Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson's Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma

Authors

  • Ali Akbar Sayyari Professor of Pediatric Gastroenterohepatology, Pediatric Gastroentrology, Hepatology and Nutrition Research Center, Research Institute For Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
  • Amirhossein Hosseni 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.
  • Farid Imanzadeh Associated Professor of Pediatric Gasteroenterohepatology, Pediatric Gastroentrology, Hepatology and Nutrition Research Center, Research Institute for Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
  • Katayoun Khatami 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.
  • Maliheh Khodami Pediatric Pathology Research Center, Research Institute for Children Health , Shahid Beheshti University of Medical Sciences, Tehran, Iran.
  • Maryam Kazemi Aghdam Pediatric Pathology Research Center, Research Institute for Children Health , Shahid Beheshti University of Medical Sciences, Tehran, Iran.
  • Mohammad Amin Shahrbaf Medical Student, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
  • Naghi Dara 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.
  • Pejman Rohani 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.
  • Saleheh Tajalli MSc of Neonatal Intensive Care, Neonatal Health Research Center, Research Institute For Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Abstract:

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.

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Journal title

volume 6  issue 12

pages  8697- 8705

publication date 2018-12-01

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