지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아소화기영양학회 Pediatric Gastroenterology, Hepatology & Nutrition Pediatric Gastroenterology, Hepatology & Nutrition 제18권 제2호
- 발행연도
- 2015.1
- 수록면
- 121 - 127 (7page)
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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 μg/day was found in 81% cases and urinary copper following penicillamine challenge of ＞1,200 μg/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.
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참고문헌 (28)
참고문헌 신청
Cavanaugh J / 1990 / Gynecomastia and cirrhosis of the liver / Arch Intern Med 150 : 563 ~ 565
Czaja AJ / 2002 / Diagnosis and treatment of autoimmune hepatitis / Hepatology 36 : 479 ~ 497
Deguti MM / 2002 / The eye in Wilson's disease:
El-Karaksy H / 2011 / A clinical study of Wilson's disease: The experience of a single Egyptian Paediatric Hepatology Unit / Arab J Gastroenterol 12 : 125 ~ 130
Epstein O / 1981 / Prospective study of periostitis and finger clubbing in primary biliary cirrhosis and other forms of chronic liver disease / Gut 22 : 203 ~ 206
Czaja AJ / 2002 / Diagnosis and treatment of autoimmune hepatitis / Hepatology 36 : 479 ~ 497
Deguti MM / 2002 / The eye in Wilson's disease:
El-Karaksy H / 2011 / A clinical study of Wilson's disease: The experience of a single Egyptian Paediatric Hepatology Unit / Arab J Gastroenterol 12 : 125 ~ 130
Epstein O / 1981 / Prospective study of periostitis and finger clubbing in primary biliary cirrhosis and other forms of chronic liver disease / Gut 22 : 203 ~ 206
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