Prevalence of fibrodysplasia ossificans progressiva (FOP) in France: an estimate based on a record linkage of two national databases.

ABSTRACT:

Background

Fibrodysplasia ossificans progressiva (FOP) is a rare, severely disabling, and life-shortening genetic disorder that causes the formation of heterotopic bone within soft connective tissue. Previous studies found that the FOP prevalence was about one in every two million lives. The aim of this study is to estimate the FOP prevalence in France by probabilistic record-linkage of 2 national databases: 1) the PMSI (Programme de médicalisation des systèmes d'information), an administrative database that records all hospitalization activities in France and 2) CEMARA, a registry database developed by the French Centres of Reference for Rare Diseases.

Results

Using a capture-recapture methodology to adjust the crude number of patients identified in both data sources, 89 FO

SUBMITTER: Baujat G 

PROVIDER: S-EPMC5493013 | biostudies-literature | 2017 Jun

REPOSITORIES: biostudies-literature