Journal of Clinical and Translational Hepatology

Journal of Clinical and Translational Hepatology

Saturday, 10 / 16 / 2021

Abstract

Acute Hepatic Phenotype of Wilson Disease: Clinical Features of Acute Episodes and Chronic Lesions Remaining in Survivors

Hisao Hayashi1, Yasuaki Tatsumi1, Shinsuke Yahata2, Hiroki Hayashi3, Kenji Momose4, Ryohei Isaji5, Youji Sasaki5, Kazuhiko Hayashi6, Shinya Wakusawa*7 and Hidemi Goto6

1Department of Medicine, Aichi-Gakuin University School of Pharmacy, Nagoya, Japan; 2Department of Gastroenterology, Hyogo Prefectural Kakogawa Medical Center, Kakogawa, Japan; 3Department of Gastroenterology, Kita-Harima Medical Center, Ono, Japan; 4Department of Gastroenterology, Kobe University School of Medicine, Kobe, Japan; 5Department of Gastroenterology, Konan Kosei Hospital, Konan, Japan; 6Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, Nagoya, Japan; 7Department of Medical Laboratory Sciences, Nagoya University Graduate School of Medicine, Nagoya, Japan

Abstract

Background and Aims: Wilson disease (WD) is an inherited disorder of copper metabolism, and an international group for the study of WD (IGSW) has proposed three phenotypes for its initial presentation: acute hepatic, chronic hepatic, and neurologic phenotypes. Characterization of the acute hepatic phenotype may improve our understanding of the disease. Methods: Clinical features of 10 WD patients with the acute hepatic phenotype and characteristics of chronic lesions remaining in survivors were assessed by the European Association for the Study of the Liver (EASL) guidelines. Results: All six patients younger than 30 years had survived an acute episode of hemolytic anemia with residual liver disease of cirrhosis or chronic hepatitis. The acute episode was self-limiting in two of the four patients over the age of 30 years and progressed to acute liver failure in the other two patients. One of the two survivors had residual liver disease of chronic hepatitis, while the other had chronic hepatitis and neurologic disease. Neurologic disease remained in a patient who successfully received a liver transplantation. During acute episodes, serum levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) changed rapidly along with anemia. Liver-specific ALT levels were age-dependently correlated with hemoglobin (Hb) concentrations. Enzyme reduction was milder for AST than ALT, which resulted in a high AST/ALT ratio in the anemic stage. The anemic stage in two patients transformed to acute liver failure. Conclusions: All survivors of an acute episode of the acute hepatic phenotype had residual liver disease or both liver and neurologic diseases. The rapid changes in liver enzymes during the acute episode and the liver and neurologic diseases remaining in survivors may provide a better understanding of WD.

Doi: 10.14218/JCTH.2015.00032
Journal of Clinical and Translational Hepatology 2015 vol. 3, 239-245  [
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