肝豆状核变性患者的妊娠结局：一项基于中国汉族人群多中心的研究
Outcomes of pregnancy in Wilson's disease: a population-based study from multiple centres of the Han population in China.
作者信息
Rao Rao, Yu Xu-En, Zhou Zhi-Hua, Shu Shan, Du Yi-Gang, Han Yong-Zhu, Han Yong-Sheng
机构信息
Institute of Neurology, Anhui University of Chinese Medicine, Hefei, China.
Department of Neurology, Affiliated Hospital of Neurology Research Institute of Anhui University of Chinese Medicine, Hefei, China.
出版信息
Front Med (Lausanne). 2024 Aug 23;11:1436828. doi: 10.3389/fmed.2024.1436828. eCollection 2024.
OBJECTIVES
Wilson's disease is an autosomal recessive disorder related to copper metabolism which mostly patients occurs in adolescents, fertility has become a problem that WD needs to face.
METHODS
A 21 years retrospective follow up study was conducted and a total of 220 female patients were included to identify patients with outcomes of pregnancy.
RESULTS
Untreated female patients with WD had a spontaneous abortion rate of 44%. During the study period, 146 female patients with WD from multicenter, 75 patients (51.4%) had successful outcomes of pregnancy. Notably, urinary copper levels below 616 μg/24 h were strongly associated with successful pregnancy. The nomogram built on these variables were age, urinary copper, haemoglobin and Child-Pugh classification, internally validated and showed good performance.
CONCLUSION
The spontaneous abortion rate was 44% in untreated females with WD and developed a four-variable risk prediction model to accurately predict the likelihood of a successful pregnancy.
目的
威尔逊病是一种与铜代谢相关的常染色体隐性疾病，大多数患者发病于青少年期，生育问题已成为威尔逊病需要面对的一个难题。
方法
开展了一项为期21年的回顾性随访研究，共纳入220例女性患者，以确定有妊娠结局的患者。
结果
未经治疗的威尔逊病女性患者自然流产率为44%。在研究期间，来自多中心的146例威尔逊病女性患者中，75例（51.4%）妊娠结局成功。值得注意的是，24小时尿铜水平低于616μg与妊娠成功密切相关。基于年龄、尿铜、血红蛋白和Child-Pugh分级这些变量构建的列线图，经内部验证显示性能良好。
结论
未经治疗的威尔逊病女性患者自然流产率为44%，并建立了一个四变量风险预测模型以准确预测妊娠成功的可能性。
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