Clinical Analysis of 274 Cases of Wilson's Disease in Children
10.11969/j.issn.1673-548X.2024.05.020
- VernacularTitle:274例儿童肝豆状核变性临床分析
- Author:
Daiping HUA
1
;
Han WANG
;
Lanting SUN
Author Information
1. 230031 合肥,安徽中医药大学研究生院
- Keywords:
Wilson's disease;
Clinical analysis;
Clinical classification;
Gender;
Children
- From:
Journal of Medical Research
2024;53(5):98-101,107
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical characteristics of children with Wilson's disease,and to improve the attention to Wil-son's disease in children.Methods The clinical data of 274 children with Wilson's disease admitted to the First Affiliated Hospital of Anhui University of Chinese Medicine from November 2020 to October 2022 were retrospectively analyzed.Results Among 274 children with Wilson's disease,aged 3 to 15 years,with a male/female ratio of 1.88∶1,the median age of onset was 5.6 years.Clinical classifica-tion:204 cases(74.5％)of liver type,19 cases(6.9％)of brain type,41 cases(15.0％)of mixed type,10 cases(3.6％)of other type.Age of onset:liver type＜other type＜mixed type＜brain type,the differences were statistically significant(P＜0.05).The main reasons for the initial diagnosis of 274 children were physical examination,family history and respiratory tract infection.Among them,135 cases(49.3％)with asymptomatic elevated transaminase.The abnormal rate of serum ceruloplasmin and 24-hour urine copper was 100％and 96.7％,respectively.The abnormal rates of hepatobiliary and spleen ultrasound,kidney ultrasound and craniocerebral mag-netic resonance imaging were 81.0％(204/218),15.6％(34/218)and 41.7％(48/115),respectively.The abnormal rate of hepato-biliary and spleen ultrasound and craniocerebral magnetic resonance examination increased with age,and the abnormal rate of renal ultra-sound and craniocerebral magnetic resonance examination was related to clinical classification.Conclusion The onset age of Wilson's disease in children is young,the initial symptoms are different,which is closely related to age and clinical classification,and has nothing to do with gender.For suspected children,relevant examinations should be improved to achieve the purpose of early diagnosis and treatment.
