Wilson's Disease in Bangladeshi Children: Analysis of 100 Cases / 대한소아소화기영양학회지
Pediatric Gastroenterology, Hepatology & Nutrition
; : 121-127, 2015.
Article
em En
| WPRIM
| ID: wpr-72732
Biblioteca responsável:
WPRO
ABSTRACT
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 21. 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.Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Penicilamina
/
Tempo de Protrombina
/
Bangladesh
/
Ceruloplasmina
/
Cristianismo
/
Falência Hepática Aguda
/
Cobre
/
Hepatite
/
Degeneração Hepatolenticular
/
Hipertensão Portal
Tipo de estudo:
Observational_studies
Limite:
Child
/
Female
/
Humans
/
Male
País/Região como assunto:
Asia
Idioma:
En
Revista:
Pediatric Gastroenterology, Hepatology & Nutrition
Ano de publicação:
2015
Tipo de documento:
Article