PURPOSE: To evaluate clinical and laboratory profile of Wilson's disease (WD) in children. METHODS: This cross sectional study was conducted at Bangabandhu Sheikh Mujib Medical University Hospital. Bangladesh, over a period of 3 years. One hundred consecutive children of WD between 3 to 18 years of age were evaluated. RESULTS: Mean age was 8.5+/-1.5 years. Male female ratio was 2:1. Ninety-one percent of patients were Muslim and 9% Hindu. A total of 53% cases of hepatic WD presented between 5 to 10 years of age and most of the neurologic WD manifested in 10-15 years age group. Sixty-nine children presented only with hepatic manifestations, 6 only with neurological manifestations, 14 with both hepatic and neurological manifestation, 10 children was asymptomatic and 1 patient presented with psychiatric features. WD presented as chronic liver disease (CLD) in 42%, CLD with portal hypertension in 34%, acute hepatitis in 20% and fulminant hepatic failure in 4% cases. Stigmata of CLD were found in 18% patients. Keiser-Fleischser ring was found in 76% total patients. Elevated serum transaminase was found in 85% cases, prolonged prothrombin time in 59% cases and hypoalbuminaemia in 53% cases. A total of 73% patients had low serum ceruloplasmin, basal urinary copper of >100 microg/day was found in 81% cases and urinary copper following penicillamine challenge of >1,200 microg/day was found in 92% cases. CONCLUSION: Majority of studied WD children presented with hepatic manifestation of which 76% presented with CLD. Any child presented with jaundice after the age of 3 years should be investigated for WD.
Bangladesh ; Ceruloplasmin ; Child* ; Christianity ; Copper ; Female ; Hepatitis ; Hepatolenticular Degeneration* ; Humans ; Hypertension, Portal ; Islam ; Jaundice ; Liver Diseases ; Liver Failure, Acute ; Male ; Neurologic Manifestations ; Penicillamine ; Prothrombin Time

Purpose: Although the 24-hours urinary copper excretion is useful for the diagnosis of Wilson disease (WD), there are practical difficulties in the accurate and timed collection of urine samples. The purpose of this study was to verify if the spot morning urinary Copper/ Zinc (Cu/Zn) ratio could be used as a replacement parameter of 24-hours urinary copper excretion in the diagnosis of WD. Methods: A cross-sectional study was conducted at the Department of Pediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from June 2019 to May 2021 on 67 children over three years of age who presented with liver disease. Twenty-seven children who fulfilled the inclusion criteria for WD were categorized into the test group, and the remaining forty children were considered to have non-Wilsonian liver disease and were categorized into the control group. Along with other laboratory investigations, spot morning urinary samples were estimated for the urinary Cu/Zn ratio in all patients and were compared to the 24-hour urinary copper excretion. The diagnostic value of the Cu/Zn ratio was then analyzed. Results: Correlation of spot morning urinary Cu/Zn ratio with 24-hours urinary copper excretion was found to be significant (r=0.60). The area under ROC curve with 95% confidence interval of morning urinary Cu/Zn ratio measured using 24-hours urine sample was 0.84 (standard error, 0.05; p<0.001). Conclusion Spot morning urinary Cu/Zn ratio seems to be a promising parameter for the replacement of 24-hours urinary copper excretion in the diagnosis of WD.

Purpose: Acute pancreatitis (AP) is common among children in Bangladesh. Its management depends mainly on risk stratification. This study aimed to assess the severity of pediatric AP using computed tomography (CT). Methods: This cross-sectional, descriptive study was conducted in pediatric patients with AP at the Department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka, Bangladesh. Results: Altogether, 25 patients with AP were included, of whom 18 (mean age, 10.27±4.0 years) were diagnosed with mild AP, and 7 (mean age, 10.54±4.0 years) with severe AP.Abdominal pain was present in all the patients, and vomiting was present in 88% of the patients. Etiology was not determined. No significant differences in serum lipase, serum amylase, BUN, and CRP levels were observed between the mild and severe AP groups. Total and platelet counts as well as hemoglobin, hematocrit, serum creatinine, random blood sugar, and serum alanine aminotransferase levels (p>0.05) were significantly higher in the mild AP group than in the severe AP group (p=0.001). The sensitivity, specificity, positive predictive value, and negative predictive value of CT severity index (CTSI) were 71.4%, 72.2%, 50%, and 86.7%, respectively. In addition, significant differences in pancreatic appearance and necrosis were observed between the two groups on CT. Conclusion CT can be used to assess the severity of AP. In the present study, the CTSI effectively assessed the severity of AP in pediatric patients.

Purpose: Perinatal cytomegalovirus (CMV) infection can lead to biliary atresia (BA) in different entities. This study aimed to compare the clinical, hematological, biochemical, and histological features of infants with BA based on their CMV immunoglobulin M (IgM) status at presentation. Methods: This cross-sectional descriptive study was carried out between January 2019 and June 2020 at the Department of Pediatric Gastroenterology and Nutrition at the Bangabandhu Sheikh Mujib Medical University (BSMMU) in Dhaka. Forty-three patients with BA were selected purposively and categorized into either the CMV IgM-positive or CMV IgM-negative BA group. Categorical variables were compared using Fisher’s exact test and chi-square tests, while the Student’s t-test and Mann–Whitney U-test were used to compare continuous variables. For all statistical tests, a p-value <0.05 was considered statistically significant. Results: Thirty-three (76.7%) of the cases were between 2 and 3 months of age on admission.The clinical, hematological, and biochemical parameters did not differ significantly between the CMV IgM-positive and CMV IgM-negative BA groups. Most (50.0%) of the CMV IgMpositive cases had fibrosis stage F2, while 43.5% of the CMV IgM-negative cases had fibrosis stage F3, with no significant difference between the groups (p=0.391). Conclusion Our data shows no significant distinction between CMV IgM-positive and CMV IgM-negative BA, suggesting that CMV does not contribute to BA pathogenesis.