Volume 39, Issue 6 pp. 563-568

Special stain and X-ray probe microanalysis of livers with Wilson disease

Hisao Hayashi

Corresponding Author

Hisao Hayashi

Department of Medicine, Aichi Gakuin University School of Pharmacy,

Dr Hisao Hayashi, 1-100 Kusumoto-cho, Chikusa-ku, Nagoya, 464-8650, Japan. Email: [email protected]Search for more papers by this author
Yoshikazu Fujita

Yoshikazu Fujita

Medical Research Engineering,

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Yasuaki Tatsumi

Yasuaki Tatsumi

Department of Medicine, Aichi Gakuin University School of Pharmacy,

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Ai Hattori

Ai Hattori

Department of Medicine, Aichi Gakuin University School of Pharmacy,

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Kazuhiko Hayashi

Kazuhiko Hayashi

Department of Gastroenterology, and

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Yoshiaki Katano

Yoshiaki Katano

Department of Gastroenterology, and

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Shinya Wakusawa

Shinya Wakusawa

Medical Laboratory Sciences, Nagoya University Graduate School of Medicine,

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Motoyoshi Yano

Motoyoshi Yano

Department of Gastroenterology, Yokkaichi City Hospital,

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Masafumi Itoh

Masafumi Itoh

Department of Pathology, First Red Cross Hospital of Nagoya,

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Naoki Mizutani

Naoki Mizutani

Department of Pediatric, Konan Kosei Hospital, Japan

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Hidemi Goto

Hidemi Goto

Department of Gastroenterology, and

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First published: 22 May 2009
Citations: 1

Abstract

Aim: Primary copper toxicosis due to Wilson disease is clinically complex, often leading to delayed diagnosis. Because the metabolic disorder is frequently complicated by iron overload due to hypoceruloplasminemia, either a special stain or microanalysis has been recommended for liver biopsy specimens.

Methods: Liver biopsy was performed in three patients in whom Wilson disease was highly suspected. Light microscopic study included rubeanic acid stain for copper and Berlin blue stain for iron. To improve the resolution of ultra-structures and preservation of toxic metals, short-term fixation with a 0.1% osmic acid solution was applied for X-ray probe microanalysis. Their diagnosis was confirmed by genetic study and copper chelation therapy.

Results: Two patients at the age of 17 and 23 years, respectively, demonstrated cirrhotic livers surrounded by fibrous septa, while a 7-year-old patient demonstrated fatty liver with mildly expanded portal tracts. Both copper grains stained with rubeanic acid and cuprothionein by microanalysis were found in the cirrhotic livers of aged patients. However, either morphological method failed to detect copper deposition in fatty liver tissues from the young patient. Iron deposits were also found in the cirrhotic livers of aged patients. The molecular basis of Wilson disease was confirmed by gene analysis. All patients responded to copper chelation therapy.

Conclusion: A morphological method of special staining or microanalysis improved with a new fixative may be unreliable for detecting diffusely distributed copper in the early stage of Wilson liver disease.

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