Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson's Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma | ||
| Journal of Pediatric Perspectives | ||
| مقاله 9، دوره 6، شماره 12 - شماره پیاپی 60، اسفند 2018، صفحه 8697-8705 اصل مقاله (822.83 K) | ||
| شناسه دیجیتال (DOI): 10.22038/ijp.2018.33235.2932 | ||
| نویسندگان | ||
| Naghi Dara1؛ Amirhossein Hosseni* 1؛ Saleheh Tajalli2؛ Mohammad Amin Shahrbaf3؛ Ali Akbar Sayyari4؛ Farid Imanzadeh5؛ Katayoun Khatami1؛ Pejman Rohani1؛ Maliheh Khodami6؛ Maryam Kazemi Aghdam6 | ||
| 1Assiatant Professor of Pediatric Gastroenterohepatology, Pediatric Gastroenterology, Hepatology and Nutrition Research Center, Research Institute for Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran. | ||
| 2MSc of Neonatal Intensive Care, Neonatal Health Research Center, Research Institute For Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran. | ||
| 3Medical Student, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran. | ||
| 4Professor of Pediatric Gastroenterohepatology, Pediatric Gastroentrology, Hepatology and Nutrition Research Center, Research Institute For Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran. | ||
| 5Associated Professor of Pediatric Gasteroenterohepatology, Pediatric Gastroentrology, Hepatology and Nutrition Research Center, Research Institute for Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran. | ||
| 6Pediatric Pathology Research Center, Research Institute for Children Health , 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. | ||
| کلیدواژهها | ||
| Autoimmune hepatitis؛ Atypical presentation؛ Children؛ Coexistence؛ Wilson disease | ||
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