Aurélia Poujois1, France Woimant1, Solène Samson2, Pascal Chaine1, Nadège Girardot-Tinant1, Philippe Tuppin3. 1. Département de Neurologie, Centre National de Référence pour la Maladie de Wilson, Hôpital Lariboisière, Assistance publique-Hôpitaux de Paris, 75010 Paris, France. 2. 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. 3. 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. Electronic address: philippe.tuppin@cnamts.fr.
Abstract
BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND AND AIMS: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
Authors: M Opatowski; P Tuppin; K Cosker; M Touat; G De Lagasnerie; D Guillemot; J Salomon; C Brun-Buisson; L Watier Journal: Epidemiol Infect Date: 2019-01 Impact factor: 2.451
Authors: Eun Ju Choe; Jong Won Choi; Minjin Kang; Yong Kang Lee; Han Ho Jeon; Byung Kyu Park; Sun Young Won; Yong Suk Cho; Jeong Hun Seo; Chun Kyon Lee; Jae Bock Chung Journal: Sci Rep Date: 2020-08-20 Impact factor: 4.379