shortness of breath for the past 6 months. He had bilateral leg swelling
and orthopnea in the recent months. The latest cluster definition (CD4) cells count
was 804 cells/µL, and he received antiretroviral therapy Genvoya
(elvitegravir/cobicistat/emtricitabine/tenofovir alafenamide). He was previously
diagnosed with asthma and positive IgG (immunoglobulin G) serology for hepatitis C
virus (HCV). He smoked a pack of cigarettes for 20 years and engaged in unprotected
sexual acts with men.
On examination, the patient had jugular venous distension and tachypnoea with
bilateral basal crackles heard on auscultation. He had bilateral pitting pedal edema
(grade 2) reaching the bilateral tibial tuberosity.
His respiratory symptoms worsened despite the immediate resuscitation efforts at the
emergency department, and he required a mechanical ventilator due to impending type
1 respiratory failure. The CD4 cell count at the time of admission was 467 cells/µL
with the serum HIV-1 viral load of less than 20 copies/mL. The serum HCV RNA viral
load by polymerase chain reaction assay was less than 15 IU/mL. Plain chest X-ray
showed cardiomegaly and moderate diffuse pulmonary congestion. The brain natriuretic
peptide level was 574 pg/mL on admission. The initial transesophageal echocardiogram
showed signs of a dilated right ventricle, elevated pressures, and 2 large
echodensities with one tethered to the PFO () and another to the tricuspid valve
(), which
suggested RA thrombus. The left ventricular ejection fraction was around 60% with
grade 1 diastolic dysfunction associated with a compromised left ventricular size
due to the enlarged right ventricle. The interventricular septum showed dyskinesia,
secondary to elevated right ventricular pressure and volume. A large complex
thrombus with mobile lobulations was found attached to the base of the tricuspid
valve with 27.9 × 10.8 mm dimensions. The second thrombus, with complex features,
measuring 45.8 × 19.1 mm, had crossed the PFO and protruded into the left atrium.
The protruded freely mobile, small linear segment of the echo density measured 10 ×
3 mm on the left atrial side. He received low-molecular-weight heparin (enoxaparin)
1 mg/kg to prevent further thrombosis. The qualitative cardiac troponin-I enzyme
report was negative. Computed tomography of pulmonary angiogram revealed an
eccentric nonocclusive thrombus in the proximal left lower lobar artery and
bilateral embolization in the segmental arteries associated with consolidation in
the left lower lobe with minimal pleural effusion. Venous ultrasonogram showed no
deep vein thrombosis. Transesophageal echocardiogram on the 13th day of admission
showed a decreased size of the RA thrombus with features suggestive of ruptured
thrombus and distal embolization (). The RA thrombus tethered to the tricuspid valve of 3.4 × 1.5
mm size and PFO thrombus of the size 8.1 × 3.1 mm were found to be reduced in size.
During the hospital course there was no clinical evidence of any further
recognizable embolization. Further hospital stay for the patient was notable for
continued intensive care, tracheostomy, and percutaneous endoscopic gastrostomy
procedures. Goals of care were discussed with the family, and he was transitioned
from critical care to long-term care facility.
|
pmc-6166305-1
|
A 24-year-old man with a history of unspecified intermittent arrhythmia presented
with sudden-onset palpitations, sharp left-sided chest pain, left arm numbness,
shortness of breath, lightheadedness, and a feeling of impending loss of
consciousness. He described similar past episodes now occurring more frequently
lasting several minutes and abating with deep breaths and “clenching up” the chest.
The prehospital electrocardiogram (ECG) strip revealed an irregular wide-complex
tachycardia (WCT) with varying QRS width and a ventricular rate up to 300 beats per
minute (bpm). The upstroke of some QRS complexes appeared slurred ().
On arrival to the emergency department, his vitals included a heart rate greater than
200 bpm and a systolic blood pressure of 130 mm Hg. His oxygen saturation was 100%
on room air. On examination, the patient was alert and oriented with an intact
neurologic examination. His lungs were clear to auscultation bilaterally without
wheezes, rhonchi, or rales. The cardiovascular examination was notable for
tachycardia with an irregularly irregular rhythm. There were no extra heart sounds,
including murmurs, rubs, and gallops. The abdomen was soft, nontender, and
nondistended, and the extremities were warm and well perfused. He had strong
palpable pulses in his hands and feet, and there was no lower extremity edema. The
patient had no prior diagnosis of structural heart disease. He took no medications
and had no known drug allergies. Both his family and social history were
noncontributory to his current presentation.
The first 12-lead ECG acquired in the emergency department showed irregular
tachycardia with polymorphic QRS complexes of varying width, along with several
narrow normal-appearing complexes (). The heart rate demonstrated variable preexcitation with
rates up to 300 bpm. Several wide QRS complexes in the lateral leads exhibited the
slurred upstroke phase, which was not the case for the narrow complexes. The patient
was fully awake with systolic blood pressures in 130s mm Hg. Because of the initial
interpretation of the rhythm as ventricular tachycardia, the patient was given 2
rounds of amiodarone 150 mg intravenous without effect. He was then cardioverted
with 100J, synchronized. The post-cardioversion ECG showed sinus tachycardia with
the preexcitation pattern of positive delta waves in the anterolateral leads (I,
aVL, and V2-6; ).
The patient remained in sinus rhythm and was admitted to a medicine floor. The
following day, he underwent successful radiofrequency ablation of a right
posteroseptal accessory pathway. Subsequent ECG strips showed no delta waves but
revealed peaked upright T waves in leads I, aVL, and V2-6, and large inverted T
waves in leads III and aVF (). The troponin level peaked at 0.53 ng/mL, and was undetectable within
12 hours. An echocardiogram was unrevealing. The patient was discharged after 3 days
in stable condition remaining in normal sinus rhythm.
|
pmc-6166368-1
|
A 73-year-old female patient with type 2 DM was referred to our clinic for low sugar episodes. She had been receiving insulin therapy for 7 years. In medical history liver transplantation was performed 20 months ago due to end stage liver disease caused by HCV infection genotype 1b. She had a body mass index (BMI) of 31.9 kg/m2 and was using intensive subcutaneous insulin injections four times daily: insulin glargine 24 U at bedtime and insulin aspart 16U three times a day before each meal. While she was still on tacrolimus 2.5 mg and mycophenolate mofetil 1000 mg daily, after liver transplantation for nearly 20 months, she received direct acting antiviral agents (DAAs) including fix dose combination ledipasvir-sofosbuvir (90 mg-400 mg) plus ribavirin, for 24 weeks, and achieved virologic response. After DAAs and ribavirin treatment was completed, she began to experience severe hypoglycemia and therefore the insulin aspart was stopped. Since hypoglycemia persists, she also discontinued insulin glargine 1 week ago. Her vital signs were normal and physical examination was unremarkable. Biochemical analysis showed fasting plasma glucose (FG) of 105 mg/dl and postprandial glucose of 200 mg/dl. Surprisingly, glycosylated hemoglobin A1c (HbA1c) was 4.8% and c-peptide was 3.17 ng/ml. Liver and renal function test results were in the normal reference range. Home blood glucose measurements also showed a normal course of glucose and the patient was followed only by dietary regulation.
|
pmc-6166370-1
|
Breast cancer was diagnosed in this 60-year-old woman in July 2010. Initial treatment consisted of surgery, radiotherapy, and chemotherapy. AI therapy with letrozole was started in February 2011 for 5 years. She had no other risk factors for osteoporosis. A DXA performed in March 2011 revealed osteoporosis. BMD T-scores were −2.9 at the lumbar spine and −1.9 at the total hip. Vertebral morphometry confirmed the absence of fractures. The 10-year probability of major osteoporotic fractures assessed by FRAX® was 13%. A treatment with 60 mg denosumab every 6 months and adequate daily calcium and vitamin D supplementation started in March 2011. She received 12 half-yearly injections of denosumab, the last one in August 2016. Letrozole treatment ended in November 2016. A DXA performed in November 2016 showed no more osteoporosis. The lumbar spine and total hip T-score values were −1.7 SD (+18%) and −1.4 SD (+8%), respectively. Vertebral morphometry confirmed the absence of fractures. CTX (fasting blood sample in the early morning, normal range for premenopausal women: 25–573 ng/l) were measured at 33 ng/l in March 2017, 7 months after last denosumab injection. To prevent the high-turnover bone loss associated with denosumab discontinuation, an antiresorptive treatment was proposed. She refused bisphosphonates for fear of side effects. Raloxifene 60 mg daily was accepted and started in March 2017. In April 2017, CTX values were low at 100 ng/l. The patient scrupulously took her treatment. By mid-July, she experienced spontaneous low back pain. Thoracolumbar MRI performed in August revealed two D11 and L5 fractures with medullary edema. CTX, measured in August 2017, were extremely high at 2070 ng/l (). To rapidly reduce the increased bone turnover, an injection of denosumab was given at the time of fracture diagnosis.
|
pmc-6166387-1
|
A 29-year-old man, a former semiprofessional handball player, had a traumatic rupture of the proximal side of the patellar tendon of the left knee in 2016 during a match. No associated disease was reported. Primary surgery was performed in another health facility through a median approach using 2 anchors for tendon repair protected by an additional ipsilateral semitendinosus graft (patellar and tibial tunnels). The patient came to our health facility following severe functional deficits after an iterative rupture without having experienced any new trauma 13 months after the initial surgery. The iterative rupture of the knee extensor mechanism was also an iatrogenic fracture of the transverse patellar tunnel (). Clinically, walking was not possible, there was a lack of active extension and hemarthrosis with pain. There were no scar problems, no signs of deep or superficial infection, and no cutaneous wound. A huge gap was clinically observed between the patella and the patellar tendon. Considering the patient's age, his preinjury sports level, and lack of active extension, the decision was made to perform a revision procedure. An artificial ligament (LARS®) and two adjustable loops, free ends of the PULLUP® BTB (SBM SAS, France), were used to enhance the patellar tendon repair.
A preoperative lateral standard X-ray was taken of the contralateral knee at 30° of flexion to measure the Caton-Deschamps index and patellar height (). The patient was placed in a supine position under general anesthesia with a tourniquet at the proximal part of the thigh. The previous median approach was used. The patellar fracture and the site of the previous rupture were cleaned to remove fibrous tissue and hematoma. Previous anchors were left in place.
The first step was to place the ligament advanced reinforcement system (LARS® polyethylene terephthalate fibers 6 mm ref. L030307 ACFAR 32 CK). A new transverse tunnel was drilled in the tibia, distally to the tibial tunnel of the initial surgery, with a 5.5 mm drill, and the LARS® was inserted in the tibial tunnel. The artificial ligament was then passed through the lateral retinaculum and above the patella at the junction with the quadriceps tendon in a Pulvertaft manner and through the medial retinaculum to return to its origin (). Two longitudinal tunnels were drilled in the patella using a 2.4 mm drill. The loops of a PULLUP® BTB (the plate was removed from the device) were first passed into the patellar tendon and then into the patella through the two longitudinal tunnels using a shuttle relay. Next, the free ends were pulled down in the opposite patellar tunnels. At the proximal side of the reconstruction, the two free ends of the PULLUP® BTB were inserted into each braid to close the system (Figures and ). The distal and medial ends of the LARS® were tightened with a clamp in order to restore normal patellar height and secured with 2 staples. Then, the 2 PULLUP® BTB loops were tightened (). The previous tendon rupture was closed and reinforced with separate X-knots using absorbable sutures. The skin was closed. The knee was placed in an articulated brace with compressive ice therapy for 24 hours, and a postoperative X-ray was taken ().
For postoperative care, weight bearing was not allowed for 6 weeks and mobilization of the knee was immediately started between 0 and 45° for 3 weeks then from 0 to 90° from 3 to 6 weeks. No complication was reported during the postoperative period. At 3 months, the patient was pain free and could walk without the aid of crutches. He was able to resume handball practice at 6 months after a control MRI. At one-year follow-up, he was able to play handball with complete knee extension strength (compared with the contralateral side) and was able to return to a semiprofessional level. The range of motion of the knee was 0-0-130°. The MRI at 1 year showed complete healing of the patellar tendon and the bone ().
|
pmc-6166900-1
|
A 68-year-old Caucasian male with a history of hypertension, hypercholesterolemia, coronary artery disease status post five vessel coronary artery bypass grafting presented to the emergency room with chest pain for four days which radiated to his jaw and along the left arm (Figure ).
Electrocardiogram (EKG) showed old Q waves in inferior leads and new ST depression in leads I, AVL, V5 and V6 and troponin peaked up to 15.8. At the time of presentation, the patient was also short of breath with initial chest plain, a radiograph (X-ray) showing pulmonary edema is depicted (Figures and 3).
The patient was electively intubated before urgent cardiac catheterization, which was performed within 12 hours of patient’s arrival, and revealed 100% occlusion in right coronary, left main coronary, and the proximal portion of the circumflex artery as well as diffuse disease of the grafted vessels to circumflex. Furthermore, during catheterization, the patient was also noted to have hemodynamic compromise with blood pressure observed to be 86/63 mmHg and a heart rate of 115 beats per minute, which prompted emergency echocardiography (ECHO) showing partial disruption of medial papillary muscle with severely flail posterior MV leaflet and severe MR. He subsequently underwent venoarterial extracorporeal membrane oxygenation (ECMO) pump placement by cardiothoracic surgery for hemodynamic support accessing through the right common femoral artery and right femoral vein (Figure ).
Heart team specialists thus discussed the possibility of performing an MV clipping, and it was decided in favor of proceeding with the transcatheter procedure as the patient had a history of median sternotomy and previous bypass surgery. The patient remained intubated and was taken to cath lab on the fifth day of hospitalization. The femoral vein was accessed using ultrasound guidance, and the transseptal puncture was performed using intraoperative transesophageal echocardiography. This was then followed by prepping the MitraClip using standard technique and positioning it across the interatrial septum and above the atrial valve. For this procedure, two clips were used, one was on the posterior segment P2 and just beside it to cinch up P2 and A2, and the second one for P2 and P1 laterally. Once the resulting MR reduction was regarded as adequate, clips were deployed (Figures and 6).
The only complication noted was the transient Wenckebach heart block for which the patient subsequently received a pacemaker later in the hospital course. Thus, the patient underwent two successful MitraClip placements, and the severity of MR went down from 4+ to no more than 1+. The patient’s left ventricular function improved postprocedure and ECMO was subsequently followed by extubation later on. Repeat ECHO on third postprocedure day showed mild MR and normal left ventricular ejection fraction. At the 30th day, the patient was followed up in the clinic and repeat ECHO at that time showed mild MR only.
|
pmc-6166901-1
|
Mr. A, a 26-year-old English-speaking immigrant male from Afghanistan, domiciled with his mother and sister at a private residence, had an extensive history of polysubstance use (cannabis, nicotine, alcohol), and was brought into the emergency room (ER) by his family due to agitation, aggression, and a verbal altercation with his neighbors.
Upon initial interview at the ER, he was found to be grossly disorganized, unresponsive to verbal redirection, constantly argumentative, and resisting a full interview and evaluation. He admitted to smoking an unknown amount of marijuana and consuming one pill of ecstasy approximately 12–15 hours earlier. His family reported that they had never seen him in such a state before. His sister stated that prior to the ER visit he displayed uncontrollable aggression to the point of damaging furniture in the home.
She also explained that he had left home early in the morning in a fit of rage, and she had found him on the streets several hours later, banging at the glass windows of a pharmacy with a book. He experienced an episode of psychosis with a particular fixation on a recently read fictitious character, leading to suicidal ideation. Further, the patient was confused regarding his own identity as a human or a fictitious supernatural creature. For the length of time, he went missing and his family also received a phone call from their neighbors stating that he was standing outside their house, making threatening gestures at passersby.
His sister provided more details about his past trauma history, having lived with a father who was alcohol dependent. The patient, along with his family, had been a victim of racial abuse and physical assault, leading to posttraumatic stress disorder (PTSD) and a protectionist savior complex.
In the ER he refused to provide blood and urine samples for toxicology screening, and remained largely uncooperative, displaying more aggression when counseled, punching walls without provocation, and refusing oral medications. The team administered intramuscular Haloperidol 5 mg and Lorazepam 2 mg to ensure his and the staff’s safety. After receiving these medications, he became calmer and provided the blood needed for laboratory tests. All results were within normal limits except for an elevated white blood cell count of 14 (Reference range: 4.5–11.0 x 109/L). The patient was admitted into the inpatient psychiatric unit for further stabilization. Since he was much calmer than when he came into the ER, he did not require seclusion or additional observational orders.
In the psychiatric unit, the patient started becoming aggressive again for the first three days, requiring restraints and intramuscular injections of psychotropic medications on multiple occasions. He was noted to be antagonizing other patients and displaying paranoid behavior towards peers and staff. Interviews for most of his stay were replete with psychotic content and persistent delusions about being a werewolf, while he continued to be irritable and agitated. On subsequent follow-up evaluations, he made it a point to mention almost every day that he had been duped into having himself videotaped while engaging in homosexual activity with a peer.
He was started on a daily oral dose of Paliperidone 3 mg, which was then gradually titrated by 3 mg every week to 9 mg in combination with oral Valproic acid 500 mg twice daily.
He continued to remain psychotic for a week with a minimal response on this regimen. During the second week, he gradually responded and became calmer, less defiant, sociable, and polite in his interactions. By the end of the third week, his psychotic symptoms had subsided with the exception of some delusions.
The team discussed the option of long-acting injections to uphold compliance. The patient agreed and was switched to a long-acting preparation of injectable Invega Sustenna 234 mg, lowered to 156 mg after two weeks.
Toward the end of his hospitalization, the patient revealed that he had consumed ecstasy for the first time. He also said that after taking the pill he started experiencing psychotic symptoms. He admitted to spending $5–10 per day on marijuana, which he inhaled through vaporizers. The patient was discharged with the recommendation to follow up as an outpatient at our clinic and is currently being seen on a monthly basis by an outpatient psychiatrist. His delusions of being a werewolf lasted for a total of five months and eventually subsided. He continues to receive treatment at the clinic and is currently stable.
|
pmc-6166905-1
|
A 48-year-old male patient presented with dysarthria, left-sided hemiparesis, right-sided facial paresis, dysphagia, difficulty walking, and headache for the past six months. On examination, he was well-oriented to his surroundings with the impairment of the sixth cranial nerve, i.e., uvula deviation to the left, tongue deviation to the left, plantar reflex upgoing, and positive Romberg sign. Magnetic resonance imaging scans of the brain revealed a lobulated lesion in the right cerebellopontine (CP) angle extending into the middle cranial fossa, causing compression of the cavernous sinus and brainstem. The tumor was also abutting the right internal carotid artery medially. The lesion was hyperintense on the T1-weighted (T1W) sequence (Figure ), hypointense on T2W (Figure ), and showed abnormal signal dropout on susceptibility weighted imaging (SWI) representing hemorrhage/calcification (Figure ). We performed a right subtemporal craniotomy and zygomatic osteotomy and resection of the pontotemporal space-occupying lesion. Intraoperative findings include a black-colored tumor arising from the pons and invading the temporal bone.
|
pmc-6166906-1
|
A 37-year-old man without any prior comorbid conditions presented to a secondary-care hospital with a first episode of a wide complex tachycardia of two hours duration (Figure ). Besides chest discomfort and thumping sensation, no other symptoms were noted. He was treated for VT in the emergency room. A single 1 mg/kg dose of lidocaine terminated the tachycardia. Subsequently, a 12-lead electrocardiogram (ECG) pattern was obtained essentially within the normal limits with no suggestion of preexcitation or ischemia (Figure ).
He was then referred to our center for further evaluation and underwent further testing with a normal ECG and normal serial cardiac enzymes. His coronary angiogram revealed normal coronary arteries. In view of his ECG, the differential diagnosis included a VT arising from the anterolateral peri-mitral area and an aAVRT arising from an AP in the left free wall. He then underwent an electrophysiologic study (EPS) to rule out an AP.
After femoral venous access was achieved, two quadripolar catheters were placed in the high right atrium (HRA) and right ventricle (RV). A decapolar catheter was placed in the coronary sinus (CS) from the femoral vein but could not be advanced distally enough to bracket the AP, due to the CS anatomy. The ablation catheter was positioned at the His bundle region. EPS was carried out in the usual manner. Baseline intervals were within the normal limits. Retrograde conduction with ventricular pacing was concentric and decremental. Atrial pacing revealed preexcitation at 360–380 ms initially and 290–310 ms later; this occurred briefly during Wenckebach block in the atrioventricular node (AVN) with induction of tachycardia. Effective refractory periods (ERP) of anterograde AVN and APs were <220 ms. Tachycardia was induced during atrial and ventricular pacing by atrial and ventricular extrastimulation. Tachycardia cycle length (TCL) varied from 440 to 280 ms (after warm-up) in the initial and later phases of the study. The earliest local ventricular activation during tachycardia was at the distal CS, suggesting either a VT with 1:1 retrograde atrial activation or an aAVRT involving a left free-wall AP. The tachycardia was entrained from the HRA, giving an atrium-ventricle-atrium (AVA) response (Figure ) to the cessation of entrainment, which differentiated the aAVRT from VT []. Atrial fibrillation was induced during atrial pacing and terminated spontaneously, but it did not show gross preexcitation as was noted during the tachycardia (Figure ).
A transseptal puncture was then carried out using an 8 Fr Mullins sheath. Heparin 5000 units were given intravenously after attaining left ventricle (LV) electrogram. Subsequently, heparin was administered intravenously to maintain the activated clotting time (ACT) at >250 s. Mitral annulus was mapped in the left anterior oblique (LAO) at about 30 degrees during tachycardia, as the atrial pacing cycle length (CL) that caused preexcitation would invariably induce tachycardia. At the 12 o'clock position (Figure ), the earliest ventricular signal was noted.
The fractionated component of the ventricular signal was measured 22 ms prior to the surface delta wave (Figure ). To ensure catheter stability, atrial pacing was continued at the TCL (280 ms). Radiofrequency (RF) was delivered at the spot at a temperature of 600 degree Celsius and at 50 Watt. Tachycardia terminated in the AP (Figure ) within 7 s of RF application, which was continued for 62 s. Thereafter, no tachycardia was inducible. A post-ablation AVN Wenckebach block occurred at 290 ms without the induction of tachycardia or preexcitation. Post-ablation right ventricular apical and basal pacing revealed nodal conduction.
|
pmc-6166908-1
|
The patient in this study is a 23-year-old female with ESRD secondary to hypoplastic kidneys. She received an offer from a brain dead, 9-month-old female donor with a terminal creatinine of 0.2 mg% and donor weight of 10 kilograms. The recipient-to-donor weight ratio was roughly six-to-one. After performing the standard multi-organ procurement, it was noted that the donor left renal vein was essentially dismembered from the inferior vena cava (IVC). This prohibited performing the standard en-bloc technique to transplant the kidneys. Normally, the suprarenal IVC is sutured closed, and the infrarenal IVC is spatulated at the iliac bifurcation for anastomosis to the recipient iliac vein. The pediatric kidneys were divided on the back table similar to an adult kidney procedure. The left renal vein was separated completely. The aorta was split down the middle and Carrel patches were made around the renal arteries. The right renal vein was elongated using the donor IVC in the standard fashion, and a venotomy was made on the side of the IVC for the anastomosis. The transplant then proceeded normally by exposing the right iliac vessels. Adequate exposure was achieved. The internal iliac veins were ligated and divided to elevate the external iliac vein and allow for a tension-free anastomosis. The external iliac artery was dissected to its full length, from the takeoff of the internal iliac artery to the inguinal ligament. The two kidneys were transplanted by stacking them on the same side. The left kidney was placed higher to allow the longer vein to cross over the iliac artery. The arterial anastomosis was performed using Carrel patch to the external iliac artery. The iliac veins were reconstructed in the usual fashion to the external iliac veins. Schematic diagram of the techniques is demonstrated in Figure . We used 7.0 non-absorbable monofilament stitches in a running fashion for all anastomoses. Both kidneys were reperfused simultaneously (Figure ). The ureters were then reconstructed using two separate Lich-Gregoir anastomoses to the bladder. The total cold ischemia time was 22 hours and 47 minutes. The warm ischemia time was 55 minutes. Immediate postoperative ultrasound demonstrated excellent perfusion in both kidneys (Figure ). The kidneys had immediate function with excellent postoperative urine output and no dialysis needs.
The patient’s initial post-transplant eGFR was 9 ml/min, and had a creatinine of 6.4 mg/dL and eGFR of 10 ml/min on post-operative day one. On post-operative day six, the patient was discharged with a baseline creatinine of 1.3 mg/dL. The eGFR continued to improve to 82 ml/min 30 days post-transplant, reaching a maximum eGFR of 94 ml/min at 30 weeks post-transplant. About a year later the patient had a computed tomography (CT) scan for an unrelated reason and it showed significant growth in the size of the kidneys (Figure ). The patient continues to maintain the same eGFR over two years later despite an episode of BANF IIA acute cellular rejection (ACR) and antibody-mediated rejection (AMR) a few months after the transplant. This episode of ACR/AMR was treated using rabbit antithymocyte globulin followed by total plasma exchange and intravenous immunoglobulin (IVIG) and rituximab. She required several biopsies throughout the course of this episode, which were technically easy to perform using standard ultrasound-guided technique.
|
pmc-6166908-2
|
The second patient was a 36-year-old male who had ESRD secondary to adult polycystic kidney disease. He received an offer from a 6-month-old brain dead male donor who weighed seven kilograms. The donor had a terminal creatinine of 0.4 mg%. The recipient-to-donor weight ratio was nearly ten-to-one. After the multi-organ procurement operation was performed, it was noted that the suprarenal aorta was cut too close to the right renal artery (Figure ). The upper half of the circumference of the artery had no aortic cuff. A concept similar to the one used in the first case was utilized. However, during this procedure, the right renal artery was reconstructed using interrupted 8.0 non-absorbable monofilament stitches rather than using a Carrel patch. The remainder of the operation was similar in technique to the prior scenario. The patient had an excellent post-transplant outcome and was discharged on the fourth postoperative day—with an eGFR of 42 mL/min, and eGFR of 86 mL/min nine months post-transplant. There are no vascular complications to date. The patient maintains his eGFR today—nearly two years later.
|
pmc-6166913-1
|
A two-year-old boy referred to our clinic with intractable pruritus, scaling, dry skin and generalized eczematous lesions resistant to atopic dermatitis therapy. Review of his medical record showed he was born at the 37th week of gestation after an uneventful pregnancy to healthy unrelated parents. Shortly after birth, he got treatment for desquamative skin lesions. During the following seven months the desquamation resolved, but ultimately the patient developed generalized, pruritic, erythematous lesions. He got treatment with emollients, topical steroids and tacrolimus creams for severe atopic dermatitis during the next one year. At one year of age, serum IgE levels were 486 IU/ml and 530 IU/ml, respectively. He had no family history of skin disorders. He was allergic to eggs and cow's milk. On physical examination, his skin was dry, and there were erythematous scaly patches on the abdomen, face, and extremities (Figures -).
The eczematous lesions were not typical of atopic dermatitis. The height and weight were normal. Biochemical tests and serum folate, iron, vitamin B12 and zinc levels were normal. He had dry and short scalp hair. The eyebrows and eyelashes were sparse, nails, palms and mucosal surfaces were intact.
Urinary amino acid analysis, immunoglobulins (IgA, IgG, IgM, and IgG), complements (C3, C4) and lymphocyte subset counts (CD3, CD4, CD8, CD14, CD19, CD56) were normal. Serum anti-gliadin IgA and IgG, anti-endomysium IgA, antinuclear antibody (ANA), anti-dsDNA and anti-HIV tests were negative. Thyroid hormone and thyroid autoantibodies were within the normal reference range. The patients had serum eosinophilia and high serum total IgE levels (530 IU/ml). The erythroderma, Ichthyosis linearis circumflexa, elevated IgE along atopic reactions suggest the diagnosis of NS. The patient got treatment with topical corticosteroids and skin moisturizers. The family was counseled about the diagnosis and need of genetic testing for confirmation, but they refused for genetic testing. At six-month follow-up visit, his skin lesion improved but not completely resolved. We keep a close follow-up of the patient.
|
pmc-6166916-1
|
A 59-year-old woman presented for evaluation of scalp alopecia. Her past medical history was significant for PT1cN1mi estrogen receptor (ER)+, progesterone receptor (PR)+, human epidermal growth factor receptor (HER)2+ g3 invasive ductal carcinoma of the right breast diagnosed 15 months earlier. She had been treated with bilateral lumpectomy with right-sided sentinel lymph node biopsy and started chemotherapy nine months earlier; she received pertuzumab, docetaxel, carboplatin, and trastuzumab every three weeks for six cycles and was maintained on trastuzumab 6 mg/kg every three weeks for one year. Three weeks after completing taxane chemotherapy, she began treatment with anastrozole 1 mg daily (which was switched to tamoxifen 20 mg daily due to joint pain). She was also treated with radiation therapy and is currently on neratinib 240 mg daily; neratinib is a tyrosine kinase inhibitor anticancer drug used to prevent recurrence in patients with early-stage HER2+ breast cancer who have finished at least one year of post-surgery trastuzumab therapy.
She noted hair loss beginning after her first course of systemic chemotherapy. It became more extensive throughout the remainder of her treatment. She had not experienced any regrowth of scalp hair since the completion of chemotherapy nor during her current hormonal therapy.
Cutaneous examination revealed alopecia of the scalp. The clinical presentation was most consistent with female pattern alopecia with diffuse and nearly complete hair loss on the central and vertex region with retention of hair on the occipital scalp. There was partial, diffuse hair loss – to a lesser degree – on the parietal scalp bilaterally (Figure ). There was also loss of hair on the eyebrows, axillae, pubic region, and upper lip. However, these areas had already slowly started to show regrowth.
Biopsies from the right and left sides of her parietal scalp, in areas of alopecia with some preservation of follicles, were performed for horizontal and vertical sectioning. Both showed similar pathologic changes of a non-scarring alopecia. The predominant feature noted was extensive miniaturization of the hair follicles; this change was most suggestive of androgenetic alopecia. However, other findings – present to a lesser extent – included pigment casts in hair follicles, increased catagen to telogen ratio, and empty fibrous tracks; these changes may be observed in alopecia areata.
Correlation of the patient’s history, clinical presentation, and pathologic findings supported a diagnosis of antineoplastic (chemotherapy and hormonal) treatment-associated alopecia. Specifically, her features were consistent with those previously reported in patients with breast cancer after taxane chemotherapy and adjuvant hormonal therapy who developed permanent alopecia []. Treatment was initiated with minoxidil 5% foam to be topically applied to the scalp twice daily.
The patient returned for follow up four months later. She was pleased with the clinical outcome and had noticed increased scalp hair growth; however, she commented that she always used minoxidil once daily and occasionally twice daily. In addition, hair growth on the eyebrows, axillae, and pubic area continued to demonstrate clinical improvement. She decided to continue treating her scalp in a similar manner.
Her subsequent follow-up visit, six months later (after ten months of topical minoxidil therapy), showed additional hair regrowth. Specifically, the central and vertex area of her scalp had thickening of her hair; in addition, there was new hair growth on the parietal regions bilaterally (Figure ). She continues to use 5% minoxidil foam once daily.
|
pmc-6167024-1
|
A 46-year-old Caucasian female presented in November of 2017 with a longstanding history of pancreatitis. The patient is part of a family with multiple diagnosed cases of pancreatitis who had undergone genetic testing to reveal a PRSS1 gene mutation (N29I) and disease-modifying CFTR mutation ((TG)11-5T). The patient first showed symptoms of pancreatitis when she was 13 years old. Eight years later in 1992, the patient underwent a distal pancreatectomy, splenectomy, and cholecystectomy with modified Puestow procedure for symptoms of radiating epigastric pain, nausea, and vomiting typically lasting for 1 week and occurring three to four times per year (). Since that time, the patient had been followed every 6 months with accepted routine pancreatic cancer surveillance through magnetic resonance imaging and endoscopic ultrasonography. The patient did well until May of 2017 when she began experiencing recurring symptoms of acute pancreatitis, including dull waxing and waning epigastric pain with foods and liquids. On computed tomography scan, significant calcification in the right-sided pancreatic remnant was present (). Both CA 19-9 and carcinoembryonic antigen (CEA) values were elevated at 54 and 5.0, respectively. The patient elected to proceed with a completion pancreaticoduodenectomy and ethanol nerve block. Intraoperatively, there were significant adhesions present, which were lysed. The Puestow was taken down by transecting the Roux limb using a gastrointestinal anastomosis (GIA) stapler. The duodenum just distal to the pylorus was also transected using a GIA stapler in the same manner that is done during a pylorus-preserving pancreaticoduodenectomy. The hepaticojejunostomy was planned using the Roux limb from the prior Puestow procedure. The duodenojejunostomy was made with the proximal jejunum just distal to the ligament of Trietz (). The nerve block was performed by injecting 20 mL of 50% ethanol solution on either side of the aorta at the level of the celiac axis. The pathology on the resection specimen demonstrated chronic pancreatitis with parenchymal calcifications and duct ectasia consistent with PRSS1-related HP. No cancer was seen. The patient recovered well, was discharged on postoperative day 5, and was seen in follow-up fully recovered.
|
pmc-6167081-1
|
Patient A is a 45-year-old Caucasian male. He has no history of cancer in his family and is asymptomatic.
Following the recommendations of the USPSTF, the AAFP, the NCI, the CDC, the ACS, and the AUA, clinicians may not recommend PSA-based screening for patient A. However, following the recommendations of the NCCN, the NCI, and the CDC, the risks and benefits of PSA-based screening for prostate cancer may be discussed with patient A for him to make an informed decision about PSA-based screening for prostate cancer.
Case Study 2
|
pmc-6167081-2
|
Patient B is a 45-year-old African American male. He has no history of cancer in his family and is asymptomatic.
Following the recommendations of the USPSTF, the AAFP, the NCI, the CDC, and the AUA, clinicians may not recommend PSA-based screening for patient B. However, following the recommendations of the NCCN, the AAFP, the NCI, the CDC, the ACS, and ASCO, the risks and benefits of PSA-based screening for prostate cancer should be discussed with patient B for him to make an informed decision about PSA-based screening for prostate cancer.
Case Study 3
|
pmc-6167081-3
|
Patient C is a 75-year-old Caucasian male with a medical history of controlled asthma. He has no history of cancer in his family and is asymptomatic.
Following the recommendations of the USPSTF, the AAFP, and the AUA, clinicians may not recommend PSA-based screening for patient C. However, following the recommendations and guidelines of the NCCN, the NCI, the ACS, and ASCO, clinicians may discuss PSA-based screening with patient C.
Case Study 4
|
pmc-6167081-4
|
Patient D is a 60-year-old African American male. He is asymptomatic. His 62-year-old brother was successfully treated with radiation for prostate cancer.
Following the recommendations of the AAFP, the NCI, the CDC, the ACS, the AUA, the NCCN, and ASCO, clinicians would find it less difficult to discuss PSA testing with patient D since he is an African American male with a first-degree relative diagnosed with prostate cancer before 65 years of age. However, following the recommendations of the USPSTF, clinicians may not recommend PSA-based screening for patient D.
|
pmc-6167103-1
|
A 67-year-old woman with complaints of facial pain and tiredness in the masseter muscles for 7 years which got worse in the last year. She reported throbbing pains, previous TMJ clicks that evolved into crepitations. The pain got worse with chewing and during crises, it expanded to bilateral temporal region and nape. The patient was edentulous and used total dentures, removable upper and implant supported lower. She underwent numerous professional interventions such as myorelaxant splints, antidepressant medications, analgesics and anti-inflammatories without significant improvement.
Magnetic resonance imaging of the TMJ’s suggested bilaterally degenerative process, such as accentuated sclerosis of the right subchondral bone, osteophytes, displacement and sharpening of the articular disc and condylar repositioning. (Fig. A). The joint decompression test with electromyography suggested by Learreta () showed a need to recompose the lost vertical dimension even with the use of prostheses. The orthopedic neurophysiological position was obtained combined the MRI with the neuromuscular deprogramming with transcutaneous electrical stimulation (TENS). Once the rest position was established, a bite registration was performed taking in account the spatial position as seen in the MRI, as well as determination of neuromuscular rest position and free way space for orthotic construction (,). The patient made continuous use of the device, including for mastication, taking it out only for hygiene, for a total period of 16 months. A monthly evaluation protocol was established with a clinical, electromyography and kinesiography checkup, so that in neurophysiology could be addressed and the orthotic could be modified to keep orthopedic position up to date to the TMJ status (Fig. B-E). This protocol is due to the rehydration of the joint tissues during the decompression. Only two modifications of the device were needed in order to the pain disappear. There was also association in therapy with vitamin D3 supplementation, resveratrol, omega 3, N-acetylcysteine and low doses of naltrexone. All of them, used for six months.
|
pmc-6167103-2
|
A 30-year-old woman complaining of joint pain and bilaterally clicking of both TMJ for 5 years, which got worse in the last two years. She also reported face pain when she woke up and left masseter tiredness. Chewing and speaking usually aggravated pain in the left TMJ and had louder clicks in the right TMJ. After numerous interventions such as functional orthopedics, myorelaxant splints, manual therapies, laser, acupuncture, antidepressants and various analgesics for about 2 years, the patient had not experienced any significant improvement of the condition. She also had a history of hypertension controlled with medications.
The same treatment protocol was used for joint decompression orthopedic neurophysiological alignment of the mandible. Follow up showed improvements in muscle recruitment based on Learreta´s EMG test (,). The orthotic was maintained for a period of 21 months of the treatment and beyond after discharge. The ending of the symptoms occurred after 5 months of treatmen and there was a significant improvement after 60 days of decompression. Four different devices were progressively used because there was a greater need for articular space. Later after clinical discharge, the patient went to the second phase of treatment with three-dimensional volumetric orthodontics in order to replace the orthotic with teeth, keeping the vertical dimension and, therefore, joint spaces (Fig. ).
|
pmc-6167527-1
|
A 68-years-old man, with a history of diffuse lichen planus which had resolved 9 years prior, was diagnosed with stage IV squamous NSCLC. He underwent definitive radiation therapy to the right upper lung lobe with carboplatin and paclitaxel combination chemotherapy weekly for 6 weeks, with positive response to therapy. However, after 9 months, his lung nodules were noted to be progressively enlarging, and two additional nodules were identified, concerning for new metastases. He was then started on nivolumab, a PD-1 inhibitor, at a dose of 3 mg/kg infused every 2 weeks. After six cycles of treatment, he developed a widespread pruritic eruption involving chest, back, extremities, and penis. On examination, he was noted to have too numerous to count 3–10 mm pink to pink-brown thin papules and plaques, which were flat-topped with scale over the chest, abdomen, back (Figure ), arms, legs, and penile shaft, some of which had an erythematous base. The head of the penis had numerous ill-defined erosions measuring up to 1.5 cm. Additionally, he had developed a 5 mm shallow ulceration of the left lateral tongue. A punch biopsy of a characteristic lesion on the left upper arm was performed, which showed a slightly acanthotic epidermis with prominent hyperkeratosis and hypergranulosis, with a band-like lymphohistiocytic infiltrate, focal squamatization of the basal cell layer, and necrotic keratinocytes (Figure ). Given the clinical presentation and these histopathological changes, he was diagnosed with a lichenoid mucocutaneous eruption due to nivolumab. Treatment with triamcinolone 0.1% ointment to the body, clobetasol 0.05% ointment to the penis, and clobetasol 0.05% gel to the tongue twice daily was initiated. Given the widespread distribution of the eruption and the associated intense pruritus, a 5-weeks oral prednisone taper starting at 80 mg daily was also started and nivolumab treatment was held for 1 week. At the completion of the oral steroid taper, his rash had significantly improved, including complete resolution of the penile erosions and oral ulceration despite resuming nivolumab therapy. However, 6 days after discontinuing prednisone, the rash recurred on the chest and back, requiring a second prednisone taper. The eruption recurred again after completing the taper, leading to a trial 5 mg daily maintenance dose of prednisone. Due to persistence of rash and pruritus, a third prednisone taper was initiated and narrowband UVB phototherapy three times per week was started as an adjunct therapy. The prednisone was gradually transitioned to a 10 mg daily maintenance dosing. After 1 month of this treatment regimen, the patient's eruption had significantly improved, with no inflammatory papules seen on examination. Initially the prednisone maintenance dose was unable to be decreased without recurrence of the eruption. After ~70 NBUVB treatments (5–6 months), a slow prednisone taper with oral hydroxyzine was initiated. Once on 5 mg daily prednisone, the patient elected to stop NBUVB treatments as his rash and pruritus were controlled. On clinical examination 6 months later, complete resolution of the rash was observed with only residual post-inflammatory hyperpigmentation while still on 4 mg daily prednisone (Figure ). His prednisone continued to be tapered, eventually being discontinued completely after 4 weeks. Four months after stopping prednisone, the rash and pruritus have remained completely resolved. Importantly, while the patient has been treated for this rash, he has been able to remain on nivolumab therapy. To date, he has completed 52 cycles and his NSCLC has been stable.
|
pmc-6167577-1
|
A 71-year-old Japanese man with a medical history of HCC that resulted from chronic hepatitis B infection underwent a left lateral segmentectomy for HCC at another institute. Pathological findings of the resected specimens were moderately differentiated hepatocellular carcinoma (St-P, 55 × 50 × 38 mm, eg, fc(+), fc-inf(+), sf(−), s0, nx, vp1, vv0, va1, b0, im0, p0, sm(−), and lc lead to pT3 and pStageIII). Two years after surgery, his serum alpha-fetoprotein (AFP) level increased to 1800 ng/ml (normal is 0–10 ng/ml). Physical examination showed no remarkable abnormal findings. Laboratory blood and chemical examination results were also within normal limits. A follow-up examination that included an upper gastrointestinal endoscopy showed a pedunculated polypoid tumor in the middle thoracic esophagus, approximately 2 cm in diameter (). Esophageal varices were not seen at the anal side of the tumor. A barium esophagogram showed an elevated mass in the middle thoracic esophagus (). The biopsy specimen obtained from the esophageal lesion revealed tumor cells with acidophilic cytoplasm that proliferated without a tubular structure (). Tumor cells in the biopsy specimens were positive for hepatocyte stain (monoclonal mouse anti-human hepatocyte antibody) (). The esophageal tumor was diagnosed as a metastatic HCC tumor. Chest computed tomography (CT) showed an elevated mass in the esophageal lumen (). Abdominal CT detected no evidence of metastasis to the lung or of new HCC lesions in the liver, except for lymph node metastases in the lesser curvature area of the stomach. However, a portal tumor thrombus was not found. As the patient was in good general condition and preoperative imaging showed resectable disease, we performed surgical resection. Esophageal resection via right thoracotomy was performed with regional lymph node dissection, and the whole stomach for reconstruction was made to provide better protection of the submucosal vessels, compared to gastric tube approach. Esophagogastrostomy was performed at the intrathorax where the gastric tube was lifted up through the posterior mediastinal route. Intraoperative exploration revealed no peritoneal dissemination. The gross appearance of the resected specimen was a reddish polypoid tumor in the middle esophagus (). A metastatic esophageal tumor from HCC was confirmed by positive immunohistochemical staining for hepatocyte and AFP (). Two months following the operation, a follow-up CT demonstrated multinodular-type HCC in both lobes of the liver. The patient received no additional therapies and died from disease progression two months following the operation.
|
pmc-6167579-1
|
In June 2017, a 33-year-old gravida 3, para 2 female in her third trimester presented (ED) with active left nasal bleeding to the emergency department at King Abdulaziz Medical City in Riyadh, Saudi Arabia. The patient's vitals were stable upon presentation and she denied any history of trauma or nose picking. Additionally, she complained of a one-month history of persistent left-sided nasal obstruction. The patient was free of medical diagnoses and had no personal or family history of bleeding disorders or any other conditions. Anterior nasal packing was applied, and bleeding stopped two hours later. Her hemoglobin level was 9.9 mg/dL. Normal saline nasal irrigation was prescribed, and first aid instructions were given. Nasal packs were removed, and the patient was advised to come back if bleeding recurred. Ten days later, the patient returned to the ED with another episode of epistaxis that was managed conservatively. At that time, her hemoglobin levels were 9.4 mg/dL. She was discharged and advised to follow up with otorhinolaryngology. On the same day, she arrived at the ED for a second time with epistaxis of moderate severity. Again, minimal anterior nasal packing was applied, and the patient was sent home. On the next day, she returned to the ED for the third time in 48 hours with active bleeding from her left nostril. Her hemoglobin level at this point measured 8.7 mg/dL. During a bed side examination, the right nasal cavity appeared clear. However, the left nasal cavity evidenced a large clot with moderate bleeding. A nasal endoscopy was performed, which revealed a large, red, smooth, and rounded mass in the left nasal cavity that was actively bleeding upon any application of pressure. The bleeding stopped with properly sized anterior nasal packing, and the patient was rehydrated with intravenous fluids. Obstetrics and Gynecology and Otorhinolaryngology (ORL) teams were consulted. Since the patient was in her 38th week of pregnancy, a decision was made to retain the nasal pack for 48 hours more and admit her for spontaneous delivery, after which she would be reassessed. Two days later, the patient delivered, and both the mother and the baby were in good health. The day after delivering, the patient was taken to the ORL clinic for reassessment. The nasal pack was removed, after which the patient proceeded to actively bleed. Endoscopic assessment illustrated no changes to the nasal mass. Her hemoglobin dropped to 7.8 mg/dL. A blood transfusion was recommended, but the patient refused one. On the same day, a contrasted CT scan of the paranasal sinuses was scheduled, which revealed a heterogeneously enhanced soft tissue mass involving the middle and lower meatus of the left nasal cavity measuring 3.2 × 2.2 × 1.5 cm with normal adjacent bony structures (). Two treatment options were discussed with the patient. The first was to wait for spontaneous regression of the mass following hormonal withdrawal after delivery. The second was to undergo complete surgical excision for quick symptomatic relief and tissue diagnosis, which the patient agreed to. The surgery was explained to the patient and informed, written consent was obtained. During intraoperative examination using a telescope, a large, well-circumscribed, red, smooth mass occupying the left nasal cavity and originating from the medial surface of the inferior turbinate and the inferior surface of the posterior part of the middle turbinate was visualized without any attachment to the nasal septum (). The mass was initially injected with lidocaine and epinephrine (1 : 100,000) and then excised completely from its site of origin via bipolar diathermy which resulted in no significant bleeding (). Gross histopathological analysis revealed a polypoidal tissue mass measuring 3.0 × 1.5 × 1.0 cm with a smooth and glistening surface (). Microscopically, the mass was composed of numerous capillaries, likely associated with edema, and inflamed stroma with no malignant cells noted. Based on this, a diagnosis of LCH was made (). Postoperatively, left-sided nasal obstruction markedly improved. The patient was stable and recovered well with no additional episodes of epistaxis or requirement for blood transfusion. She was consequently discharged on the same day in good condition. The patient was completely asymptomatic during follow-up appointments in our clinic at two weeks, one month, three months, and six months postoperatively, with healthy mucosa and no evidence of mass recurrence during endoscopic nasal examination ().
|
pmc-6167580-1
|
Six years after an uncomplicated cesarean delivery of a first child and one year after being diagnosed with CML, a 37-year-old woman presented to our institution at 36 weeks of gestation with worsening fatigue associated with abdominal discomfort.
The couple had rejected the option for medical-assisted abortion during the first trimester and, due to her low risk of CML progression determined by an European Treatment and Outcome Study (EUTOS) score of 86 [], her hematologist opted for imatinib cessation, which was given initially at a dose of 400 mg per day, during the first and second trimester. She received interferon-α in the later half of pregnancy. Response to treatment was assessed regularly and at the end of 35 weeks she was found to have a palpable spleen (increased from 4 cm to 8 cm below the lower left costal margin) and leukocytosis at 245,000WBC/mm3 including 32% blast cells. Platelet count and hemoglobin concentration were within normal ranges. Bone marrow aspiration was performed and the patient was diagnosed with CML in acute phase. Cytogenetic analysis of the bone marrow cells by using the Giemsa Banding technique revealed the karyotype 46,XX,t(9,22)(q34;q11.2) in 100% of the analyzed cells without any additional abnormality. FISH analysis was not performed.
Due to the significant deterioration and urgent need for chemotherapy initiation, cesarean delivery was planned for the end of 36 weeks, which was uneventful under general anesthesia. The rate of circulating blasts made the choice of general anesthesia mandatory and judicious rather than a risky perimedullary anesthetic technique. The newborn was healthy and did not require any medical interventions. Postoperatively, she received a multimodal analgesia and an effective thromboprophylaxis. Afterwards, she was started on treatment with imatinib, 800 mg daily, without any satisfactory response. Because of disease progression, the patient received hydroxyurea as palliative treatment with partial response.
|
pmc-6167582-1
|
A 78-year-old man with HSK visited our hospital due to asymptomatic gross hematuria and renal hypofunction in August 2016. He had comorbidities of Charlson index of 4, including chronic kidney disease (CKD; creatinine 1.71 mg/dL, estimated glomerular filtration rate [eGFR] 31 mL/min/1.73m2), atrial fibrillation requiring warfarinization, and type 2 diabetes (HbA1c 6.7%). He had undergone coronary artery bypass grafting at his age of 69 years.
Blood tests did not show any abnormalities other than elevated serum creatinine. Urinalysis and urinary sediments revealed proteinuria (2+) and hematuria (erythrocyte count 50-99/HPF). Voided urine cytology was suspicious for UC. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated an atrophic right renal unit and a right renal pelvic tumor of 28 mm, which exhibited high signal intensity on diffusion-weighted MRI (). Extrapelvic extention of the tumor, lymph node swelling, or distant metastasis were not evident. Only one renal artery was identified for each renal unit on MRI. Cystoscopy revealed no bladder tumor.
Under the clinical diagnosis of UC complicated with HSK (cT2 or less N0M0), the patient underwent RoboSurgeon gasless single-port retroperitoneoscopic right RNU with isthmusectomy in December 2016. In the RoboSurgeon system, intracorporeal surgical procedures are conducted under the three-dimensional (3D) magnified vision using Endoeye flex 3D deflectable videoscope (Olympus, Tokyo, Japan) and a high-definition 3D organic electroluminescent head-mounted display (Sony Corporation, Tokyo, Japan). Briefly, a pararectal incision of 6 cm was made in the right upper quadrant. The surgical plane between the transverse fascia and paraperitoneal fat was developed to make a wide working space extraperitoneally. Alexis Wound Retractor (Applied Medical, California, USA) was placed to make a single port of 5.5 cm in diameter (), and Omni-Tract FastSystem (Integra, Pennsylvania, USA) and PLES retractors (Innomedics Medical Instruments Inc., Tokyo, Japan) were used to keep an extraperitoneal working space (). The lateroconal fascia was incised to develop the Gerota's fascia anteriorly and to identify the isthmus and inferior vena cava (IVC). The fibrofatty tissue was dissected from the IVC, and small branches from the IVC and aorta were divided using clips and LigaSure (Covidien, Minnesota, USA). Finally, main branches of the right renal artery and the right renal vein were ligated and divided. The right ureter was then clipped to shut the urinary flow off. Then, the right kidney was completely mobilized, preserving the right adrenal gland. Isthmusectomy was conducted using Sonicision (Covidien, Minnesota, USA) and LigaSure (). The right ureter was dissected to the ureterovesical junction and RNU with bladder cuff excision being completed. The bladder defect was closed with absorbable sutures and water-tightness was confirmed. The operative field was irrigated with saline of 2L, a drain tube was placed in the right retroperitoneal space (), and the wound was closed. The operation time and blood loss were 304 minutes and 510 mL, respectively.
Gross appearance of the resected specimen showed a nodular pedunculated tumor measuring 3.2∗2.4∗1.0 cm in the right renal pelvis (). Specimen weight was 162 g and the size of the kidney part of the specimen was 11.0∗5.0∗3.5 cm. The pathological diagnosis was high grade (grade 3) invasive UC with parapelvic fat microinvasion (pT3). No lymph node metastasis (pN0, 0/1), lymphovascular invasion, or positive surgical margin was noted.
The patient resumed diet intake and ambulance on the first postoperative day, and the drain tube and the urinary catheter were removed on the 4th postoperative day. He left the hospital on the 6th postoperative day without any postoperative complication. He did not select any adjuvant therapies. He is free of the disease 19 months after RNU and his serum creatinine level is 1.92 mg/dL with eGFR of 27.0 mL/min/1.73m2.
|
pmc-6167587-1
|
A 27-year-old male with underlying congenital hydrocephalus and paraplegia was admitted to the hospital due to infected sacral sore. He had a placement of a ventriculoperitoneal shunt when he was a child. Upon admission, he also complained of progressively having difficulty in passing urine and leaked urination but he denied dysuria, hematuria, and pyuria. On examination of his genitalia, the prepuce was deformed and enlarged with phimosis. To our surprise, there was a huge stony hard foreign body under the prepuce measuring 5 × 5 cm in size (). It was visualised through the stenosed prepuce. Otherwise, the testes were normal and abdominal examination revealed no significant finding.
A pelvic radiograph was arranged and revealed a well-rounded radiopaque lesion in his penile region representing a large stone (). Ultrasound of the kidney, ureter, and bladder (KUB) revealed an absence of proximal tract stone. A Foley's catheter was inserted to assist his urination, and it drained minimal amount of clear urine. His serum creatinine level was elevated initially but resolved after hydration. He was then planned for circumcision and removal of the preputial stone once his sacral sore improved. After almost a month in the ward, he finally underwent the circumcision. A flexible cystoscopic examination beforehand showed a trabeculated and small contracted bladder with no urethral stricture seen. The circumcision was undertaken using a dorsal slit technique. A huge preputial stone measuring 4 × 4 cm was uneventfully retrieved (Figures and ). His recovery went well without any complication postoperatively.
|
pmc-6167593-1
|
A previously healthy 14 year-old boy presented to the Emergency Department (ED) for evaluation of right eye injury that was sustained one to two hours prior to arrival. According to the patient, he was standing on a street corner on his way to school when he was struck in the right eye with a rock thrown from a passing car. He reported no loss of consciousness and no other injury. He denied nausea or vomiting. His past medical history and family history were not significant. The patient did not have any prior ocular injuries, history of ocular disease, or prior ocular surgeries. The patient reported that he does not wear glasses.
In the ED, he was alert, awake, answering questions, and following commands with a GCS of 15. His only complaint was pain to the right eye. His vital signs were as follows: temperature 98°F, heart rate 79 beats/minute, respiratory rate 18 breaths/minute, blood pressure 151/73 mmHg, and oxygen saturation 99% on room air. On examination, blood was oozing and pooling in his right eye with eye lid swelling and surrounding abrasions ().
The patient expressed severe photophobia in his right eye and was unable to open it. Detailed examination of the right eye could not be performed due to the possibility of right ocular globe rupture. A metal eye shield was placed on the patient's right eye for protection. His left eye refractory status was normal with a visual acuity of 20/20. The patient had a negative battle sign, no hemotympanum, no rhinorrhea, and no cervical spine tenderness. The remainder of his examination was noncontributory.
A non-enhanced CT scan of the orbits was obtained and revealed rupture of the right globe with associated orbital and periorbital swelling with intraconal hematoma ().
An ophthalmology consultation was obtained. He was then taken to the operating room where a 20 mm linear laceration was noted crossing the iris and sclera with uvea protruding. The laceration was repaired. The patient tolerated the procedure well and was followed-up in the clinic on the next day. The patient received an explanation regarding the poor prognosis of his right eye vision. The open globe injury caused blindness in his right eye. The patient was advised to wear protective eye gear to prevent future eye injuries while participating in activities that carry a risk of eye injury.
|
pmc-6167596-1
|
A 16-year-old, Sinhala ethnic Sri Lankan woman in her first pregnancy, was admitted with severe preeclampsia at 29 weeks of gestation. She has made her booking visit at ninth week of gestation and all the booking investigations were normal except for the platelet count which was 112,000 per liter. During her pregnancy, the lowest platelet count was 80,000 per liter at 27 weeks of gestation and no specific intervention has been done except for regular monitoring of the platelet count. She had been diagnosed with gestational hypertension at 22 weeks of gestation and prescribed labetalol and methyldopa. Other than that, she has had few erythematous, itchy macular lesions over the palm of her right hand from early in the first trimester onwards and had persisted throughout the pregnancy. She has had mild pain in her right small finger from first trimester onwards. But she had not worried about these symptoms so they had gone unnoticed. She had been apparently well until late 28 weeks of gestation and then she has developed a severe headache and worsening of bilateral lower limb oedema with frothy urine leading to hospitalization. She was diagnosed with severe preeclampsia (blood pressure of 185/115 mmHg) at 29 weeks of gestation. An emergency caesarean delivery was arranged soon after this presentation. Her baby was admitted to the premature baby unit with a birth weight of 1000 grams. She was in intensive care unit in first 24 hours after delivery and received intravenous magnesium sulphate as a prophylactic anticonvulsant.
Her pain in the right finger worsened after delivery and erythematous macular lesions have been increased in number and spreading over the dorsal aspect of the right forearm. She was not worried and lesions have gone unnoticed especially with her dark skin complexion. Her blood pressure was under control with oral nifedipine. At the eighth postpartum day, her right small finger was noted to be cold with increased pain. Discoloration of the above skin lesions was more prominent and started to appear over the palm and the ventral aspect of the forearm of the right hand too, with preserved capillary refilling time. Both radial and ulnar artery pulsations were felt. There were no similar lesions in any other part of the body. She was soon transferred to a medical ward for further management.
She was subjected to an urgent arterial duplex study, which revealed proximal ulnar artery thrombosis in the right side with partial occlusion to the blood flow. And soon she was started on unfractionated heparin and eventually bridged with oral anticoagulants (warfarin) in order to archive the target international normalized ratio (INR) of 2.0-3.0. With anticoagulation treatment, her symptoms and signs were markedly improved. Sequential macroscopic changes of the affected arm and fingers have been shown in .
Routine laboratory analyses were within the normal range including subsequent platelet count, but she got positive results for direct Coombs test. Her reticulocyte count was high with normal haemoglobin concentration. Her ANA titre was strongly positive (1:320). And also anti-cardiolipin antibodies (anti-CL) and anti-β2 glycoprotein-I (anti-β2GPI) levels were also noted to be positive. However, her ds DNA and C3/C4 levels were within normal limits. Her blood pressure readings too have come back to normal level with no requirement of medications. Also proteinuria was settled. Her laboratory tests for APS were positive even after 12 weeks of initial testing. Therefore, it was diagnosed as a case of primary APS.
|
pmc-6167598-1
|
A 25-year-old male patient presented to emergency department after an assault with an incise wound in the external canthus of the left eye and severe alcohol intoxication, which prevented history taking. The physical exam of the ocular globe was not possible due to the large hematoma that hindered opening the eyelid. The exploration of right eye was normal. Therefore, the skin wound was initially sutured with a polypropylene 5/0 interrupted suture and an orbital computed tomography (CT scan) and skull X-ray were performed. An intraorbital foreign body with triangular shape of 4.6 cm x 2 cm was seen in the left orbit, passing through the orbit and the ocular globe, fracturing the superomedial wall of the orbit, with a probable associated fracture of the ethmoidal cells, and reaching the anterior cranial fossa, causing pneumocephalus (Figures and ). The patient did not present any neurological symptoms beyond his alcohol intoxication nor did he develop rhinorrhea at any time, and the Glasgow Coma Scale/Score was normal (15/15). He remained under observation and was treated with intravenous antibiotic (ciprofloxacin 200 mg twice daily, for five days, selected due to its broad spectrum for gram negative and positive) and corticosteroids (methylprednisolone 80 mg per day for 3 days). Surgical extraction was performed. The foreign body turned out to be a fragment of a dagger. The extraction was done locating the end of the foreign body after removing the suture of the wound and disinserting the lower eyelid to have a wider surgical field. The foreign boy was carefully extracted without exerting force. It was then possible to see a corneoscleral wound 2 cm long affecting the upper cornea 7 mm and the sclera 8 mm located from 9 to 2 o'clock positions. It was closed with nylon 10/0 suture and polyglactin 910 7/0 suture. The entrance area in the orbit was revised, with special attention to the upper nasal quadrant, ruling out the need for repair by neurosurgery (). One week after the surgery, the cornea was transparent, but there were amaurosis, hemophthalmos, and hypotony (). The patient remained painless. Evolution to phthisis bulbi was evident, with clouding and folds in the cornea, shrinkage of the eyeball and a very soft tone, and six months later the eye was eviscerated. Two years later there were no signs of sympathetic ophthalmia in the right eye, whose examination remained completely normal.
|
pmc-6167757-1
|
A 65-year-old woman, obese (body mass index-BMI 33.2 kg/m2), with no history of smoking habit underwent a nodulectomy for the incidental finding of a pulmonary nodule on chest radiography and on computed tomographic (CT) scan (Fig. A and B, white circles), with subsequent histological diagnosis of atypical mycobacterial infection.
She had a clinical history of poliomyelitis at 9 years old, resulting in right hemiplegia and a hysterectomy including the ovaries at 45 years old. Before surgery, her clinical condition was normal; the functional respiratory tests showed an increase in residual volume, probably caused by an expiratory muscle weakness due to the poliomyelitis in the past (Fig. C).
The postoperative course was complicated by subcutaneous, right, parietal emphysema, which extended up to the neck (Fig. A and B). Five months later, the patient underwent a chest CT scan for the subsequent appearance of chest pain, dyspnoea, and asthenia; the lung hernia (67 X 13 mm) was revealed as a hole of 35 mm in the space between the fifth and the sixth rib of the right chest wall (Fig. C and D, white circles). Next, the lung hernia was reduced surgically, resulting in a right pleural effusion and significant subcutaneous emphysema. The almost complete resolution of the clinical and radiographic (Fig. A and B) conditions occurred after a year and a half, during which time the patient underwent periodic follow-up.
|
pmc-6167758-1
|
A 42-year-old male who was exposed to human papilloma virus (HPV) at birth developed chronic upper airway papillomatosis. He was exclusively managed by ear, nose, and throat surgeons (ENT) from 20 months old (first manifestation) until 42 years of age, when his papillomatosis disease extended to involve his upper trachea. At this time, in 2013, he was referred to our respiratory service for management.
Past medical history included a current 22 pack-year smoking history, with no other comorbid medical conditions. There is no family history or personal history of immunodeficiency. He worked as a shopkeeper and had no other known exposures.
The patient developed marked symptoms as the papillomas grew. These included: constant shortness of breath, reduced exercise tolerance, cough, wheeze, hoarse and quiet voice. The severity of these symptoms resulted in him being unable to work due to poor voice projection. On examination, auscultation revealed bilateral polyphonic expiratory wheeze and monophonic inspiratory wheeze. Direct visualization via bronchoscopy demonstrated significant HPV polyposis of the larynx, vocal cords, supraglottis, subglottis, 40–50% of his trachea involved and significant narrowing of airways (see Fig. ). The distal trachea was not affected.
He has had 166 exacerbations requiring intervention for regrowth of polyps. Between 2013 and 2015, he was managed with numerous multiple laser ablation procedures via mircolaryngoscopy and flexible bronchoscopy. Previously tried therapies included: argon plasma coagulation (APC), topical mitomycin, CO2 laser, diathermy snare, electrocautery ablation, dietary changes and natural remedies. The histopathology of the resected respiratory papillomas demonstrated squamous papillomas with focal koilocytic change. There was no evidence of high-grade dysplasia or malignancy in resected specimens.
In 2016, a decision was made to use intralesional cidofovir, with the aim of achieving better disease control and reducing the frequency and severity of exacerbations. Fibre-optic bronchoscopy facilitated the removal of the papillomas via pulsed APC, a circumferential catheter at flow rate of 1.8 L/min, effect 1 and maximum watts of 40. This was followed by an injection of intralesional cidofovir via a 19G Wary Transbronchial histology needle. A vial of 375 mg/5 mL of cidofovir was diluted in 30 mL of normal saline. The cidofovir was injected circumferentially every 0.5 cm down the trachea, with a total of 50 injections. The procedure was performed under general anaesthesia with suspended laryngoscopy, along with an ENT team that treated disease of the cords. This regimen has been tried twice before, and on follow-up procedure at 18 months, only 20% of his trachea was affected. The next follow-up period was in 6 months, where a 1.9 mm cryoprobe of effect 2 was used to remove the papillomas. This was followed by an intralesional cidofovir injection via a 19G Wary Transbronchial histology needle. At this follow up, <20% of his trachea was found affected (see Fig. ). Previous exacerbations were occurring with a frequency of 4×/12 months (2013), 7×/12 months (2014) and 5×/12 months (2015).
|
pmc-6167792-1
|
The proband (Fig. ) was a 5-year-old Chinese boy admitted to the hospital with a chief complaint of language articulation disorders for 1.5 years, uncoordinated movements for half a year, and repeated episodes of seizures for one week. A stutter was noted without obvious causes one and a half years before, and he had gradually developed unclear enunciation, clumsy speech, and slow response. Within the previous 6 months, he began to have uncoordinated movements, such as ataxia and instable gait. Three episodes of generalized tonic–clonic seizures had occurred in the week just preceding the clinic visit; every episode lasted for about 1 min and occurred about once every 2 days. He achieved appropriate developmental milestones before age 3.5 years and was born at full-term by vaginal delivery without any complications. There was no exposure to alcohol or medications during the pregnancy, and Apgar scores were 10 and 10 at 1 and 5 min, respectively. His parents had no known consanguinity, and both they and an older sister were healthy.
On physical examination, length, weight, and head circumference were normal for age, and he was conscious and showed normal muscle strength and muscle tone. Patellar reflex and Achilles tendon reflex were normal, and Babinski signs were negative. He was inarticulate and responded to questions slowly. He could not complete the hand-alternating movement test, heel–knee–tibia test, or finger–nose test because of poor cooperation with the instructions. The intelligence quotient value, measured by combined Raven’s test, was 80 (a medium level). The tests for blood lactic acid, homocysteine, ammonia, ceruloplasmin, and liver and kidney function were normal. Tests for antibodies for autoimmune encephalitis in cerebrospinal fluid and blood were negative. The screening for genetic metabolic diseases in blood and urine showed no obvious abnormalities. An electroencephalogram (EEG) showed multiple spikes and slow-wave discharges bilaterally. A brain MRI scan showed high hyperintensities adjacent to the bilateral posterior horns of the lateral ventricles on T2-weighted images and broadened cerebellar fissures (Fig. ). Leukoencephalopathy was considered, and genetic analysis for hereditary leukoencephalopathies was recommended.
With written consent from his parents, peripheral blood samples were collected from the proband and his parents. DNA was extracted using the Puregene Extraction Kit (Qiagen, USA). The NextSeq500 sequencer (Illumina Inc., USA) was used to screen the exons in the genes related to hereditary leukoencephalopathies. The genes in the panel are listed in Additional file . The data obtained were analyzed using accompanying software, and the variants were called according to the protocol. The variants were interpreted according to the guidelines from the American College of Medical Genetics and Genomics and patient phenotype []. Direct sequencing validated the detected missense mutations. Direct sequencing was performed on DNA from the proband and his parents using the ABI3500sequencer (Life Technology, USA), and the samples were subjected to sequence analysis using Sequence Scanner v1.0 (Applied Biosystems, USA). The matched Chinese controls were obtained from the Shenyang Kingmed for Clinical Laboratory (Shenyang, China). The sequencing procedure and mutation validation were performed by Shenyang Kingmed for Clinical Laboratory (Shenyang, China), which provides third party inspection services.
The possible effects of the mutations on protein function were analyzed using the Polymorphism Phenotyping v2 (PolyPhen-2) prediction tool (), SIFT (), and MutationTaster ().
The genetic analysis showed that the proband had a homozygous missense point mutation c.892G > A(p.Glu298Lys) (reference sequence: NM_017882.2) in exon 7 in CLN6 and that both his parents were heterozygous for the mutation (Fig. ). The mutation was not detected in 259 control subjects. LINCL was diagnosed, and a visual test was performed and showed no obvious abnormalities. PolyPhen-2, SIFT, and MutationTaster analysis suggested that the mutation would negatively affect protein function (Table ). The mutation in the patient is the first reported, and we can define it as recessive.
Because of the history of seizures and the EEG results, the patient was diagnosed with epilepsy, and oral sodium valproate (VPA) was ordered. VPA administration was initiated at 15 mg/kg per day, administered in two doses, increasing to about 25 mg/kg per day in 2 weeks. The blood concentration range was 56–78 μg/ml over 6 months. At the 6-month follow-up, the episodes were decreased to about once a month, articulation disorders and uncoordinated movements persisted, and visual loss was not detected.
At the locus of CLN6, 31 missense mutations including the reported ones and ours were analyzed; 22.6% (7/31) were located in the cytoplasmic domains, 32.2% (10/31) in the TM domains, and 45.2% (14/31) in the luminal domains of the protein. Regarding each domain of the protein, the mutations were mostly located in the TM3-TM4 loop (6/31), TM1-TM2 loop (4/31), and C-terminus (4/31), and no mutations were reported in the TM4-TM5 loop, TM5-TM6 loop, and TM7 domain (Table ).
|
pmc-6167793-1
|
A 63-year-old Caucasian male veteran was referred to the chiropractic clinic with a 2-year history of insidious, worsening low back and bilateral leg pain. The patient complained of pain and cramping in his lower legs that was provoked with walking and immediately relieved with sitting. He further described the left leg as mildly worse than the right. His walking was limited to approximately 50-ft due to pain, but leaning forward on a grocery cart greatly increased his capacity. His medication list included 81 mg aspirin once daily and short-term dose of hydrocodone/acetaminophen 30/300 mg for an unrelated condition (excision of a cervical sebaceous cyst). He was previously prescribed a trial of 300–900 mg Gabapentin, but discontinued without relief. His relevant medical history included right femur internal fixation for a traumatic intertrochanteric fracture, diabetes mellitus, open mandible surgery with hardware placement in the 1970’s, and left ankle surgery with temporary hardware in 1995. He did not have any history of anabolic or corticosteroid use, Cushing’s disease, or history of epidural steroid injections.
Relevant physical examination included a body mass index of 38. He reported weight gain in response to his pain, and chart notes corroborated a BMI of 32.5 approximately 2-years prior to presentation. The patient had moderate flexion and extension limitation with lumbosacral pain on extension. Sensory, motor and tendon reflexes were within normal limits. Lumbosacral pain was present with facet loading. Hip internal rotation and flexion limited bilaterally due to hamstring and hip musculature tightness. All other lumbar and pelvic orthopedic tests were unremarkable. On initial presentation, pain disability questionnaire [] was scored as 63 out of 150, with a functional status component of 37 and a psychosocial component of 26. The patient presented with computed tomography; revealing suspected left foraminal disc protrusion at L4–5 and bilateral L4–5 and L5-S1 facet hypertrophy, but no bony spinal stenosis (Fig. ).
He was diagnosed with neurogenic claudication and treated six times with flexion-distraction to the lumbar spine and high-velocity low amplitude manipulation to the lumbar and thoracic spine. Soft tissue manual therapy was performed on the hip external rotators bilaterally. He was instructed in repetitive lumbar end-range flexion (centralization phenomenon observed), hip mobility exercises, and sciatic nerve glides. At his seventh session a re-examination was performed. His hip flexion range of motion was improved, however he continued to experience pain with walking and cramping in his lower legs. Orthopedic testing was without significant change. His updated pain disability questionnaire scored 96/150, indicating a potential progression of his disability.
At this time the patient was able to be cleared for a lumbar MRI which revealed no signs of bony or discogenic spinal stenosis; however circumferential epidural fat was present at L5-S1. Inspection of the patient’s T1 weighted MRI revealed the grade III pathognomonic “Y” sign (Fig. ). A measurement of the patient’s epidural fat using the modified method (a method used when a straight anterior to posterior measurement is not possible) developed by Borre et al. [] revealed the following: dural sac / epidural fat value of 0.19 and epidural fat / spinal canal value of 83.9% as measured by the authors (Fig. ). This measurement also categorized the patient as a grade III. The patient was seen for two additional visits without any further durable gains and was referred by his medical provider for a neurosurgery consult. After meeting with the neurosurgeon, the patient opted not to pursue surgical intervention.
|
pmc-6167793-2
|
A 51-year-old African American male veteran was referred to a pain management physician for a lumbar epidural steroid injection (LESI) for low back pain with radiculopathy. The patient had a history of low back pain and lower extremity pain for three and half years. Previous treatments included naproxen, arch supports, proper lifting education, and physical therapy. Radiographs demonstrated minimal spondylosis at the L4 and L5 vertebral bodies and a MRI demonstrated a mild posterior central disc herniation at L5-S1, but no evidence of SEL (Figs. & ).
The patient’s medical history included low back pain, hip pain, plantar fasciitis, obesity, and benign prostatic hyperplasia. The patient’s active medications included: meloxicam, terazosin HCL, ibuprofen, bisacodyl, cyclobenzaprine, methocarbamol, tramadol HCL, acetaminophen/hydrocodone, omeprazole, and ketorolac tromethamine. The patient had no history of anabolic or corticosteroid use or Cushing’s disease; BMI at the time of treatment was 34.
Over a five-week period the patient received a series of three interlaminar L4–5 LESI. The patient reported short-term relief with each injection in the series. Three months after the final injection the patient was referred for a neurosurgery consult. The patient’s neurological exam was fully intact and a repeat MRI was ordered with the following impression: L2 level degenerative changes of the lumbar spine with epidural lipomatosis at lower lumbar levels which result in severe central canal stenosis at L4–5 and L5-S1 and varying degrees of neural foraminal narrowing. When measured by the authors using the method developed by Borre et al. [], the dural sac diameter/epidural fat diameter was 1.07, and the epidural fat/spinal canal diameter was 48.3%, categorizing the patient as a grade I (borderline grade II) (Figs. & ). The initial MRI measured 2.09 (DS/EF) and 32.4% ((EF/SC) as measured by the authors, which results in a grade 0 categorization. It is important to note that in one review [] only 14.5% of grade II cases, and 0% of grade I, were symptomatic. The time between the initial and repeat MRI was 5 months and the only two interventions during this time were a series of three LESI and the introduction of acetaminophen/hydrocodone. As a result of the repeat MRI findings surgical decompression was recommended.
|
pmc-6167846-1
|
A 78-year-old woman was admitted to the Department of Neurology on the third occurrence of generalized tonic clonic seizures (GTCS). A glioma had been diagnosed, and resection was performed 5 years previously. Following surgery, levetiracetam (LEV), 500 mg once daily was prescribed but discontinued by the patient 1 month later. The past medical history was otherwise unremarkable, except for 14 years of warfarin use at 1.875 mg per day prescribed for the secondary prevention of embolic events from paroxysmal atrial fibrillation (AF). The patient’s INR had not been monitored for 6 months, but there was no overt bleeding.
On the day of admission, 10 mg of diazepam was given intravenously to terminate a five-minute of GTCS while en route to a brain computerized tomography (CT) scan. The working diagnosis was status epilepticus (SE), and a loading dose of intravenous valproate sodium (1200 mg) was administrated to relieve the recurrent GTCS and frequent focal aware seizures. The patient remained physically well during interictal phase. Oral LEV of 500 mg twice daily was prescribed when the patient had regained consciousness. Oral warfarin was not discontinued based on the initial INR of 2.02. The patient was also on 40 mg oral isosorbide mononitrate sustained release tablets once daily and 12.5 mg succinate metoprolol tablets twice daily as needed. The brain CT scan showed left frontal and parietal craniectomy and encephalomalacia at the left frontal lobe.
On the second day of admission, routine laboratory studies revealed otherwise unremarkable results, including PT 22.70 s, PT% 36, albumin 38.5 g/L, total protein 59.50 g/L, TBIL 41.6 μmol/L, DBIL 7.10 μmol/L, IBIL 34.50 μmol/L, LDH 243 U/L, and NT-pro BNP 1906 pg/ml. ECG showed paroxysmal AF with a ventricular rate of 73 bpm. Carotid Doppler ultrasonography showed hypoechoic plaques on the anterior wall of the bifurcation of the right common carotid artery (Table ).
The intravenous valproate sodium was discontinued at 44 h after admission, and a total dosage of which was approximately 2200 mg. Although seizure ceased from the start of VPA infusion, 18 h of video-monitoring electroencephalogram (EEG) commenced 5 h later. This demonstrated global interictal θ waves (4-7 Hz), but without epileptiform discharges.
On the 3rd hospital day, the patient developed a renal dysfunction based on the results of BUN 13.74 mmol/l and serum creatinine 126 μmol/l, which was attributed to insufficient fluid intake. Coincidentally, the INR was found to be 8.26, and INR 9 h later was 5.52. Oral warfarin was paused but was mistakenly given again 6 h later by a caregiver. A third INR revealed an almost identical value of 5.32. Following consultation with a hematologist, 5 mg of vitamin K1 was given intravenously and the INR 8 h later was 2.16. The patient remained asymptomatic and without evident bleeding. A repeat brain CT scan revealed no intracranial hemorrhage. The renal dysfunction was corrected on the 4th day. The repeat BUN and serum creatinine were 9.33 mmol/l and 79 μmol/l, respectively. Based on the observation of an INR of 2.16, warfarin was carefully restarted with a dose of 1.25 mg and titrated back to 1.875 mg with steady monitoring of the INR to ensure a value between 2 and 3 (Table ).
|
pmc-6167848-1
|
A Chinese 15-month-old boy presented to the Urology department of a tertiary care center with right flank pain. He had no hypertension or fever. In addition, there were no urinary symptoms such as crying or straining during micturition, frequent urination, or hematuria for the child. According to the history taken from his parents, the pregnancy was uneventful, with no significant antenatal and postnatal history. His birth weight was 2.9 kg, height 51 cm, and head circumference 36 cm. Past medical, environmental, and family history were also not significant. There were no congenital anomalies in the family. He had no siblings. On examination, his pulse rate was 115 beats per minute, body temperature 36.7 °C, respiration rate 28 breaths per minute, and blood pressure 95/46 mmHg. There was no tenderness and no mass palpable in his flank.
The abdominal ultrasound from the local hospital revealed multiple cystic lesions in both kidneys. Magnetic resonance urography (MRU) indicated the possibility of a renal cyst (right; Fig. ) and calyceal diverticula (left); a parapelvic cyst was not excluded. For further evaluation, retrograde pyelography was done during an operation (Fig. ). At last, the child was reliably diagnosed as having right renal cyst and calyceal diverticula on the other side. No significant abnormality was found in the results of laboratory findings, including complete blood count (CBC), liver and renal functions, urine analysis, serology, and microbiology. (Table ).
A robotic-assisted procedure was planned. On September 1, 2016, the procedure was performed using a three-arm da Vinci Robot, standard version, starting from the right side. Our patient was secured in a flank position with the table slightly bent. Regarding the port placement, five ports in the abdomen region were placed for the procedure, with a 12.0 mm camera port, two 8.0 mm robotic ports, and a 12.0 mm and 5.0 mm assistant port (Fig. ). Subsequently, the robot was docked over the shoulder with the camera oriented in accordance with the kidney. The robotic grasper in Arm 1 for retraction and monopolar scissors in Arm 2 were used under the camera guidance with 30-degree down-scope. The ascending colon was mobilized and reflected medially to expose the kidney. After carefully clearing away the perinephric fat to expose the cyst, it was incised and fluid was carefully aspirated. Most of the cyst wall was excised and sent for histopathological examination (Fig. ). This was followed by contralateral procedure on right side in same sitting. The calyceal diverticula was found using the same previously reported procedures and unroofed completely without renal arterial clamping. Renorrhaphy was performed in two layers using sliding-clip technique. Surgical drains were placed on both sides. Operating time was 90 minutes. Postoperative drainage from two drains was approximately 50 cc. No prophylactic antibiotics were given before surgery. Intravenously administered cefodizime (1 g, twice a day) was given 48 hours after surgery for prophylactic purpose. His postoperative period was uneventful and he was discharged on day five postoperatively. With 1-year follow up after the surgery, the child did not have any complaints, and postoperative ultrasound images showed no hydrops or diverticulum associated with either kidney.
|
pmc-6167873-1
|
A 38 year-old man with vitiligo and hypothyroidism initially presented in 2011 with gross hematuria. Diagnostic imaging (Fig. ) revealed a 6-cm renal mass concerning for malignancy, for which he underwent a right radical nephrectomy at the recommendation of his treating urologic oncologist (WCH). Gross pathology (Fig. ) revealed a 6 × 5-cm encapsulated hilar mass with hemorrhage and central necrosis. The mass was limited to the renal parenchyma, without evidence of renal sinus or vascular invasion, and surgical margins were negative for tumor cells. Histologic sections (Fig. ) demonstrated sheets of epithelioid cells with sarcomatoid and rhabdoid features as well as round, polygonal cells with pleomorphic nuclei and prominent nucleoli. Mitotic figures were visualized at a rate of approximately three per high-powered field. Immunohistochemical staining (Fig. -) revealed tumor cell positivity for: HMB45, melan-A, carbonic anhydrase IX, and to a lesser extent, Cam5.2, vimentin and SMA (cytoplasmic), and negativity for: EMA, keratins (AE1/3), CK7, CK20, P63, Pax-2, AMACAR, S-100, and CD10. Based on these histo-pathologic features, the patient was diagnosed with primary EAML.
The patient had an uneventful course for the next 3 years until April, 2014, when surveillance imaging detected an asymptomatic 13-cm renal fossa mass for which he underwent repeat surgical resection. Surgical pathology confirmed recurrent EAML, again with negative margins. The patient’s tumor recurred again in October, 2014, prompting a third surgical resection. Pathologic evaluation this time demonstrated indeterminate margins, prompted referral to medical oncology for further management.
December, 2014 surveillance imaging obtained by the treating medical oncologist (AVB) demonstrated new retroperitoneal and pelvic implants consistent with metastatic EAML. The patient’s tumor DNA was subjected to FoundationOne® targeted next-generation sequencing [], which revealed four oncogenic alterations: truncating mutations in TP53 and APC, a frameshift mutation in ATRX, and a deletion in TSC2, specifically, TSC2 H1746_R1751del, which has been reported both as a somatic variant in AML [] and as a germline mutation in TSC []. Of note, the FoundationOne® assay demonstrated no genomic alterations in the four genes encoding key DNA mismatch repair proteins: MSH2, MSH6, PMS2, or MLH1.
Based on the TSC2 deletion, the patient was initiated on everolimus in January, 2015. Imaging at 3 months (Fig. ) demonstrated marked decrease in size of the majority of the soft tissue masses throughout the right nephrectomy bed, retroperitoneum, and mesentery, and no new sites of disease. The patient remained clinically asymptomatic for 8 months, until he noted unintended weight loss in September, 2015. Imaging demonstrated slight enlargement of the dominant right renal fossa mass (Fig. ), which in the context of progressive anemia, was interpreted as disease progression. Everolimus was discontinued, and the patient was referred for a treatment-directed biopsy for consideration of a clinical trial.
He underwent a biopsy of the dominant 6 cm retroperitoneal mass, from which DNA was isolated and subjected to paired tumor-germline next-generation sequencing via MSK-IMPACT [], which confirmed the absence of a TSC2 germline mutation. However, no new somatic variants were identified to explain the tumors’ acquired resistance, and he was not eligible for any clinical trials. Given the well-known activity of anti-PD-1 checkpoint inhibition across a range of advanced solid tumors, including renal cell carcinoma [], the patient was offered a trial of off-label nivolumab via the Bristol-Myers Squibb Expanded Access program, and he began treatment in October, 2015. After two cycles of nivolumab (administered at 3 mg/kg IV every 2 weeks), the patient felt well, and resolution of his anemia suggested possible clinical benefit. Imaging after 5 cycles demonstrated responding disease (Fig. ).
Nivolumab was well-tolerated, with the exception of immune-related exacerbation of pre-existing hypothyroidism (Fig. -) after 2 months of therapy, and immune-related pruritic cutaneous eruption predominantly within areas of pre-existing vitiligo (Fig. -), occurring after 18 months of treatment. Over the course of nivolumab therapy, the patient required increasing doses of levothyroxine to maintain a euthyroid state. An archival thyroid ultrasound reveals an enlarged heterogenous thyroid gland suggestive of possible autoimmune thyroiditis. With regard to cutaneous toxicity, the patient was referred to dermatology (ANF), and a skin biopsy was obtained of an involved area on the upper back. Histologic evaluation by the dermatopathologist (SAM) revealed a thin granular layer, confluent parakeratosis with collections of neutrophils, and dilated capillaries throughout the papillary dermis, consistent with psoriasis (Fig. -).
Although these toxicities were not dose-limiting, the patient had already completed 2 years of therapy [], and nivolumab was therefore discontinued. The most recent imaging at the time of discontinuation in November, 2017 demonstrates continued response and interval calcification of his intra-abdominal tumors (Fig. ). Archival tissue derived from the initial surgical resection was analyzed for PD-L1 and CD8 expression via immunohistochemistry (Fig. ) utilizing a modified Agilent/Dako 22C3 pharmaDX kit, revealing high PD-L1 expression (> 50% of cells) and a brisk CD8+ T cell infiltrate. Finally, immunohistochemical analysis demonstrated preserved expression of key DNA mismatch repair (MMR) proteins MSH2, MSH6, PMS2, and MLH1 [], thus confirming MMR proficiency (Fig. ).
|
pmc-6167882-1
|
An 18-months-old twin girl was referred to our hospital for evaluation of fever of unknown origin in January 2014. She had previously been seen by her paediatrician for daily fever up to 40 °C for one week. Empirical treatment for presumed bacterial infection with a first-generation cephalosporin did not lead to defervescence. No other symptoms including cough, vomiting, diarrhoea, skin rash or weight loss were reported. There were no sick contacts or exposure to pets. The girls’ previous medical history was unremarkable. She had travelled to Tuscany for a three-week holiday six months earlier. The girl’s twin sister, two older siblings, aged four and six years and the parents were well at the time of her presentation.
On physical examination, the girl’s weight and height was 9.9 kg (10th percentile) and 84 cm (75th percentile). She was pale and febrile (38.9 °C), without a focus of infection on clinical examination. Splenomegaly noted on clinical examination was confirmed by ultrasound with a spleen size of 9.9 cm (normal size for age < 9 cm). Her chest radiography was normal. Laboratory investigations (normal values in brackets) showed: haemoglobin 75 (105–135) g/l, platelets 50 (150–450) × 109/l, white blood cells 2.6 (6–17.5) × 109/l with 1.75% of suspected atypical cells, C- reactive protein (CRP) 73 (< 5) mg/l and erythrocyte sedimentation rate (ESR) of 47 (3–13) mm/h. Liver function tests were abnormal for aspartate aminotransferase (ASAT) 145 (26–55) U/L, alanine aminotransferase (ALAT) 80 (9–15) U/L, lactatdehydrogenase (LDH) 1096 (< 338) U/l, and normal for gamma-glutamyltransferase (GGT) and alkaline phosphatase (AP). Renal function tests were normal. Serology was negative for cytomegalovirus, parvovirus B19, Epstein-Bar virus, human herpes virus 8, human immunodeficiency virus (HIV), hepatitis A and toxoplasmosis. Further investigations for haemophagocytic lymphohistiocytosis (HLH) showed a ferritin of 9667 (9–107) μg/l, triglycerides of 1.57 (0,7-0,8) mmol/l and interleukin 2-receptor levels of 7950 (< 800) IU/ml. For suspicion of leukaemia or HLH, a bone marrow aspiration was performed which showed a normal distribution of lymphocyte subsets with predominant T-cells but revealed intracytoplasmic localized Leishmania parasites (Fig. ).
Serology for leishmaniasis was strongly positive with an antibody titre of 1:1280 (cut-off < 1:80) and was performed with an in-house immunofluorescence antibody test (IFA) using liver sections of Leishmania donovani infected hamster. In brief, diluted serum was applied to the sections, counter stained with a fluorescein isothiocyanate-labeled conjugate and examined with a fluorescence microscope. Antibodies elicited by all members of the L. donovani complex are cross-reactive to the L. donovani IFA. Identification and differentiation of Leishmania spp. was performed from a bone marrow aspirate by polymerase chain reaction amplification of the Miniexon with subsequent sequencing and revealed infection with Leishmania infantum [, ].
Treatment with liposomal amphotericin B was initiated with 5 mg/kg on the first day, followed by 3 mg/kg on each day 2–4 and 10. She received a red blood cell transfusion on day three. The girl tolerated the treatment well and her clinical condition gradually improved. She defeveresced after the fourth treatment dose and was discharged on day 7 with the fifth dose (day 10) administered at the district hospital. At follow-up 1.5 years after diagnosis the girl was well with Leishmania IFA titre of 1:320.
Nine month later (in September 2014), the twin-sister developed fever up to 40 °C with rhinitis. There were no sick contacts. As her sister, she had travelled to Tuscany for a three-weeks holiday 16 months earlier but not returned to Tuscany afterwards or travelled to any other country considered endemic for visceral leishmaniasis. Laboratory investigations at the district hospital showed a haemoglobin of 79 g/l, platelets of 60 × 109/l, white blood cells of 4.9 × 109/l and an elevated LDH of 398 IU/l. Because of pancytopenia and the twin sisters’ previous diagnosis, the girl was referred to our hospital.
On physical examination the girl’s weight was 12.2 kg (25th percentile) and height was 89 cm (50th percentile). She was afebrile and presented with splenomegaly of 11.2 cm in an abdominal ultrasound. Further laboratory investigations showed a CRP of 67 mg/l, normal liver and renal parameters, and negative serology for HIV. Leishmania IFA was positive with a titre of 1: 1280 (in-house L. donovani IFA as described above) and polymerase chain reaction amplification of the Miniexon sequence from blood confirmed infection with L. infantum.
Treatment with liposomal amphotericin B (5 mg/kg/d) was started on the first day followed by 3 mg/kg/d each on day 2–5, 10 and 21. She received a red blood cell transfusion on day 3 because of further haemoglobin decline to 67 g/l. Thrombocytes and white blood cells increased on day four of admission and the girl was discharged on day six with subsequent doses given as an outpatient. At the follow-up 1 year after diagnosis the girl was thriving well and the Leishmania IFA had decreased to 1:320.
Testing of the remaining asymptomatic family members revealed a Leishmania IFA of 1:640 for the father (in-house L. donovani IFA as described above). He remained asymptomatic, did not receive treatment but is regularly followed the at the outpatient clinic of the Swiss Tropical and Public Health Institute in Basel. The mother and the two older siblings remained asymptomatic with negative Leishmania IFA test results.
|
pmc-6167906-1
|
A 65-year-old female presents to clinic with 1 week cough. She denies fever, dyspnea, chest pain or constitutional symptoms. She had a history of type II diabetes but with an inadequate controlled serum glucose level for 10 years. Her neutrophils, C-reaction protein (CRP), procalcitonin and tumor markers such as carcinoembryonic antigen (CEA), neuron-specific enolase (NSE), squamous cell carcinoma antigen (SCC) were all in normal range. Her T-SPOT.TB assay examination were negative. A CT scan of her chest reveals a 25–29 mm, round-like, heterogeneous lobulated solitary pulmonary nodule without showing any cavity lesions or calcification, in the peripheral right lower lobe. No obvious enlarged hilar or mediastinal lymph nodes were observed (Fig. ). Preoperative brain magnetic resonance imaging (MRI) scans showed negative findings. The patient hadn’t received PET/CT scan for economic reasons.
The patient received lobectomy of right lower lobe and systemic mediastinal lymph nodes dissection. A gross examination revealed a 3.5 × 2 × 1.5 cm, well-demarcated, white-pan solid mass. Microscopically, the tumor was composed of spindle cells with a whorl pattern. No atypical cells and mitotic figures were observed (Fig. and ). Immunohistochemically, the tumor cells were positive for vimentine, EMA, PR (progesterone recepters), CD34, D2–40, S100 (focal) and 2% tumor cells positive for Ki-67 (Fig. –). The tumor cells were negative for synaptophysin, SMA (smooth muscle actin), TTF-1 (thyroid transcription factor-1), Chromogranin A, desmin, GFAP, WT-1 (Wilm’s tumor gene-1), HBME-1 (Hector Battifora mesothelial epitope-1). Based on the above findings, the tumor was diagnosed as primary pulmonary meningioma (PPM).
|
pmc-6168050-1
|
The first patient is a 64-year-old female with a history of CD of the colon and small bowel and cirrhosis secondary to autoimmune hepatitis (AIH). She was diagnosed with CD 13 years earlier and had failed treatment with certolizumab and infliximab, thus requiring a colectomy with end ileostomy. She continued to be symptomatic with significant ileostomy output and arthropathy, and ileoscopic findings were consistent with persistent small bowel CD; therefore, vedolizumab therapy was considered. Prior to starting treatment, she had a modified model for end-stage liver disease (MELD-Na) score of eight and a Child Turcott Pugh (CTP) score of A5. She was started on vedolizumab 300 mg every eight weeks and azathioprine 100 mg daily. Thirty-six months after treatment, she is doing well with mucosal healing on follow-up ileoscopy. The patient has not had any complications related to her cirrhosis and continues to have well-preserved liver function. Her MELD-Na and CTP scores remain unchanged. Surveillance imaging for hepatocellular carcinoma has been negative, and she has not required hospitalization for infectious complications.
|
pmc-6168050-2
|
Our second case is of a 57-year-old male with a history of small bowel CD and cirrhosis due to chronic hepatitis C infection. Cirrhosis was confirmed by liver biopsy and the patient achieved a sustained virologic response after completing therapy with interferon and ribavirin. He has a five-year history of CD involving the small bowel, had undergone a prior resection of the distal ileum, and has previously been treated with budesonide and 6-mercaptopurine (6-MP). Due to persistent clinical and endoscopic disease in the neoterminal ileum, vedolizumab therapy was considered. Prior to therapy, his MELD-Na score was six and CTP A5. Thirty-two months post treatment, the patient continues on vedolizumab 300 mg every eight weeks and his CD is in clinical remission. He has not had any episodes of hepatic decompensation and his post-treatment MELD-Na score is seven and CTP A6. He has not had any infectious complications, nor has he developed a hepatic or an extra-hepatic malignancy.
The last case is of a 65-year-old male with an 11-year history of small bowel CD and cryptogenic cirrhosis. He was initially treated with steroids, infliximab, and azathioprine; however, due to non-response, vedolizumab therapy was considered. Prior to treatment, his MELD-Na was nine and CTP A6. After initiation of vedolizumab 300 mg every eight weeks for five months, the patient’s MELD-Na was 11 and CTP A6 with no decompensated cirrhosis or significant infection.
|
pmc-6168053-1
|
A 37-year-old male with no past medical history presented to the emergency room complaining of a two-week history of an enlarging right testicular mass and a three-day history of aching groin pain. A review of systems at admission was negative. A physical examination revealed diffuse enlargement of the right testicle, which was firm and tender to palpation.
Basic laboratory evaluation with a complete blood count (CBC) and complete metabolic panel (CMP) was unremarkable. Lactate dehydrogenase was elevated to 697 U/L, alpha fetoprotein was <2.5 ng/ml and β-hCG was over 278,800 mIU/ml. An ultrasound was performed, which showed an enlarged hypoechoic right testicle measuring 8.9 cm x 5.8 cm x 6.3 cm. A computed tomography (CT) scan of the abdomen and pelvis revealed a right scrotal mass with features consistent with malignancy, nonspecific splenic lesions, retroperitoneal soft tissue implants, and large bilateral pulmonary masses, which were further characterized with a CT scan of the chest (Figures -).
Following subspecialty evaluation, the patient underwent a right radical orchiectomy as well as a port-a-cath placement for chemotherapy. Pathology of the testicle revealed 100% choriocarcinoma invading the epididymis as evidenced by extensive parenchymal replacement by biphasic pattern of syncytiotrophoblasts and cytotrophoblasts. β-hCG and keratin AE1/AE3 were positive, and markers CD30, CD117, and OCT4 were negative (Figure ). It was decided that the patient would be started on bleomycin/etoposide/cisplatin (BEP) therapy, and he was discharged with an appointment to begin treatment as an outpatient.
Ten days later, the patient was found unconscious at home. According to friends, he had been acting unusual for the past few days, was nauseous, and had several episodes of emesis and one episode of hematemesis. He had also started complaining of a bilateral occipital headache radiating to the frontal region. On physical examination, the patient had a flat affect, was slow to respond, and was acting unusual. A CT scan of the head showed multiple bilateral supratentorial hemorrhagic masses, with the largest mass in the right frontal lobe resulting in midline shift. The patient was admitted to the intensive care unit for close monitoring and treated with mannitol, blood pressure control, and hypertonic saline. Neurosurgery evaluated the patient and decided to hold off on surgical intervention given his intact neurological function. Chemotherapy was then initiated. After one dose of cisplatin, the patient became increasingly altered. Magnetic resonance imaging (MRI) of the brain revealed subfalcine herniation and effacement of the basilar cisterns with slight uncal herniation, in addition to multiple hemorrhagic lesions, the largest of which was in the right frontal lobe (Figure ). The patient underwent a right frontal craniotomy with removal of the largest mass.
The patient then developed anemia and started having melanotic stool. An esophagogastroduodenoscopy was performed, which showed only mild gastritis and a hiatal hernia. Pathology revealed gastritis and reactive gastropathy. A colonoscopy was noted to have blood in the terminal ileum but was otherwise unremarkable. A push enteroscopy revealed a small ulcerated mass in the jejunum. Two hemoclips were placed there. The enteroscope could not be advanced any further, and it was decided that an angiogram would be performed if the hemoglobin continued to drop or his gastrointestinal bleeding persisted. However, after enteroscopy, his bleeding resolved and hemoglobin levels stabilized.
The patient subsequently developed several clustered erythematous-violaceous firm subcutaneous nodules without ulceration or other epidermal changes on the zygoma, with a larger nodule on the anterior neck and right posterior shoulder. A punch biopsy was performed on the shoulder lesion and showed findings consistent with choriocarcinoma with small nests and cords of epithelioid cells with abundant cytoplasm and enlarged highly atypical and pleomorphic nuclei (Figure ). The patient completed cycle 1 of BEP and then elected to move to be closer to his family.
|
pmc-6168054-1
|
A 72-year-old female presented to the emergency room with the complaint of left thigh pain and the inability to walk after tumbling. She had been suffering from osteoarthritis of the left hip but was able to walk with a cane and sit on a couch. She had also received the diagnosis of osteoporosis and had been taking alendronate 35 mg weekly for six years. The initial radiograph in the emergency room showed a non-comminuted subtrochanteric fracture of the left femur with cortical thickness and the beaking of the lateral cortex at the fracture site. The fracture line was transverse on the lateral side and oblique on the medial side. The radiographic findings and the weakness of the force that caused the fracture satisfied the criteria for an atypical fracture of the femur. The radiograph showed Kellgren-Lawrence grade 4 osteoarthritis of the left hip as well, with superomedial migration, the external rotation of the femoral head, and the shortening of the femoral neck (Figure ).
Osteosynthesis with intramedullary nailing was planned. However, the deformity of the hip joint appeared to preclude correct nail insertion. Furthermore, whenever we used a fracture table in the past, we often observed that the perineal post of the fracture table impeded the adduction of the proximal fragment of the subtrochanteric fracture while the whole leg was being adducted, resulting in varus malalignment. On the other hand, we were aware of easy access to the entry point via the standard lateral decubitus position without using the perineal post. For these reasons, we decided to fix the fracture with a nail in the lateral decubitus position. Total hip arthroplasty with fracture fixation was not chosen because of its invasiveness, which was inappropriate for the rather mild, pre-injury symptoms due to osteoarthritis, and because of the patient’s wish to preserve the femoral head.
In surgery, a flat, radiolucent operating table was used. The whole injured leg was sterilized and draped, and the hip was slightly flexed. The C-arm was placed at the ventral side of the patient. Although the proximal fragment was externally rotated, the entry point was located easily by adjusting the C-arm to obtain a correct A-P view. A guide pin was inserted in line with the long axis of the proximal fragment to the level of the fracture without any interference by the torso (Figure ).
Then, the fracture was reduced with gentle manual traction of the thigh followed by the distal insertion of the guide pin. At this time, the fracture was perfectly aligned (Figure ).
After reaming, a trochanteric-entry cephalomedullary nail (Trigen Trochanteric Antegrade Nail, Smith & Nephew, Massachusetts,
US), 11.5 mm in diameter, was inserted, again, without any obstruction by the torso. After assuring rotational alignment, two cephalomedullary screws and a distal interlocking screw were inserted. Accurate, non-varus reduction was confirmed by a postoperative radiograph (Figure ).
The operating time was 122 minutes. The total blood loss was 86 ml. Toe-touch weight bearing was initiated soon and full weight bearing was allowed in five weeks. A solid union was confirmed by radiograph after 10 months (Figure ). She was able to walk with a cane as she did before her injury without a worsening of the pain. Alendronate was discontinued after the injury and replaced with vitamin D supplements. No anabolic agent, such as teriparatide, was administered.
|
pmc-6168055-1
|
A 78-year-old African-American man weighing 53 kilograms with a past medical history significant for chronic lymphocytic leukemia (CLL), hyperuricemia with chronic gouty arthropathy, chronic kidney disease (CKD) stage 3 presented to the Emergency Department (ED) with the chief complaint of shortness of breath and fatigue for one day. His outpatient medication included ibrutinib 420 milligrams (mg) and allopurinol 300 mg daily. He was diagnosed with CLL two years prior to presentation. Initially, he was started on bendamustine with an appropriate response. However, an escalation in his lymphocyte count was appreciated one month prior to presentation and ibrutinib was initiated for CLL progression. One day prior to admission to the hospital, the patient was evaluated by his primary oncologist at an outpatient visit. He was febrile with a temperature of 101.6 degrees fahrenheit (°F) but otherwise asymptomatic. Blood and urine cultures were obtained and was started on oral levofloxacin empirically. His labs were significant for worsening hyperuricemia with a uric acid level of 13.0 milligram per deciliter (mg/dl). A single dose of intravenous rasburicase 3 mg was then administered.The following day, he presented to the ED complaining of significant fatigue associated with dry nonproductive cough of one day duration. Examination was remarkable for a fever of 102.8°F, an oxygen saturation (SPO2) of 85% on room air, conjunctival pallor and scleral icterus. A left lower lobe infiltrate was observed on his computed tomography (CT) angiogram of the chest (Figure ). Laboratory results were noteworthy for a drop-in hemoglobin (4.9 mg/dL), indirect hyperbilirubinemia (7.2 mg/dL), low haptoglobin (<10 mg/dL) and elevated lactate dehydrogenase of 1611 International Units per liter (IU/L) compared to (756 IU/L) the day before; depicting acute hemolytic anemia. SPO2 persisted at 85% despite the use of nasal cannula, nonrebreather and non-invasive positive pressure ventilation. The patient was admitted to the intensive care unit (ICU). Arterial blood gas on 50% fraction of inspired oxygen (FiO2) was significant for an arterial oxygen level (PO2) of 222 millimeters mercury (mm Hg) and an oxyhemoglobin of 85.9%. Co-oximetry was then obtained and methemoglobin level was 13.4%. The patient was started on vancomycin and cefepime and transfused with three units of packed red blood cells. Methylene blue was not administered as the patient was assumed to have G6PD deficiency given his race and this episode of hemolysis.
After an ICU stay of two days, the patient clinically improved, SpO2 normalized, his hemoglobin levels improved, methemoglobin levels trended down and was transferred back to the floor (Table ). A G6PD level sent during the acute attack yielded a result within the normal range. In addition, mycoplasma serum antibodies were negative. Despite broad spectrum antibiotic coverage, the patient continued to spike fevers as high as 102.7°F from day one to day four. He subsequently started complaining of right knee pain associated with right knee swelling and tenderness on examination. Arthrocentesis revealed monosodium urate crystals and was started on colchicine and prednisone. Fever, knee pain, swelling and tenderness resolved. The patient was discharged to a skilled nursing facility on prednisone taper, allopurinol and colchicine. He was continued on ibrutinib to date (five months later) and his white blood cell count is currently within normal limits. A repeat CT chest performed three monthts later documented resolution of the previously seen left lower lobe pulmonary inifiltrate.
|
pmc-6168519-1
|
A 45-year-old Brazilian gentleman presented to KRC at a movement disorders clinic with a potential diagnosis of ‘severe’ PD. Enquiry revealed that he developed late-onset hyposmia when aged about 35 years and also showed signs of unilateral bradykinesia about the same time. He had an introverted and anxious personality. He was seen in several clinics where initially a diagnosis of PD was considered and he was advised to take levodopa because of severe akinesia, rest tremor and a poor quality of life. However, the patient had severe anxiety about taking levodopa based on information provided by his father who also refused to allow him to try levodopa. When seen in 2016 in London, he had bilateral severe akinesia with an almost unintelligible speech, bradyphrenia, dribbling of saliva, paroxysmal rest tremor, high non-motor symptoms questionnaire score. He was ambulant on a wheelchair with upper limb contractures, although he could walk when asked. The patient and father were sceptical about dopamine loss and a Datscan was performed and confirmed severe presynaptic dopamine transporter loss with putamen-binding ratios being < 1 (right 0.64, left 0.5) (Fig. ). ‘Levodopa Phobia’ was diagnosed and a neuropsychological ‘anti-phobia’ support programme was initiated. After several weeks the patient and the father agreed to the use of levodopa, which was started at a daily dose of 150 mg increasing to 300 mg with good motor and non-motor response. At 1-year follow-up the patient is able to perform many activities of daily living.
|
pmc-6168519-2
|
A 48-year-old female media journalist in the United Kingdom, who was extremely well read about PD developed right-sided slowness of movement noticed when using her laptop and general slowness that was commented upon by her boyfriend and media colleagues. She also had marked fatigue and herself considered a diagnosis of PD. She then self-referred herself to KRC for further advise and management. She was felt to need immediate dopamine replacement therapy and levodopa was likely to be the most suitable drug given her bradykinesia and postural instability. However, she had severe ‘Levodopa Phobia’ being afraid about dyskinesias, which she had seen in people with PD as reported in the media and also by being member of several PD patient groups. She had also read some papers that had suggested levodopa is toxic to brain cells in animal models. As such initiation with levodopa was impossible and she was started on a dopamine agonist along with rasagiline. After 1 year she presented to the clinic with recurring bradykinesia interfering with her media work. However, even inspite of the bradykinesia and related issues she was not convinced about the need for levodopa to be started and wanted to stay on the same dose of treatment she was on. She tried various forms of complimentary medicine (herbal therapy, acupuncture, swimming and nutritional supplements) with no beneficial effect. After several consultations, a decision to use a wearable wristwatch sensor was made to document her bradykinesia in an objective manner. The report confirmed severe bradykinesia and she finally agreed to take levodopa after seeing the extent of bradykinesia but refused to go beyond 300 mg of levodopa per day. As a result, after an initial response to levodopa, bradykinesia remained a major problem and there were continuing issues related to her work in the media world.
|
pmc-6168664-1
|
A 55-years-old Caucasian male patient was diagnosed with a stage IVB head and neck squamous cell carcinoma (HNSCC) in May 2015. He was a heavy smoker and social drinker with no other significant medical history, and was initially treated with concurrent cisplatin-based chemoradiotherapy.
On routine follow-up visit in September 2016, Computed Tomography (CT) scans showed lung metastases. The patient was enrolled in a clinical trial and was randomized to pembrolizumab monotherapy every 3 weeks. Following the first two cycles of immunotherapy, the patient presented with stiffness, swelling and pain of the right knee. Physical examination showed inflammatory monoarthritis, with diffuse swelling and tenderness of the right knee. Laboratory tests were remarkable for an elevated erythrocyte sedimentation rate (ESR, 40 mm/h) and C-reactive protein (CRP, 50 mg/L); rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies were negative and serum uric acid was normal. Following rheumatologic consultation, knee joint aspiration was performed, and synovial fluid (SF) analysis revealed a yellow, cloudy appearance, decreased viscosity and a cell count of 7040 cells/mm3 with 80% neutrophils, indicating an inflammatory arthritis. SF cultures were sterile and no crystals were found on microscopy. The patient was initially treated with prednisone 5 mg twice a day with significant improvement over the following days. Inflammatory arthritis was attributed to pembrolizumab therapy and the third cycle was eventually postponed. Importantly, restaging imaging at that timepoint showed complete response of the disease.
Following reinstitution of pembrolizumab therapy, bilateral arthritis of the knees, accompanied by arthritis of interphalangeal joints of both hands (Figure ), developed after the fifth cycle, Ultrasound of the knees showed evidence of active synovitis (Figure ) and a diagnosis of inflammatory polyarthritis was established. The patient was retreated with prednisone 5 mg twice a day and pembrolizumab therapy was interrupted. Due to the patient's clinical deterioration, and because protocol limitations did not allow increase of prednisone dose or administration of immunomodulatory drugs, pembrolizumab was permanently discontinued. Of note, the patient remained in complete remission. Methotrexate 7.5 mg po as a single weekly dose was added to control synovial inflammation and, following also pembrolizumab discontinuation, the patient's symptoms gradually improved.
A written informed consent was obtained from the patient for the publication of this case report.
|
pmc-6168754-1
|
A 26 years old primigravida unbooked case belonging to lower socioeconomic status, presented in antenatal clinic with 23 weeks of pregnancy with distension of abdomen. She had past history of left salpingo-oopherectomy done two years back for left ovarian mucinous adenocarcinoma. Postoperatively, she had received six cycles of combined chemotherapy with carboplatin and paclitaxel and was advised contraception. Subsequently she was lost to follow up until 23 weeks of period of gestation. Per abdomen examination revealed ascites and 24 weeks size uterus. with palpable fetal parts.CA-125 was 80 IU/ml. Ultrasound examination revealed intra-uterine pregnancy of23 weeks anda right ovarian mass of 12.8 x8.8 cm with solid and cystic areas containing free fluid with internal echoes. Diagnosis of recurrent ovarian cancer in pregnancy was made and she was advised surgical management which was declined by the patient. Hence two courses of chemotherapy with single dose carboplatin 450mg i.v. was started at three weekly intervals. However, third course of chemotherapy could not be administered due to derange liver functions tests. In spite of chemotherapy, ascites was progressive, leading to maternal respiratory distress and fetal growth restriction and oligohydroamnios ensued. Hence, elective Cesarean section with staging laparotomy was planned at 34 weeks for breech presentation with decreased liquor and gross ascites, after completion of steroid cover. Intraoperatively, 20litersof hemorrhagic ascetic fluid was drained, omentum was agglutinated over intestine and deposits were present over uterus. Right ovary was replaced by a tumor of 15x15cm with rupture and hemorrhage ().
A live healthy baby girl 2.3kg with APGAR 6/10 and 8/10 at 1 and 5minute respectively was delivered. The placenta appeared normal at the time of delivery. Total abdominal hysterectomy with right salpingo-oopherectomy, infracolic omentectomy, and appendectomy was done. Peritoneal washings were collected and multiple peritoneal biopsies were taken. Palpation of pelvic and para-aortic lymph nodes was negative. The patient had an unremarkable postoperative course. Histopathology revealed mucinous adenocarcinoma of ovary. She was given six cycles of chemotherapy (carboplatin and paclitaxel) commencing from postoperative day 14.At 6 month follow up, mother and baby are healthy.
|
pmc-6168954-1
|
A 60 year-old left-handed female developed progressive behavioral change, speech disturbance and episodic memory impairment. Initially she developed prominent apathy, reduced social interaction, sweet tooth, disinhibition, and new obsessions, followed by reduced speech output and use of stereotypic phrases. Two years after onset she had difficulty remembering recent events and conversations, and got lost in familiar places, then developed dyscalculia. She had occasional visual hallucinations of family members. Her condition rapidly worsened four years after onset and she was admitted to a nursing home with impaired spatial awareness, prosopagnosia, wandering and incontinence. She later developed an asymmetrical extrapyramidal syndrome with dystonic limb movements, worse on the right. She died aged 68 with a final clinical diagnosis of behavioral variant FTD. The patient donated her brain for post-mortem analysis to the Queen Square Brain Bank for Neurological Disorders (QSBB) at University College London Institute of Neurology.
The patient’s medical history included progressive right-sided facial hemi-atrophy, diagnosed 15 years prior to onset of cognitive symptoms, with no cause identified. Two years after onset of cognitive symptoms she developed a persistent cough and peripheral eosinophilia, pericardial thickening and an anterior pericardial effusion. The cause of these symptoms was not identified despite thorough investigation, but was felt to be autoimmune in origin. There were no vascular risk factors, no prior smoking history, and consistently normal blood pressure. Her mother had developed acute psychosis with delusions and odd behavior aged 65, followed by progressive cognitive decline and admission to a mental health unit for 20 years, with an eventual diagnosis of dementia. There was no other family history of dementia or neurological or vascular disease.
Investigations included a volumetric 1.5 T MRI brain performed 2.6 years before the patient died. This showed bilateral but asymmetric frontal, temporal, and parietal atrophy, worse on the left, and significant bilateral periventricular WMH, worst in the frontal lobes and on the left side. Cerebrospinal fluid analyses were normal or negative. A wide variety of blood tests were normal, except for peripheral eosinophilia of 1.11 (range 0.0–0.4 x 109/L) and positive rheumatoid positive particle agglutination test (1/80 titer). DNA analysis identified a heterozygous GRN Q130fs mutation but no other FTD-associated mutations.
|
pmc-6168996-1
|
A 66-year-old female with known VHL disease was referred for ocular evaluation. Systemically, she had cerebellar and midbrain hemangioblastomas. She had prior trauma to her left eye 40 years earlier for which she had undergone evisceration. She had open-angle glaucoma in the right eye for which she was using latanoprost and timolol maleate once daily.
On presentation, visual acuity was 20/60 in the right eye with intraocular pressure of 15 mmHg. The anterior segment examination of the right eye was unremarkable. On funduscopic examination, a VHL-related retinal hemangioblastoma was seen superotemporal to the macula associated with dilated feeding and draining vessels (Fig. ). There was no peripheral retinal detachment, but there was intraretinal fluid extending from the hemangioblastoma towards the temporal macula seen by optical coherence tomography (Fig. ).
Widefield fluorescein angiography was performed, which showed fluorescein uptake and leakage from the hemangioblastoma and peripheral retinal nonperfusion anterior to the tumor in the superotemporal quadrant. Treatment of the hemangioblastoma and peripheral retinal nonperfusion was recommended; but because this was her only eye, the patient preferred close observation with monitoring for progression.
|
pmc-6169008-1
|
Here we present the case of a 42-year-old man from Western Africa who had been immigrating to Germany recently. Initial admission was due to a seizure and a history of wasting and worsening health condition for six months. Upon admission he presented with recurrent fever, hepatosplenomegaly and acute kidney failure. Laboratory test results revealed a mild pancytopenia (leukocytes 2.28 × 109/L, hemoglobin 6.2 g/dL, platelets 113 × 109/L), increased LDH (702 IU/L) and nephrotic syndrome (proteinuria 12 g/d). Cranial magnet resonance imaging (MRI) presented no pathological findings. Bone marrow histology identified single atypical cells in intravascular position. Due to the rarity of these atypical cells further specification of their nature including immunohistochemistry and molecular techniques was not possible. Cytogenetic testing of the bone marrow aspirate but not the peripheral blood revealed a complex karyotype in single metaphases.
Ultrasound of the abdomen showed hepatosplenomegaly but no lymph node swelling. Further diagnostic approaches included computed tomography (CT) scan of the chest which revealed atypical pulmonary infiltrates and bilateral hilar lymphadenopathy. Transbronchial biopsy was negative for infectious agents, sarcoidosis or malignancy. As bronchial lavage was positive for Aspergillus antigen, the patient was treated with Voriconazole. However, the assumption of pulmonary aspergillosis did not explain all of the patient’s symptoms. Further diagnostic approaches included high positive proteinase 3 titer (1:135) and therefore granulomatosis with polyangiitis was suspected. However, given low platelet counts biopsy of the kidney was not performed.
Considering the clinical symptoms, the patient was treated with high-dose steroid burst. However, upon that the patient’s condition worsened rapidly with further loss of weight and intermittent high fever, despite antibiotic and antifungal treatment. Thus, steroids were withdrawn. Infectious disease testing, including human immunodeficiency virus, Tuberculosis, Schistosomiasis, Malaria, and Leishmaniosis were negative, expect of previous Hepatitis B and EBV infection (anti EBV VCA IgM-ELISA negative, anti EBV VCA-ELISA IgG 11 E/ml; anti EBNA IgG-ELISA 14 E/ml). While no signs of chronic HBV infection were seen, EBV DNA in the peripheral blood reached high levels (2.8 × 106 copies/mL). Along with the clinical symptoms CAEBV was therefore assumed, without evidence of previous immune deficiency. Positron emission tomography (PET)-CT revealed a high glucose uptake in the thyroid, liver, adrenal gland and in the examined bone areas (Fig. ).
Based on PET-CT findings a targeted biopsy of the bone marrow and the thyroid gland were performed to facilitate a definite diagnosis. Histological evaluation of the bone marrow biopsy now revealed a hyperplastic hematopoiesis with prominent erythropoiesis and left shifted myelopoiesis. In addition, in this biopsy conventional histomorphology (Fig. ), underlined by immunohistochemical findings revealed small aggregates of partly intravascular localized, highly proliferated (Ki67 90%) middle–sized lymphoid cells with coexpression of CD3 (Fig. ) and perforin (Fig. ) but missing expression of CD4 and CD8. Additional findings included negativity of the atypical T-cells for CD34, TdT, CD20 and CD56. Performing EBER-in-situ-hybridization active EBV infection within these T-cells was detected (Fig. ). Parallel examination of thyroid gland tissue provided large aggregates of the identical middle-sized highly pleomorphic and proliferated T-cell population with consecutive displacement of thyroid follicles (Fig. -). T-cell receptor clonality analysis by amplification of Vɣ1–11-region und multiple Jɣ-regions of T-cell receptor-gamma-gene provided a polyclonal pattern (Fig. ). Altogether, histological findings along with flow cytometry analysis and molecular data excluded diagnosis of T-cell lymphoma or lymphoblastic leukemia. As hemophagocytic lymphohistiocytosis (HLH) is often associated with CAEBV the clinical criteria for HLH were evaluated but were not fulfilled. Moreover, no typical findings of HLH were seen in the bone marrow biopsy. According to the 2017 WHO classification diagnosis of CAEBV of T/NK-cell type, systemic form was finally made [].
Based on the data by Sawada et al. [], the patient was then treated with methylprednisolone and cyclophosphamide as cooling down step to reduce the high viral load. Given the persisting renal failure we refrained from immunosuppression with cyclosporine A. In the further course the patient developed an epileptic seizure and clinical symptoms of meningitis. To clarify whether these symptoms were related to CAEBV or EBV-associated T-cell LPD a lumbar puncture was performed and cranial MRI was repeated. Indeed, cerebrospinal fluid was positive for EBV DNA but did not include atypical T-cells. Cranial MRI now showed typical radiological signs for viral meningitis. We regarded this as a clinical progression and initiated chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP). Despite its limited activity for EBV-infection we also started treatment with foscavir. Upon this and 2 cycles of CHOP the EBV load decreased remarkably from 2.8 × 106 copies/mL to < 200 copies/mL. An additional cycle of CHOP was administered and the patient remained in complete remission so far (Fig. ).
|
pmc-6169020-1
|
The proband is a 17-year-old Caucasian male who first presented at the neonatal stage with prolonged jaundice with an unconjugated bilirubin level of 400 μmol/L without hemolytic anemia data. Following an uneventful pregnancy, the patient was the first child of non-consanguineous Caucasian parents. Due to jaundice and elevated indirect bilirubin levels, he was diagnosed with GS soon after delivery as he had the A(TA)7TAA allele in homozygous state (identified by fragment analysis as previously described []). During his childhood years, the patient did well. He periodically underwent phenobarbital treatment for the reduction of bilirubin levels associated with jaundice; the mean bilirubin levels achieved as the result of these treatments were 100–200 μmol/L. The patient’s parents had no complaints until puberty.
After reaching puberty, the boy became severely jaundiced and his bilirubin levels increased to 300–350 μmol/L. However, there were no other complaints, he was doing well in school and his weight and height parameters were according to his age. The boy was referred to a hepatologist and multiple investigations were carried out. No pathological findings were observed in the laboratory analysis and hepatitis viral markers were negative. Furthermore, there was no evidence of other inborn errors of metabolism, i.e. organic acidurias and amino acidurias or changes in the fatty acid profile.
The results of abdominal ultrasound and abdominal MRI were unremarkable. Procurement of a percutaneous liver biopsy revealed no pathological findings. Based on isolated elevation of indirect bilirubin levels from standard laboratory investigations, reduced glutathione (0.89 mmol/l; reference value 1.12–1.216 mmol/l), a glucuronide level in 24-h urine of up to 102 mg/ml (reference value 430–600 mg/ml), phenobarbital responsiveness, and no evidence of kernicterus (as the child had no complaints about his health condition), CNS-II was suspected.
To confirm the CNS-II diagnosis, bidirectional sequencing of five exons and exon/intron boundaries of the gene UGT1A1 (OMIM: 191740) was performed using previously described primers [] and a BigDye 3.0 kit (following the manufacturer’s protocol; Applied Biosystems, USA). The nomenclature of the identified variations was identified by using Mutalyzer () and assessed sequentially against the Single Nucleotide Polymorphism Database (dbSNP; ), Exome Aggregation Consortium (ExAC; exac.), ClinVar (), and the UGT1A1 variant database []. A search was performed with MEDLINE if the variation was not found in any of the aforementioned databases. The biological significance of observed nucleotide changes located at splice sites was assessed using the Human Splicing Finder (HSF) 3.0 [] and MutationTaster [].
Four different variants in the UGT1A1 gene were identified in the patient: g.3664A > C (c.1352A > C, rs3755319); g.4963_4964TA[7] (c.-53_-52insTA, A(TA)7TAA, UGT1A1*28, rs8175347); g.5884G > T (c.864 + 5G > T, IVS1 + 5G > T); and g.11895_11898del (c.996+2_996+5del) (reference sequence NG_033238.1) .
In the ClinVar database, the variant g.4963_4964TA[7] is described as a variant affecting response to drug treatment. This is the most common variant identified in patients with GS. The variant g.3664A > C, as reported in the ClinVar database, causes transient familial neonatal hyperbilirubinemia (OMIM: 237900). The variant g.5884G > T located in the first intron has previously been reported in a patient with CNS-II [], and from exome sequencing has been identified only in Europeans in five alleles (exac.). The second intronic variant g.11895_11898del (located in second intron two nucleotides after the second exon) is reported for the first time (sequence showed in the Fig. ). Considering that one of the criteria for evaluating variant pathogenicity is its frequency in the healthy population, 90 healthy individuals from the Genome Database of the Latvian Population (LGDB), a government-funded biobank (the principle of LGDB has previously been reported []), were randomly selected and screened for the two lesser-described variants g.5884G > T and g.11895_11898del. The screened variants were not identified for any of the selected individuals. In order to identify variant segregation in the family, the parents were assessed for all the variants (Fig. ). Both parents demonstrated normal-range levels of bilirubin from multiple measurements.
The intronic variants not mentioned in the ClinVar database were annotated according to ACMG standards and guidelines []. Both variants are classified as likely pathogenic (for variant g.5884G > T – fulfilled criteria PM2, PM3, PP3, PP4, PP5; for variant g.11895_11898del – fulfilled criteria PM2, PM3, PP3, PP4; Table ).
|
pmc-6169071-1
|
A 46-year-old Moroccan man with a history of cocaine and alcohol abuse, former smoker of 10 packs/year, detained in a penitentiary for 3 months, presented to an emergency department because of the finding by penitentiary doctors of severe anemia: hemoglobin (Hb) 43 g/L. He did not report previous concomitant comorbidities and he did not take any medication prior to hospital admission. It was difficult to collect a detailed family history because of a language barrier; he worked as a street vendor. He complained of progressive fatigue, arthromyalgia, upper finger paresthesia, mild abdominal pain, left ear tinnitus, and recurring headache for the previous 2 months. He denied fever, bleeding, and changes in bowel habits. At admission, severe normocytic anemia with Hb of 36 g/L, mean corpuscular volume (MCV) 87 fl, hematocrit (htc) 10.8%, and random distribution of red cell width (RDW) of 27% was confirmed, with neutropenia (0.59 × 109/L) and normal platelet count (15 × 109/L). On presentation he was oriented, afebrile (axillary temperature of 36 °C), and hemodynamically stable with blood pressure of 110/70 mmHg and a heart rate of 80 per minute. A physical examination showed pale skin, slight epigastralgia, and left tympanic membrane perforation; no lymphadenopathy, purpura, or hepatosplenomegaly were detected. A neurological examination was normal without any motor, sensory, or cranial nerves dysfunction except for slight upper finger paresthesia. Initial laboratory investigations revealed renal impairment with creatinine up to 176.8 μmol/L and azotemia 24.9 mmol/L, marked anisopoikilocytosis and multiple schistocytes (10%) on peripheral smear, lactate dehydrogenase (LDH) increase (19.7 μkat/L), haptoglobin less than 1 mg/L, and normal bilirubinemia (17.1 μmol/L). Coagulation studies were normal except for slight elevation of D-dimer (2.63 nmol/L); markers of inflammation were negative; liver function was normal with aspartate aminotransferase (AST) 30 U/L and alanine aminotransferase (ALT) 18 U/L. A direct Coombs test was negative, reticulocytes count was consistent with inappropriate bone marrow response (reticulocytes production index 0.061), and ferritin was within normal range.
Hydration with normal saline and blood transfusions with packed red blood cells were started. We observed an initial improvement of renal function but anemia did not improve enough despite transfusions with five bags of packed red blood cells, and it was associated to hemolysis (schistocytes, LDH further increase and haptoglobin consumed). In parallel we observed a progressive rapid decrease of platelet count down to 46 × 109/L and severe neutropenia was persistent without peripheral blasts. A diagnosis of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome was considered, but the rapid improvement of renal function with only hydration and the pancytopenia suggested a bone marrow deficiency associated to a hemolytic component of unclear origin. We decided to keep on with blood transfusion support and to strictly monitor our patient until the results of further investigations; meanwhile, we started intramuscular vitamin B12 1000 mcg daily because of extremely low, barely detectable, plasma levels (< 36.9 pmol/L). Serology for cytomegalovirus, Epstein–Barr virus, parvovirus B19, and Toxoplasma gondii were negative (past infection) as were serology for human immunodeficiency virus and hepatitis virus. Bone marrow aspirate revealed normal cellularity with different cell types at various stages of maturation and without dysplastic alterations. An abdomen ultrasound showed normal kidneys and very slight splenomegaly (bipolar diameter 12.2 cm) without hepatomegaly and lymphadenopathy. See Additional file : Figure S1 for the timeline of the diagnostic and therapeutic flow of the present case report.
A week after beginning the vitamin B12 supplement we observed a dramatic hematological improvement with simultaneous decrease of hemolysis indexes; marked anisopoikilocytosis with teardrop cells (5%) persisted on peripheral smear without schistocytes (Fig. ). Anti-parietal cell antibodies were negative; upper endoscopy showed moderate gastric corpus atrophy, without presence of Helicobacter pylori. Recovery was complicated by pneumonia and urinary tract infections which were treated with intravenously administered amoxicillin/clavulanic acid.
We discharged our asymptomatic patient 2 weeks after admission with normal renal function, moderate normocytic anemia (85 g/L), and normal platelet and neutrophil count (484 × 109/L and 2.79 × 109/L, respectively).
At a follow-up of 6 months, he was alive but it was not possible to collect further medical information because we were informed that he was a fugitive.
|
pmc-6169104-1
|
We describe the case of a Caucasian 13-year-old girl who was first referred to our department with a diagnosis of retinitis pigmentosa.
She underwent a full clinical examination which included visual acuity, ophthalmoscopic evaluation, structural OCT, and photopic and scotopic ERG. The autofluorescence of both eyes showed a hyperautofluorescent halo around the fovea covering 360°. According to Gelman et al. [], the “hyperautofluorescent ring border” corresponds to impending photoreceptor loss.
Her best corrected visual acuity (BCVA) was 75 letters Early Treatment Diabetic Retinopathy Study (ETDRS) in her right eye (RE) and 60 letters in her left eye (LE). The visual acuity impairment was consistent with the abnormalities detected using OCT which revealed a deformation of the retinal profile and the presence of cystic spaces in both eyes (Fig. ). This report was referred to as “schisis at an early stage.”
ERG, performed according to International Society for Clinical Electrophysiology of Vision (ISCEV) standard, showed non-recordable rod response, and reduced maximal and cone responses with a decreased b-wave to a-wave ratio. Responses to 30 Hz flicker were reduced and delayed. Specialized ERG recordings of responses mediated prevalently by short-wavelength-sensitive cones (S-cones) and ML-wavelength sensitive cones (ML-cones) were obtained from both eyes. S-cone-mediated ERGs were recorded in response to a blue (420 nm) stimulus of 30 degrees flickered at 4 Hz and presented on a steady yellow background. ML-cone-mediated ERGs were obtained in response to a red (580 nm) stimulus of 30 degrees flickering at 4 Hz and presented on a steady blue (420 nm) background. Blue and red stimuli were photopically matched. In normal individuals the ML cone ERG is three times larger in amplitude (a-b wave peak) and 10 ms shorter (b-wave peak) compared to S-cone ERG. In this particular patient, S-cone ERG was of larger amplitude (1.2 times) compared to ML cone. The peak times of both responses were comparable. These findings indicated the enhanced S-cone sensitivity for this patient (Fig. ).
Highly suspected on the basis of clinical and electrophysiological findings the diagnosis of ESCS was made.
Our patient was evaluated every 6 months for a year. BCVA was measured every time: 6 months after the first examination, it was found to be 65 letters ETDRS in her RE and 75 letters in her LE, while, a year later, it was 82 letters in her RE and 75 letters in her LE.
Two years after the first examination, BCVA decreased to 65 letters in her RE and 40 letters in her LE, consistent with OCT findings. Structural OCT was performed using the DRI OCT Triton™ swept source OCT device (Topcon Medical Tokyo, Japan) and revealed an increased retinal thickness and a markedly altered retinal structure due to macular schisis. These features were detected in both eyes.
Macular schisis, as seen on OCT B scans (Fig. ’) (Fig. ’), affected both the outer and the inner nuclear layers: hyporeflective cystic-like spaces were seen in both these layers, even though they were much larger in the outer one. The outer plexiform layer appeared to be interrupted in the center of the macular region as holes in the inner and outer nuclear layers joined together. The vertical septa were observable mainly in the RE probably due to a greater disruption of retinal tissue in the LE. Hyporeflective holes separated by hyper-reflective partitions could also be visualized in the macular region on “en face” OCT images (Fig. ’) (Fig. , a’-a″) (Fig. , a’) performed at the level of the inner and outer nuclear layers. These spaces had different shapes: they were round-shaped in the inner nuclear layers, oval in the outer one.
At the same time an OCT-A was performed using the same device and it enabled the visualization of the vascular involvement. Superficial and deep vascular network can be separately evaluated by OCT-A. OCT-A scans of the superficial plexus showed the insignificant involvement of circulation of this layer (Fig. , c-c′) in both eyes, while the deep plexus revealed a marked remodeling of this vascular layer. In fact, there was an absence of capillary details secondary to the sliding of vessels at the margin of the schisis (Fig. , d-d’). The choriocapillaris vascular network did not seem to be affected by any abnormalities.
Changes in retinal structure were monitored by performing swept source OCT 1 month and 3 months after the first evaluation: 1 month later, a decrease in retinal thickness and in size of the hyporeflective cavities were noticed on macular OCT B scans carried out on her RE (Fig. , b); these features were consistent with the aspect observed on “en face” scans (Fig. , b’-b″) which showed a reduction in number and size of the cystic-like spaces. Three months after the first examination, a mild deterioration was detected (Fig. , c) (Fig. , c′-c″). A general improvement in retinal structure was congruent with visual acuity increase from 65 letters ETDRS to 80 letters; by contrast, no difference was noticed on horizontal scans (Fig. , a’-b′-c′) and on “en face” OCT images (Fig. , a’-b’-c’) performed on her LE, while visual acuity improved from 40 letters ETDRS to only 50 letters.
Genetic analysis showed two variants in NR2E3 (NM_014249.3): a missense variation c.1118T>C, which leads to substitution of leucine with proline in amino acid position 373, and c.349+5G>C, which involves a gene sequence near a splicing site.
|
pmc-6169107-1
|
This 39-year-old right-handed female suffered from epileptic seizures that began to develop in early 2014. The epileptic seizures were initiated by dyscognitive seizures and secondarily evolved into tonic-clonic seizures. The patient was started on anticonvulsive medication in August 2014 (Lamotrigin and Levetiracetam). Under this regimen, which was adapted regularly, the frequency of seizures stabilized at about one seizure every six months.
Some weeks prior to the first epileptic seizure, the patient had complained of novel subacute bifrontal headaches, which persisted intermittently over the course of the following months and were not directly correlated with the occurrence of epileptic seizures. Additionally, she reported a subjectively progressive impairment of her short-term memory. Her husband reported a temporary change in his wife’s personality during May 2015, which was accompanied by promiscuous behavior in social media and had not recurred since then.
Gastrointestinal symptoms (diarrhea, weight loss) had occurred for the first time in 2003. The diagnosis of CD was confirmed histologically via ileocolonoscopy in 2006. Symptoms were treated with a combination of steroids and mesalazine (5-ASA) from 2006 to 2008 and with methotrexate (15 mg once per week) from 2008 to 2016. The patient reported lack of gastrointestinal symptoms since 2013. Apart from nicotine abuse (25 pack-years) she reported no further comorbidities and did not suffer from any allergies. There was no family history of headaches, epilepsy, cancer and no exposure to toxic chemicals at home or at her workplace. There were no signs for lymphomatoid granulomatosis (no pulmonary and cutaneous lesions) or leukocyte oxidase deficiency (no recurrent infections). The patient did not travel outside of Europe in her entire life.
|
pmc-6169215-1
|
A 13-year-old child with learning disability was referred for ophthalmic assessment as she complained of bilateral blurring of vision. At presentation, her visual acuity was 1/60 in the right eye and 6/18 in the left eye. Relative afferent pupillary defect (RAPD) was present in the right eye. Both eyes anterior segment examination findings were normal. Grade 1 vitritis was noted in the right eye. Funduscopic examination of the right eye revealed a pale optic disc and pigmented scar over the macula with salt and pepper appearance. Intense retinitis with focal areas of haemorrhage was present nasally (). There was mild pallor of the left optic disc with macula and peripheral granular retinitis ().
Systematically, she was diagnosed with combined T and B cell deficiencies by the immunologist at the age of 11. At that time, she presented with high fever, recurrent episodes of diarrhoea, oral thrush, and failure to thrive, with the weight of only 12kg. PIDD screening showed low T cell, very low B cell counts, and low immunoglobulin levels (). Her systemic therapy consisted of 3 weekly intravenous immunoglobulin, sulfamethoxazole, and trimethoprim prophylaxis as well as empirical therapy for fungal infection which include syrup fluconazole 6mg/kg/day and syrup nystatin 1ml QID. She was also treated for CMV colitis as HPE of the colon showed CMV inclusion bodies. She completed 6 weeks of intravenous ganciclovir 3 months prior to presentation of her ophthalmic symptoms.
We diagnosed the child of having bilateral eye CMV retinitis based on typical fundus features and history of treated CMV colitis. She was planned for right eye intravitreal ganciclovir injection in view of poor visual function with posterior pole involvement. However, she was deemed unfit to undergo general anaesthesia due to concomitant hospital acquired pneumonia. IV ganciclovir 75mg (6mg/kg) 12 hourly was started and good response was noted after 2 weeks of therapy (). The treatment was continued for 8 weeks until the retinitis lesions had healed with scarring (). However, the right visual acuity reduced to light perception and improved to 6/9 in the left eye.
|
pmc-6169218-1
|
An 84-year-old man was admitted to our hospital complaining of muscular weakness while rising up from a chair that led to a fall. He was admitted for further studying, but his head CT and MRI did not report any abnormal structural findings. He reported a significant 10% weight loss in the past 6 months associated with decreased appetite and diminished mobility associated with progressive muscular weakness and difficulty rising from chair, with preservation of activities as combing his hair, or lifting small objects.
The physical examination was remarkable for muscular weakness with 3/5 muscle strength in the lower extremities and 4/5 of the upper extremities, confined to the proximal muscles. Tendon reflexes were diminished and the tone examination revealed mild bilateral quadriceps hypotonia and atrophy. There were no other clinical findings on the physical examination.
The patient reported a medical history of stage G4 chronic kidney disease, erythroid and megakaryocyte-predominant myelodysplastic syndrome, and high blood pressure, receiving medication with azacytidine, diltiazem, and darbepoetin. The patient did not smoke, consume alcohol, or use illicit drugs, and his family history was negative for neuromuscular diseases.
During the present admission, laboratory investigation showed an elevated creatine kinase level of up to 78,924 U/L (more than 50 times the normal reference range) and an aldolase value of 181 U/L (more than 20 times the normal reference range). Elevated serum creatinine was found (4.4 mg/dl; steady-state level 3 mg/dl), with mild hypocalcemia (7.2 mg/dl) and mild hyponatremia (130 mg/dl) with normal albumin (4 mg/dl). Thyroid hormones were normal, and cardiac enzymes were also in normal range. Coprologic examination revealed positive testing for rotavirus. Antibody testing reported negative results for antinuclear antibodies (ANAs), anti-Jo1, anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR), anti-Mi-2, and also for antiganglioside antibodies.
The electromyography (EMG) of the upper and lower limbs showed myopathic changes in proximal muscle, with lower limb predominance, with short duration, low-amplitude polyphasic potential with no positive sharp waves, and spontaneous electrical activity.
An FDG-PET/CT scan was performed searching for neoplasia due to the patient's past history and revealed increased FDG uptake in bilateral quadriceps, without posterior compartment muscle uptake or increased metabolism in any other region of the body (). No other location of FDG uptake was found.
We performed an open biopsy of the left vastus lateralis muscle, which on light microscopy showed 50% myonecrosis, mild fiber atrophy, and lymphocytic infiltrate with CD8+ predominance and perivascular involvement. There were no immune deposits in the skin microscopic examination ().
The diagnosis of PM was made and then the patient started on intravenous hydration with medium saline solution/bicarbonate for rhabdomyolysis and prednisone 0.5 mg/kg/day and intravenous immunoglobulin with a total dose of 2 g/kg distributed in 5 days, along with calcium supplementation and azacytidine. For the myelodysplastic syndrome diagnosis, steroid-sparing drugs such as azathioprine or methotrexate were not considered. The renal function of the patient improved within 5 days, with partial recovery of lower limb strength, and the patient was discharged for external consultation follow-up. After 2 months from discharge, the patient had increased muscle strength and diminished CPK levels, with low-dose prednisone as maintenance therapy.
|
pmc-6169222-1
|
An asymptomatic 6-year-old boy was referred by a dermatologist because of lesions on the inner side of his lower lip that firstly appeared 4 years ago.
He was a skinny boy, light-coloured skin, blond, and green-eyed that was always eating small meals. He had no previous family history of PJS. On clinical examination, we found seventeen café au lait spots ranging from 0.3–3 cm on the anterior and posterior body surface and extremities ().
Blood tests showed mild anemia. Abdominal ultrasound and computed tomography showed a large polypoid gastric mass in the antrum and the beginning of the duodenum ().
A large, 8 × 5 cm in size, multilobed polypoid gastric mass situated in the antrum was found in gastroscopy. The mass was hemorrhagic, wide-based, and seemed to enter duodenum but moved back to the antrum with peristaltic movements. Two smaller polyps, 0.5 cm in size, were found at the 2nd part of the duodenum near the ampulla of Vater.
The operation was scheduled for polyp removal. Under general anesthesia, a hard epigastric mass was palpated. We made a midline supraumbilical incision. The hard mass could be palpated at the lower third of the stomach. Palpation also revealed one lesion at the second part of the duodenum and another in the jejunum. We did a gastrotomy on the anterior surface of the pyloric antrum. The polyp was wide-based (), occluding almost completely the pylorus and the duodenum only leaving a space for a hand's little finger to pass. We proceeded to a lower third gastrectomy involving the duodenal bulb, pylorus, and antrum and performed a Billroth I anastomosis. We also did a longitudinal incision of the jejunum 15 cm away from the ligament of Treitz and managed to remove one wide-based polyp, which is 1.5 cm in length. His postoperative course was uneventful.
STK11/LKB1 gene identification (a gene encoding a serine/threonine kinase that is responsible for the appearance of the syndrome) (1, 2) showed the splicing mutation: c290 + 1 G > A, in intron 1, which results in aberrant splicing. No family history was reported, so it is highly likely that this mutation was a de novo event. This could not be confirmed, as the patient's parents did not consent to genetic testing.
Postoperative results were excellent. We advised for clinical examination and ultrasound of testis yearly and capsule endoscopy, colonoscopy, and gastroscopy biannually.
At a second look gastroscopy, six months later, we managed to endoscopically remove two smaller polyps near the ampulla of Vater. Colonoscopy was clear. Histology verified the diagnosis of PJS. All 4 polyps showed findings suggestive of hamartomas ().
So far, one polyp was detected in the duodenum and another in the sigmoid colon. Polyps were removed by gastroscopy and colonoscopy, respectively. The duodenal polyp was found to be histologically a Peutz-Jeghers polyp, whereas sigmoid polyp was found to be of a hyperplastic type. The gastric polyp was also found to have abnormal (resembling neoplastic) growth in muscularis mucosae where smooth muscle fibers followed the growth of exophytic gastric pits. Biopsies of the esophagus were normal, and gastric biopsies revealed only signs of mild chronic gastritis.
|
pmc-6169228-1
|
The patient is a 30-year old now gravida 2 para 2, status post complete left nephrectomy in the setting of multidrug resistant Klebsiella urosepsis and left pyelonephritis during her immediate postpartum phase. The Anuak speaking woman immigrated from Kenya to the United States nine months prior to her second pregnancy and presented to care at 15 weeks' gestation. Her history included chronic hypertension without a previous history of UTI.
At her new obstetrical visit, a urinalysis demonstrated 4-10 white blood cells (WBC) per high power field and gram stain positivity for gram-negative bacilli and gram-positive bacilli. Urine culture yielded multidrug resistant Klebsiella pneumoniae, 10(4) to 10(5) colony forming units (cfu/mL). The organism was susceptible to quinolones, carbapenems, and piperacillin/tazobactam. An Infectious Disease consultation recommended a repeat clean catch culture with treatment using IV ertapenem if the culture showed the same organism. The repeat urine culture showed mixed flora without a specific organism identified. Because the patient remained asymptomatic, she did not have an additional gram stain or urine culture through the remainder of pregnancy.
At 37 weeks' gestation, the patient developed superimposed preeclampsia and underwent induction of labor with a normal spontaneous vaginal delivery without complications. On postpartum day (PPD) 0, she was afebrile but reported left sided abdominal and flank pain. A urine culture on PPD1 was positive for multidrug resistant Klebsiella/Raoultella species (sp) > 10(5) cfu/mL and sensitive to quinolones, gentamicin, and piperacillin/tazobactam. She began PO ciprofloxacin 500 mg twice daily with creatinine rising to 1.1. By PPD3, she continued to have abdominal and flank pain with creatinine rise to 1.5, and based on urine culture sensitivities, antibiotic was changed from ciprofloxacin to PO levofloxacin 500 mg daily. By PPD4 she developed new onset tachycardia, tachypnea, fever of 38.6 degrees celsius, and continued pain. As such, she was transferred to the intensive care unit with lactate 2.8 and WBC 18,000 and started on IV piperacillin/tazobactam 3.375 grams every 6 hours. A chest computerized tomography (CT) was negative for pulmonary embolism, showing a moderate left pleural effusion, and abdominal/pelvic CT compatible with pyelonephritis but no abscess. Blood cultures were positive for Klebsiella/Raoultella sp. On PPD5, when stable, she was transferred to the postpartum unit and switched to IV meropenem 500 mg every 6 hours due to persistent fevers. On PPD7, with ongoing fevers, tachycardia, and pain, a left kidney ultrasound confirmed a 5.1 cm left subcapsular abscess which was aspirated 30 cc of purulent discharge. An echocardiogram for endocarditis and HIV testing were negative. By PPD9, with continued fevers to 39.3 degrees celsius, abdominal CT imaging was concerning for left renal multifocal infection and parenchymal necrosis (). On PPD10, after Urology and Maternal Fetal Medicine consultations, the patient underwent an open left nephrectomy. Pathology confirmed severe diffuse pyelonephritis, multifocal abscesses, and diffuse parenchymal infarction ().
The patient recovered well thereafter with symptom resolution, creatinine 1.0, and was discharged on postoperative day 3 with a 14-day course of IV ertapenem 1 gram daily.
|
pmc-6169230-1
|
The 8-year-old boy first presented in January 2012, having been referred by the family dentist, to the Department of Orthodontics at the Medical Center of the University of Göttingen for a routine orthodontic control and evaluation of treatment need (). The clinical examination of the asymptomatic patient showed no extra- or intraoral pathological findings. The medical history of the boy included a mild pulmonary valve stenosis and a secundum atrial septal defect with a left-right shunt. He showed a good physical and cardiac fitness and a normal nutritional status. The family history was positive for maxillofacial anomalies: the boy's older sister had been previously diagnosed with a dysplastic fibroma, a rare benign fibrovascular defect in the mandible, and a resection of the affected area in the mandible had been performed. His father and paternal grandmother had a positive history of odontogenic cysts as well as basal cell carcinomas, although the family history of OKCs was negative. The radiological examination showed three suspicious hypomineralisations visible as radiolucencies in the panoramic radiograph associated with the retained teeth 13 and 23 and the ectopic tooth 27 (). The young patient was referred to the Department of Maxillofacial Surgery for a surgical examination of the radiologic anomalies.
The operation was performed under general anesthesia. The suspected pathological area around teeth 23 and 13 showed no visible intraoperative pathological signs. A bone and soft tissue biopsy for histological examination was taken. In the area of tooth 27, a well-marked membrane was revealed, filled with a viscid fluid and fully enclosing the dental crown. The clinical aspect was consistent with a follicular cyst. During the radical cystectomy, tooth 27 was removed due to massive attachment loss. The histopathological biopsy showed a fibroosseous lesion in the area of teeth 13 and 23. The biopsy from region 27 showed an odontogenic connective tissue cyst wall with intramural odontogenic cell islands. On request of the surgeon, samples were sent for further diagnosis to the Bone Tumor Reference Center of the Swiss Society of Pathology at the University Hospital in Basel, Switzerland. The initial histological diagnosis was corrected to an OKC of the parakeratin variant. Microscopically, the cyst shows a squamous epithelium. The basal cells are palisading, with hyperchromatic nuclei (HE staining, 5x magnification) (). Due to the high recurrence of OKCs, a radiological control interval of 6 months was indicated (). Furthermore, orthodontic treatment was initiated.
In August 2014, during a regular radiological control, a new radiolucency was detected, associated with the retained and displaced teeth 47 and 48 (). The surgical removal of the cystic lesion and tooth 47 was performed under general anesthesia. The pathological finding was consistent with an OKC.
The regular control examinations were interrupted by missed appointments, so the next evaluation took place one year later, in October 2015 (). New radiolucencies were detected in the panoramic radiograph associated with the retained teeth 18, 17, 37, 38, and 48 as well as an evident enlargement of the radiolucency around the crown of tooth 13. A cone beam computer tomography scan was performed and showed well-defined radiolucent areas, associated with the retained teeth. Details of the surgical enucleation of the cysts with the extraction of teeth 18, 17, 13, 37, 38, and 48 are shown below. The postoperative radiological examination is depicted in . Clinical and radiological examinations were then performed every 6 months.
We describe the surgical enucleation of the cystic lesions using the example of the third operation (2015). This was performed under general anesthesia; the affected regions were exposed after lifting a mucoperiosteal flap. After a careful removal of a thin bone cortex, the cystic capsule was found () and separated from the bone with an obtuse instrument. The aim was to leave no epithelial remnants on the trabecular bone. All four lesions were associated with a retained tooth, which was only loosely anchored in the alveolar bone. Due to the high recurrence rate of the cystic lesions in this particular case, all affected teeth were extracted. In the area of the mandible, the use of Carnoy's solution was not indicated because of the exposure of the lower alveolar nerve (). Due to their large size, the cystic cavities were filled with a collagen graft, which stabilized the formation of a coagulum. No reconstruction with iliac crest bone or allogenic bone grafts was attempted. Subsequently, the mucoperiosteal flap was reverted back to its original position and fixed by sutures.
By October 2016 and August 2017, bone remodeling of the affected area had been detected and no new lesions were observed (Figures and ).
Due to the recurrence and the appearance of new lesions, Gorlin-Goltz syndrome was suspected in the patient. After genetic counselling at the Institute of Human Genetics of the University Medical Center of Göttingen, molecular genetic analysis of the genes PTCH1 and PTCH2 was performed in 2015. Sanger sequencing revealed the heterozygous germline variant c.2779_2793del (p.Ser927_Val931del) in the PTCH1 gene. This variant leads to an “in-frame” deletion of 5 amino acids between amino acid positions 927 and 931 of the protein. This variant is listed neither in the Human Gene Mutation Database (HGMD) nor in the Leiden Open Variation Database (LOVD). However, a pathogenic effect of the variant seemed likely as many pathogenic variants have already been described in this region of the PTCH1 gene, even several in-frame deletions [, ]. Since the boy's father had shown similar symptoms (odontogenic cysts, basal cell carcinomas) that could be in line with a Gorlin-Goltz syndrome, he, too, was tested for the PTCH1 variant and resulted to be carrier of the variant.
In summary, clinical and molecular data together with the positive segregation analysis led to the classification of the variant as “probably pathogenic” and being responsible for Gorlin-Goltz syndrome in the patient and his father. The importance of talking precautions (e.g., sun protection due to the high risk of basal cell carcinomas) and regular medical surveillance (e.g., regular orthodontic care and annual dermatologic examinations) was emphasized.
|
pmc-6169237-1
|
An 85-year-old black woman was admitted to the hospital with complaints of crampy abdominal pain, vomiting, and several episodes of watery diarrhea for two days. Her past medical history included end-stage renal disease (on continuous cycling peritoneal dialysis), congestive heart failure, atrial fibrillation, chronic obstructive pulmonary disease, and cervical cancer (status after total abdominal hysterectomy). She was started on peritoneal dialysis three months ago for her end-stage renal disease during a hospital admission for congestive heart failure. She was discharged to rehab, where she stayed for a month. Her last home dialysis session was a day prior to the onset of symptoms. She denied any fever, bloody stool, outside food ingestion, any sick contact, and recent travel history. On physical examination, her vital signs revealed a pulse rate of 76 beats per minute, blood pressure of 121/70 mmHg, respiratory rate of 16 breaths per minute, and a temperature of 99°F. Abdominal examination revealed nondistended abdomen with diffuse tenderness on palpation and hyperactive bowel sounds with voluntary guarding; however, dialysis catheter was intact with no surrounding erythema or purulent discharge. Initial laboratory analysis showed WBC of 13,400/μL, neutrophils of 88.5%, hemoglobin of 11 g/dl, and serum albumin of 2.9 mg/dl. Peritoneal fluid analysis revealed white blood cell count in peritoneal effluent of 8359/μL, with 93% neutrophil predominance. Gram strain did not show any microorganisms. Given the overall clinical status, peritonitis was suspected, and she received empiric vancomycin and ceftazidime. However, stool C. difficile toxin B assay returned positive. She was concurrently started on oral metronidazole. Within 24 hours, there was improvement in the clinical symptoms, and her broad-spectrum antibiotics were discontinued after a couple of days. The patient was diagnosed with PD-related peritonitis accompanying C. difficile-associated diarrhea, with continuation of oral metronidazole. Repeated effluent white blood cell count the day after starting metronidazole decreased to 133/μL, with complete resolution of peripheral leukocytosis. Surgical consultation done on admission concluded that the peritoneal catheter was not the source of infection and, therefore, the catheter was not removed. The patient's symptoms resolved, and she was then discharged on a total of three weeks of metronidazole with continuation of peritoneal dialysis.
|
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.