[Zhonghua yan ke za zhi] Chinese journal of ophthalmology · 2008
[Ophthalmic manifestations of Wilson's disease].
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Huo Li-Jun, Liao Rui-Duan, Chen Xue-Mei
Аннотация
OBJECTIVE: To investigate the ophthalmic manifestations of Wilson's disease (WD).
METHODS: In this nonrandomized retrospective case series, fifty-two WD patients were investigated retrospectively, based on the family history, clinical signs and the laboratory test Patients were divided into 4 groups: asymptomatic group, only with hepatic disorders, only with neurological disorders and with both hepatic and neurological disorders group.
RESULTS: Thirty two males and twenty females were studied. The ratio of male to female was 1.6:1.0. The mean age was (20 +/- 1) years, ranged from 4 to 53 years. The majority (78.8%) of patients belonged to younger age groups (10-30 years). The family history showed members with WD in 14 patients (26.9%), probable WD in 3 patients (5.8%). Out of 52 patients, 2 asymptomatic patients (3.8%) were detected by family screening, 12 patients (23.1%) only had hepatic disease, 11 patients (21.2%) only had neurological disorders and 27 patients (51.9%) had both hepatic and neurological disorders. Among 45 patients (86.5%) with Kayser-Fleischer (K-F) ring, 1 patient was asymptomatic, 7 patients had hepatic disease, 11 had neurological disorders and 26 had both hepatic and neurological disorders. One patient (1.9%) was presented with sunflower cataract. Fifty one patients (98.1%) had low serum ceruloplasmin levels and high urinary copper excretion.
CONCLUSIONS: K-F ring is a common and characteristic manifestation of WD. This is often found in patients with neurological disorders.
METHODS: In this nonrandomized retrospective case series, fifty-two WD patients were investigated retrospectively, based on the family history, clinical signs and the laboratory test Patients were divided into 4 groups: asymptomatic group, only with hepatic disorders, only with neurological disorders and with both hepatic and neurological disorders group.
RESULTS: Thirty two males and twenty females were studied. The ratio of male to female was 1.6:1.0. The mean age was (20 +/- 1) years, ranged from 4 to 53 years. The majority (78.8%) of patients belonged to younger age groups (10-30 years). The family history showed members with WD in 14 patients (26.9%), probable WD in 3 patients (5.8%). Out of 52 patients, 2 asymptomatic patients (3.8%) were detected by family screening, 12 patients (23.1%) only had hepatic disease, 11 patients (21.2%) only had neurological disorders and 27 patients (51.9%) had both hepatic and neurological disorders. Among 45 patients (86.5%) with Kayser-Fleischer (K-F) ring, 1 patient was asymptomatic, 7 patients had hepatic disease, 11 had neurological disorders and 26 had both hepatic and neurological disorders. One patient (1.9%) was presented with sunflower cataract. Fifty one patients (98.1%) had low serum ceruloplasmin levels and high urinary copper excretion.
CONCLUSIONS: K-F ring is a common and characteristic manifestation of WD. This is often found in patients with neurological disorders.