2024-03-29T08:46:54Z
https://nagoya.repo.nii.ac.jp/oai
oai:nagoya.repo.nii.ac.jp:00017409
2023-11-16T05:19:50Z
499:508:509:1563
Biochemical Staging of the Chronic Hepatic Lesions of Wilson Disease
KATANO, YOSHIAKI
51164
HAYASHI, KAZUHIKO
51165
HATTORI, AI
51166
TATSUMI, YASUAKI
51167
UEYAMA, JUN
51168
WAKUSAWA, SHINYA
51169
YANO, MOTOYOSHI
51170
TOYODA, HIDENORI
51171
KUMADA, TAKASHI
51172
MIZUTANI, NAOKI
51173
HAYASHI, HISAO
51174
GOTO, HIDEMI
51175
AST
ALT
Phenotype
Stage
Wilson disease
2014-02
Background: Copper toxicity steadily affects the livers of patients with Wilson disease. However, the toxic effect of copper on serum aspartate and alanine aminotransferase levels remains to be clarified as a prerequisite for diagnostic tests. The clinical records of 33 cases were analyzed to clarify the natural history of Wilson disease. Phenotypes were simplified into hepatic, acute, and neurologic. The bio-low stage of both enzymes was less than 40 IU/L, the bio-moderate stage was intermediate between 40 and 200 IU/L, and the bio-high stage was more than 200 IU/L of either or both enzymes. Rebounded enzyme levels at the recovery period from anemia were presumed to be the chronic baselines when pre-anemic enzyme levels were not available in the acute phenotype. We investigated whether these enzyme levels may provide information useful for screening patients. The natural history of chronic Wilson disease consisted of the first increasing and second decreasing phases. The clinical courses of a 4-year-old boy and 12-year-old girl were representative of the 2 phases, respectively. All but one patient were in the decreasing phase. Negative correlations were obtained between age and enzyme level in the decreasing phase. The hepatic phenotype may be a prototype found throughout the 2 phases, and acute and neurologic phenotypes may be major complications in the bio-moderate and bio-low stages of the decreasing phase, respectively. Biochemical staging may provide a better understanding of Wilson disease when combined with phenotypes. Bio-high stage patients should be referred to a medical center for diagnosis.
departmental bulletin paper
Nagoya University School of Medicine
2014-02
Nagoya Journal of Medical Science
1-2
76
139
148
http://hdl.handle.net/2237/19492
2186-3326
0027-7622
eng
http://www.med.nagoya-u.ac.jp/medlib/nagoya_j_med_sci/7612/7612.html