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Information Journal Paper

Title

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

Pages

  8697-8705

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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    APA: Copy

    DARA, NAGHI, HOSSEINI, AMIRHOSSEIN, TAJALLI, SALEHEH, Shahrbaf, Mohammad Amin, SAYARI, ALI AKBAR, IMAN ZADEH, FARID, KHATAMI, KATAYOUN, ROHANI, PEJMAN, KHODAMI, MALIHEH, & KAZEMI AGHDAM, MARYAM. (2018). Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson's Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma. INTERNATIONAL JOURNAL OF PEDIATRICS, 6(12 (60)), 8697-8705. SID. https://sid.ir/paper/337707/en

    Vancouver: Copy

    DARA NAGHI, HOSSEINI AMIRHOSSEIN, TAJALLI SALEHEH, Shahrbaf Mohammad Amin, SAYARI ALI AKBAR, IMAN ZADEH FARID, KHATAMI KATAYOUN, ROHANI PEJMAN, KHODAMI MALIHEH, KAZEMI AGHDAM MARYAM. Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson's Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma. INTERNATIONAL JOURNAL OF PEDIATRICS[Internet]. 2018;6(12 (60)):8697-8705. Available from: https://sid.ir/paper/337707/en

    IEEE: Copy

    NAGHI DARA, AMIRHOSSEIN HOSSEINI, SALEHEH TAJALLI, Mohammad Amin Shahrbaf, ALI AKBAR SAYARI, FARID IMAN ZADEH, KATAYOUN KHATAMI, PEJMAN ROHANI, MALIHEH KHODAMI, and MARYAM KAZEMI AGHDAM, “Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson's Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma,” INTERNATIONAL JOURNAL OF PEDIATRICS, vol. 6, no. 12 (60), pp. 8697–8705, 2018, [Online]. Available: https://sid.ir/paper/337707/en

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