A Study on Frequency of Renal Impairments in Wilson’s disease among Children Attending at a Tertiary Care Hospital

Background: Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism that leads to hepatic, neurological, psychiatric, and renal complications. Early diagnosis is crucial, as timely treatment can prevent severe organ damage. Although renal involvement in WD is less common, its increasing prevalence necessitates further investigation. The prevalence and spectrum of renal manifestations in Bangladeshi children with WD remain unknown. This study aims to evaluate the pretreatment renal impairments in children diagnosed with WD. Objective: To assess renal impairments before treatment initiation in children with Wilson’s disease. Methods: This cross-sectional observational study was conducted in the Department of Pediatric Gastroenterology and Nutrition at Bangabandhu Sheikh Mujib Medical University from January to December 2021. A total of 36 children (both sexes, aged ≤18 years) diagnosed with WD were included. Informed written consent was obtained from parents or caregivers. Clinical, biochemical, and radiological parameters were analyzed using SPSS 20.0. Results: Among the 36 cases, 64% were male, with a predominant age range of 5–10 years. Renal impairment was observed in 19.4% of cases. The most common presenting feature was jaundice (58.3%), followed by a family history of liver disease (47%) and parental consanguinity (36%). Renal manifestations included proteinuria (55.6%), hematuria (44.5%), and bilateral pedal edema (30.6%). Hepatomegaly (69.4%), splenomegaly (63.9%), and ascites (66.7%) were frequently noted. Biochemical analysis revealed elevated serum ALT, prolonged INR, and hypoalbuminemia, indicating hepatic dysfunction. The mean hemoglobin level was low (10.09±1.38 g/dL), with Coombs-negative hemolytic anemia. Renal function tests showed elevated serum creatinine in 22.2% of cases. Urinalysis detected RBCs in 44.4% and urinary albumin in 55.6% of cases, with significant RBC (>10/HPF) in two cases and significant proteinuria ...