Journal of Clinical and Translational Hepatology

Journal of Clinical and Translational Hepatology

Wednesday, 01 / 29 / 2020

Articles

Alanine Aminotransferase as the First Test Parameter for Wilson’s Disease

ORIGINAL ARTICLE

Alanine Aminotransferase as the First Test Parameter for Wilson’s Disease

Hisao Hayashi*,1, Kazumasa Watanabe2, Ayano Inui3, Ayako Kato1, Yasuaki Tatsumi1, Akihiko Okumura2, Tomoo Fujisawa3 and Koichi Kato1

1Department of Medicine, Aichi Gakuin University School of Pharmacy, Chikusa-ku, Nagoya, Japan
2Department of Gastroenterology, Kainan Hospital, Yatomi, Japan
3Department of Pediatric Gastroenterology and Hepatology, Saiseikai Yokohama Toub Hospital, Yokohama, Kanagawa, Japan

*Correspondence to: Hisao Hayashi, Department of Medicine, Aichi Gakuin University School of Pharmacy, 1–100 Kusumoto-cho, Chikusa-ku, Nagoya 464-8650, Japan. Tel: +81-52-757-6779, E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

Journal of Clinical and Translational Hepatology 2019;7(4):293-296 DOI: 10.14218/JCTH.2019.00042
Received: September 5, 2019 Accepted: October 30, 2019 Published online: November 29, 2019

Abstract

Background and Aims: The liver is the first organ affected by toxic copper in the classical and severe hepatic forms of Wilson’s disease (WD). Because their associated chronic liver damage is mostly asymptomatic, an intervention using a special test including serum alanine aminotransferase (ALT) activity is needed for detecting WD.

Methods: Using the modified international criteria for the diagnosis of WD, 45 patients were selected from the collective databases of our institutions, and 7 infants were reviewed from the literature. Two patients had the severe hepatic form, with normoceruloplasminemia and no mutations in ATP7B. The rapid ALT change during hemolytic anemia was adjusted for a baseline. The diagnostic potential of the ALT test was assessed from the age-dependent natural course of the liver damage of WD.

Results: The natural course had three stages. ALTs were still low in some infants younger than 4 years-old. They were high in all children between the ages of 4 and 8 years-old; then, they reduced to low levels in some patients over 9 years of age. The high ALT stage represents chronic active hepatitis, and the subsequent low ALT stage is due to silent cirrhosis. The hepatic copper content is a reliable but invasive test, while urinary copper secretion is an alternative, non-invasive test for copper toxicosis of WD. The serum ceruloplasmin and ATP7B analyses are subtype tests of WD. The response to anti-copper regimens is the final test result.

Conclusions: ALT could be the first parameter to test to detect WD in children between the ages of 4 and 8 years.

Keywords

Alanine aminotransferase, ATP7B, Ceruloplasmin, Chronic active hepatitis, Wilson’s disease

Journal of Clinical and Translational Hepatology 2019 vol. 7, 293-296  [ Html ] [ PDF Full-text ]

© The Authors 2019. This article is published under the terms of the Creative Commons Attribution-Noncommercial License (CC BY-NC 4.0), which permits use, distribution, and reproduction in any medium, provided the original work is properly cited, the use is non-commercial and is otherwise in compliance with the license.

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