The Clinical Analysis of Wilson Disease in Children
Author：HUANG Ying, HE Ting-yan
affiliation：Department of Kidney and Immunology, Shenzhen Children's Hospital, Shenzhen 518026, Guangdong Province, China
PDFAbstract
Objective Clinical data of children with Wilson Disease (WD) was reviewed to make early diagnosis and improve the prognosis. Methods The clinical and laboratory characteristics of 38 children with Wilson Disease were investigated and retrospectively analyzed. All patients were divided into two groups as younger age group and elder age group. Differences between groups were further analyzed. Results The mean age at diagnosis was 6.5 years old. The initial manifestations were impairment of liver function (18cases), symptoms of central nervous system (11cases), mixed symptoms of both organs (4cases), and others (5cases). Serum ceruloplasmin level was decreased in 31cases (91.2%). Fleischer ring was checked in 29 patients and only 12 cases were positive, which made a difference between the two age groups. Brain CT or MRI was performed in 15 cases, and symmetrical abnormal signal of bilateral basal ganglia area was found in 8 cases. Conclusion Clinical manifestations of Wilson Disease in children are complex and diverse. Further research is needed to make early diagnosis and improve prognosis.
【Keyword】Wilson Disease; Ceruloplasmin; Children
【Chart number】R725.8
【Document Identification Number】A
【DOI】10.3969/j.issn.1009-3257.2016.04.014
Journal of Rare Diseases
th23Volume， th 5 Issue
2019Year11Month
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