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To report a case of partial limbal stem cell deficiency and characteristicmucocutaneous triad in an 11-year-old boy.

Systemic features along with ocular features are described in this case report.

Ophthalmic evaluation showed bilateral partial limbal stem cell deficiency (LSCD) and systemic examination revealed characteristicmucocutaneous triad of oral leukoplakia, skin hypopigmentation, and naildystrophy suggestive of the Zinsser-Engman-Cole syndrome or dyskeratosis congenita.

Thorough ocular and systemic evaluation can help in clinching the diagnosis of this rare condition. Timely referral to a dermatologist and hemato-oncologist would help in the management of life-threatening complications likemalignancy or progressive bone marrow failure.
Thorough ocular and systemic evaluation can help in clinching the diagnosis of this rare condition. Timely referral to a dermatologist and hemato-oncologist would help in the management of life-threatening complications like malignancy or progressive bone marrow failure.Statins are the primary class of medication used to lower serum cholesterol concentration for both primary and secondary prevention of cardiovascular disease. Muscle pain is a frequent adverse effect of statins. Severe myonecrosis leading to clinical rhabdomyolysis is rare. We encountered a 63-year-old male with a medical history of hypertension, type 2 diabetes mellitus, and coronary artery disease with angioplasty in 2008 and 2020. He was started on rosuvastatin 40 mg (0-0-1) along with dual anti-platelets post angioplasty and was discharged home. He traveled back to his hometown and noticed progressive symmetric muscle weakness with decreased urine output. After visiting another hospital he presented to us with severe proximal muscle weakness and acute renal failure. Laboratory investigations were initiated which demonstrated clinically significant derangement in serum creatinine phosphokinase, serum creatinine, urine myoglobin along with deranged liver enzymes. He was subjected to nerve conduction studies for his muscle weakness which was normal and electromyography showed abnormal spontaneous muscle activity in all examined muscles (fibrillations, positive sharp waves, and pseudomyotonic discharges) suggestive of an irritable myopathy. The medication was stopped and he was treated with eight cycles of hemodialysis until his muscle weakness and laboratory parameters improved. He was then discharged with some improvement in muscle weakness. On two week follow-up, the patient showed partial improvement after discontinuation of all lipid-lowering medication.Objective Assess variability of surgical technique for minimally invasive sacral colpopexy (MISC) among Female Pelvic Medicine and Reconstructive Surgery (FPMRS). Methods A voluntary anonymous questionnaire was given to the 2018 American Urogynecologic Society (AUGS) annual meeting attendees. Comparisons were made by age, gender, experience (years), practice setting, and U.S. region. Results There were 59 responses from 671 physician conference attendees. Most were male (64.4%), U.S. physicians (94.6%), completed Obstetrics and Gynecology residencies (91.5%), practicing in University settings (66.1%). The mean age was 47.4±8.6 years, practicing>15 years (47.5%). Predominant routes were 53.8% robotic, 42.2% laparoscopic, and 4.0% open. Surgeons used 3-4 ports (both 50.0%), with 0-degree (46.0%) or 0 and 30 degree laparoscopes (36%). For sacral mesh attachment, 83.1% used suture as opposed to tacking devices, most often Gortex (56.3%). Anterior (48.1%) and posterior (50.0%) vaginal attachment used 5-6 sutures. Concomitant procedures included anterior repair (83.4% "not usually"/"not at all"), posterior repair/perineorrhaphy (77.8% "yes, often"/"yes, sometimes"), midurethral sling (42.6% "yes, often"/51.9% "yes, sometimes"), and hysteropexy (86.5% "not usually"/"not at all"). Post void residual (PVR) was assessed after surgery by 89.8%, 75.5% via retrograde fill voiding trial. Most patients were discharged post-operative day 1 (POD1) (47.6% AM, 29.1% PM) or day of surgery (15.2%). Females more commonly performed hysteropexy (p=0.028) with no other significant differences by age, gender, experience, practice setting or region. Conclusion Most FPMRS surgeons perform MISC, equally robotic and laparoscopic. Concomitant posterior wall procedures and midurethral slings are common. Other than more hysteropexies performed by females, no other variables predicted technique variations, suggesting technique homogeneity.Gossypiboma is a term used to describe a pseudotumor caused by accidental retention of surgical swab or sponge in the body after surgery. The abdominal cavity is the most common site of retained surgical sponge. c-RET inhibitor It is quite an infrequent surgical complication which is usually rarely reported because of the fear of medico-legal consequences. Here, we are reporting a case of a 26-years-old woman referred to our outpatient surgery clinic (OPD) from another hospital with complaint of intermittent abdominal pain, fever, and abdominal lump for 4 months following removal of IUD, which was attempted laparoscopically, and later converted to open laparotomy. She also had a history of cesarean section done one and half year ago in the same hospital. Clinical examination revealed a palpable abdominal mass in the para-umblical region. However, a computed tomography (CT) revealed a huge intra-abdominal mass. A diagnosis of intra-abdominal gossypiboma was suggested and the patient underwent exploratory laparotomy where the diagnosis was confirmed and the mass was excised.Isotretinoin or 13-cis-retinoic acid, a derivative of vitamin A, is a highly effective therapy for severe and resistant acne. Its usage is restricted worldwide because of its teratogenic potential. The most common side effects are dermatologic, cheilitis, increased skin fragility, and susceptibility to sunburns. Rare side effects include myalgias and arthralgias. It also causes transient laboratory abnormalities such as elevated creatine kinase (CK). Very few cases of isotretinoin-induced severe muscle injury leading to rhabdomyolysis have been reported in the literature. We report a 15-year-old adolescent who developed rhabdomyolysis after a bout of vigorous exercising while on long-term isotretinoin therapy.Kawasaki disease (KD) is an autoimmune disease that generally affects children under the age of five years. It has a variety of clinical manifestations which may be either specific or nonspecific. Intravenous immunoglobulin and aspirin are the mainstays of treatment. There are unusual circumstances where patients are resistant to conventional treatment. We report a one-year-old girl who presented with a 12-day history of fever in association with erythema at the site of Bacille Calmette-Guérin (BCG) scar. She did not respond successfully to conventional treatment although she was diagnosed to have Kawasaki disease. Eventually, she responded to intravenous methylprednisolone and was diagnosed as having refractory Kawasaki disease.Objectives The main aim is to find out the clinical feature and outcome of status epilepticus (SE) in children managed in a teaching hospital. The secondary aim is to identify the risk factors influencing the adverse outcomes. Methods In this prospective cohort, children aged 1 month to 14 years with SE as per the International League Against Epilepsy's new guideline (2016) who presented to the emergency department during the period of November 2017 to October 2019 were enrolled. Clinical profile, treatment, and outcome of cases (n = 94) were noted. Results The majority of children, 60 (63.82%), were less than five years of age. Prior history of seizures was present in 33 (35.1%) cases, whereas 61 (64.9%) cases presented with SE as the first episode of seizure. In 14 (42.4%) previous seizure cases, SE was due to drug default. No response to first-line antiepileptic drug (AED) was seen in 84 (89.37%) cases. Acute symptomatic etiology was the commonest etiology of SE in 64 (68%) cases, of which neuro-infections accounted for 44 (46.80%) cases. Longer duration (>60 minutes) of status (p less then 0.01), ventilator support (p less then 0.0001), and circulatory impairment (p less then 0.0001) were attributable risk factors for mortality. A total of 28 children died (mortality rate, 29.8%), and 11 showed the persistence of their neuro-deficit. Conclusions Neuro-infection is the most common etiology of SE in children. Longer duration of SE, more lag time for receiving the first AED, respiratory failure, and presence of shock are independent predictors for poor outcome. Hence, cessation of convulsion at the earliest leads to improved outcomes.Solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) have been combined into a single designation in the most recent World Health Organization (WHO) guidelines as solitary fibrous tumor/hemangiopericytoma (SFT/HPC). These rare intracranial tumors can present as WHO grade I, II, or III tumors, with the risk of recurrence, metastasis, and mortality worsening with higher-grade tumors. We present a case of a patient with a WHO grade III SFT/HPC with an emphasis on the imaging features that help differentiate this type of tumor from meningiomas, which are much more common and can appear similar. Being able to help differentiate these tumors by their imaging appearance is important to help triage and risk-stratify patient management decisions.We report the case of a 31-year-old male patient with underlying psychiatric illness and substance abuse who presented to the emergency department with a chief complaint of abdominal pain. CT scan of the abdomen revealed a large retroperitoneal mass wrapping around the aorta and obstructing the left ureter causing hydronephrosis. Physical examination found a painless left testicular mass. The ultrasound revealed a left scrotal mass measuring 32 x 24 x 16 mm with evidence of increased vascularity and calcifications. The patient underwent ureteral stent placement and left testicular orchiectomy with the pathology evaluation revealing seminoma. Although follow-up care plans were made, the patient relapsed IV heroin abuse causing failure to attend oncological treatment appointments. Two months later upon readmission, a repeat of the CT scan of the abdomen and pelvis showed a persistent large left retroperitoneal mass with an increase in size and significant mass effect. The patient would be ultimately considered for hospice if lack of compliance were to continue for his chemotherapy, along with his comorbid underlying substance abuse and psychiatric illness. This case highlights the effect of mental illness on medical care and treatment, demonstrating how a treatable malignancy may result in greater morbidity and mortality in psychiatric populations.Background Neonatal sepsis is one of the most common causes of neonatal mortality and morbidity, particularly in developing countries. Its causative bacteria and their respective sensitivity patterns are different in each hospital and region. The objective of this study was to determine the causative bacteria and their antibiotics sensitivity patterns at the neonatal unit. Methods This prospective study was carried out at the Neonatology Unit of Kharadar General Hospital (KGH) from January 2017 to Jun 2019. A total of 162 neonates with suspected sepsis and positive blood cultures were included in the study. Blood culture was done by standard microbiological techniques (BACTEC Method). Continuous data were presented as mean and standard deviation, while categorical data were presented in frequency and percentages. Result Out of a total of 162 neonates with blood culture positive neonatal sepsis, males were 106 (65.4%). Gram-positive and Gram-negative bacteria were found with a frequency of 83 (51.5%) and 79 (48.
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