Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson s Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma
- سال انتشار: 1397
- محل انتشار: مجله بین المللی کودکان، دوره: 6، شماره: 12
- کد COI اختصاصی: JR_INJPM-6-12_009
- زبان مقاله: انگلیسی
- تعداد مشاهده: 453
نویسندگان
Assiatant Professor of Pediatric Gastroenterohepatology, Pediatric Gastroenterology, Hepatology and Nutrition Research Center, Research Institute for Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Assiatant Professor of Pediatric Gastroenterohepatology, Pediatric Gastroenterology, Hepatology and Nutrition Research Center, Research Institute for Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
MSc of Neonatal Intensive Care, Neonatal Health Research Center, Research Institute For Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Medical Student, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
چکیده
Background: Wilson s disease (WD) is a genetic disorder with various clinical presentations due to excessive accumulation of copper in the liver and other organs. It can present as acute/chronic hepatitis, liver failure, extrahepatic and neuromuscular manifestations. Autoimmune hepatitis (AIH) is a necroinflammatory disease of the liver, which affects a lot of people particularly the children population. AIH has a broad clinical presentation that is similar to WD. Coexistence of WD with elevated creatinine phosphokinase (CPK) and AIH, may be a diagnostic dilemma. Case Report: We presented a 6 years old boy with dysarthria, aggressive behavior, weak attention, concentration and weight loss with abnormal physical examination. Laboratory, histochemical, genomic studies, muscle/liver biopsy and atomic absorption test confirmed the diagnosis of both WD and AIH in the boy. Conclusion Although CPK and liver enzyme elevation is a rare presentation of chronic hepatitis with dominant feature of WD and AIH; however, simultaneous therapy with immunosuppressive drugs and Penicillamine may have superior benefit with a significant response.کلیدواژه ها
Autoimmune hepatitis, Atypical presentation, Children, Coexistence, Wilson diseaseاطلاعات بیشتر در مورد COI
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