Poujois Aurélia, Woimant France, Samson Solène, Chaine Pascal, Girardot-Tinant Nadège, Tuppin Philippe
Département de Neurologie, Centre National de Référence pour la Maladie de Wilson, Hôpital Lariboisière, Assistance publique-Hôpitaux de Paris, 75010 Paris, France.
Direction de la stratégie des études et des statistiques, Caisse Nationale d'Assurance Maladie, 50, avenue du Professeur-André-Lemierre, 75986 Paris cedex 20, France.
Clin Res Hepatol Gastroenterol. 2018 Feb;42(1):57-63. doi: 10.1016/j.clinre.2017.05.011. Epub 2017 Jun 23.
Only a few epidemiological studies on the incidence and prevalence of Wilson's disease (WD) have been performed to date, and the results vary widely according to the reports. The aim of the study was to investigate the prevalence, ambulatory care and treatments of patients with WD in France.
Among the 58 million general health scheme beneficiaries (86% of the French population), people managed for WD in 2013 were identified using hospitalisation diagnosis in 2011-2013 or specific long-term disease status with a 100% reimbursement for specific healthcare in 2013. Data were derived from the Sniiram (National Health Insurance Information System database). Prevalence by age and sex were calculated.
In 2013, 906 prevalent cases were identified, yielding a crude prevalence of 1.5 cases per 100,000; 1.65 per 100,000 in males and 1.44 per 100,000 in females. This prevalence is comparable to that reported in other population-based studies in European countries and to a study using a similar method. Almost 40% of patients were treated by D-penicillamine and 14.3% were treated by zinc acetate. Trientine, delivered on a compassionate basis, was not available in the reimbursement database. In 2013, 1.3% of patients underwent liver transplantation and 4% had already undergone liver transplantation in previous years. Fifteen per cent of patients received antidepressants, a higher rate than in general population.
This is the first French population-based epidemiological study of WD in a comprehensive population based on administrative data and constitutes an important step to understand the impact of WD and to study quality of care.
迄今为止，仅开展了少数关于威尔逊病（WD）发病率和患病率的流行病学研究，且结果因报告而异。本研究旨在调查法国WD患者的患病率、门诊治疗及治疗情况。
在5800万基本医疗保险受益人（占法国人口的86%）中，通过2011 - 2013年的住院诊断或2013年特定长期疾病状态（特定医疗保健100%报销）来确定2013年接受WD治疗的患者。数据来源于Sniiram（国家医疗保险信息系统数据库）。计算了年龄和性别的患病率。
2013年，共识别出906例现患病例，粗患病率为每10万人1.5例；男性为每10万人1.65例，女性为每10万人1.44例。该患病率与欧洲其他基于人群的研究以及一项采用类似方法的研究报告的患病率相当。近40%的患者接受青霉胺治疗，14.3%的患者接受醋酸锌治疗。在报销数据库中未提供基于同情用药提供的曲恩汀。2013年，1.3%的患者接受了肝移植，4%的患者在前几年已接受过肝移植。15%的患者接受了抗抑郁药治疗，这一比例高于普通人群。
这是法国首次基于行政数据在全面人群中开展的WD流行病学研究，是了解WD影响和研究医疗质量的重要一步。
