ArticlePediatric gastroenterology, hepatology & nutrition2015
Wilson's Disease in Bangladeshi Children: Analysis of 100 Cases.
Article in Pediatric gastroenterology, hepatology & nutrition, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 54 citations in OpenAlex.
- Clinical Characteristics, Treatment Effects and Risk Factors of Liver Cirrhosis in Patients with Wilson's Disease Hepatic Type.Journal of clinical and translational hepatology · 2025Article
- Efficacy and safety of D-penicillamine, trientine, and zinc in pediatric Wilson disease patients.Orphanet journal of rare diseases · 2024Article
- Addressing the Challenges in the Diagnosis and Management of Pediatric Wilson's Disease-Case Report and Literature Review.Medicina (Kaunas, Lithuania) · 2023Review
- Brain MRI in the Decision for Liver Transplantation in Pediatric Neurological Wilson's Disease.Movement disorders clinical practice · 2022Article
- Mutational analysis of exon 8 and exon 14 of ATP7B gene in Bangladeshi children with Wilson disease.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2022Article
- Early Diagnosis of Wilson's Disease in Children in Southern China by Using Common Parameters.Frontiers in genetics · 2022Article
- Screening for Wilson's disease in acute liver failure: A new scoring system in children.Frontiers in pediatrics · 2022Article
- Mental Health, Cognitive, and Neuropsychiatric Needs in Children and Young People With Wilson Disease.JPGN reports · 2021Article
- Case Report: The Association of Wilson Disease in a Patient With Ataxia and GLUT-1 Deficiency.Frontiers in pediatrics · 2021Article
- The optimal threshold of serum ceruloplasmin in the diagnosis of Wilson's disease: A large hospital-based study.PloS one · 2018Article
- Functional Characterization of NovelFrontiers in pediatrics · 2018Article
- Neurological features and management of Wilson disease in children: an evaluation of 12 cases.Turk pediatri arsivi · 2016Article
- Pediatric Wilson's disease: findings in different presentations. A cross-sectional study.Sao Paulo medical journal = Revista paulista de medicinaArticle
- High Prevalence of Wilson's Diseases with Low Prevalence of Kayser-Fleischer Rings among Patients with Cryptogenic Chronic Liver Diseases in Bangladesh.Euroasian journal of hepato-gastroenterologyArticle
- Wilson disease in children and young adults - State of the art.Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology AssociationReview
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1 author at 1 institution in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
purposeTo evaluate clinical and laboratory profile of Wilson's disease (WD) in children.
methodsThis 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.
resultsMean 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 µg/day was found in 81% cases and urinary copper following penicillamine challenge of >1,200 µg/day was found in 92% cases.
conclusionMajority 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.
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