Chediak-higashi syndrome

Chediak Higashi Syndrome is a rare inherited autosomal recessive disorder of immune system. Susceptibility to infection due to phagocyte dysfunction ranges from recurrent skin infection to over whelming fatal systemic infection. A five years old male child was admitted on 31st March 2006 with the complaints of pallor, fever & ear discharge for 6 month. He was treated by several medications but had temporary relief. The ear discharge was bilateral, foul smelling and of yellowish color. Past history was significant with episodes of abscess involving the different parts of body. Myeloid precursors shows giant, purple stained granules mainly in metamyelocytes, band cells and mature neutrophils

Bedside index (BISAP) v/s ranson scores in predicting mortality and severity in patients with acute pancreatitis

Objective: To determine the diagnostic accuracy of the bedside index for severity in acute pancreatitis in comparison with Ranson scores in predicting mortalities and severities in patients with acute pancreatitis.Methods: The cross-sectional study was conducted at the Department of Emergency Medicine, Aga Khan University Hospital, Karachi, from July 1, 2017, to January 1, 2018, and comprised patients who presented with acute pancreatitis. The bedside index for severity in acute pancreatitis score was applied in the emergency department and the patients were followed up in ward/intensive care unit where Ranson scores were calculated within the following 48 hours. Both the scores were calculated and compared for the prediction of severity and mortality for each patient. Data was analysed using SPSS 20.Results: Of the 136 patients, 88(64.7%) were males and 48(35.3%) were females. The overall mean age was 42.04±16.42 years (16-75 years), On the basis of two scores, mild and moderate acute pancreatitis was diagnosed in 123(90.4%) and 119(87.5%) patients respectively, while severe condition was diagnosed in 13(9.6%) and 17(12.5%) patients respectively. The bedside index had specificity 94.62% compared to 91.54% for Ranson score; sensitivity 100% vs 100%; negative predictive value 100% vs 100%; positive predictive value 46.15% vs 35.29%; and diagnostic accuracy 94.85% vs 91.91%.Conclusions: The bedside index for severity in acute pancreatitis and Ranson score were both found to be reliable tools in predicting mortalities and severities in patients with acute pancreatitis

Unintentional, paediatric domestic injury in a semi rural area of Karachi.

OBJECTIVE: To identify the characteristics of unintentional domestic injury in children living in a semi-rural area of Karachi. METHODS: This retrospective descriptive study was carried out at a private hospital located at the northern outskirts of Karachi, Pakistan, from January 2005 to January 2007. We enrolled 271 out-patient children of either gender with a positive injury history. A questionnaire inquiring socio-demographic characteristics, timing of injury, monthly variation, injury pattern and factors was filled out. Descriptive analysis and Chi-square test were applied to find out the statistically significant differences. RESULTS: Among the 271 patients, the male-to-female ratio was 1.2:1. Fall from height 145 (54%) or at the same level 52 (19%) was the commonest form of domestic unintentional injury across all ages. Children younger than 2 years of age, 140 (52%), were more prone to injuries, with 49 (51%) having fallen from the lap. Cuts with sharp domestic utensils were noted in 19 (6%), while 15 (6%) had burns. Submersion in underwater tank was noted in 20 (7%) cases and poisoning in 7 (3%). Majority of the injured children were from the low socio-economic stratum and more injuries, 194 (72%), were reported during the summer months. CONCLUSION: Male children are more prone to suffer unintentional domestic injuries especially during summer vacations. Fall was the commonest pattern noted

Use of steroids in COVID-19 patients: What is known?

COVID-19 pandemic has exaggerated the role of steroids in the standard of care despite minimum direct evidence of their efficacy in COVID-19 patients and their well-known adverse effects. The literature abounds on the side effects of steroids affecting different organ systems of the body. COVID-19 patients, who are on long-term steroids, are more susceptible to their adverse effects. We, herein, briefly review the potential uses and the adverse effects of steroids on different organ systems of the body. Key Words: Steroids, COVID-19, Adverse effects