Early mortality and cardiorespiratory failure in patients with fibrodysplasia ossificans progressiva.

ABSTRACT:

Background

Fibrodysplasia ossificans progressiva, a rare genetic disorder of progressive extraskeletal ossification, is the most disabling form of heterotopic ossification in humans. However, little is known about the lifespan or causes of mortality in these patients. We undertook this study to determine the lifespan and causes of mortality in individuals who had fibrodysplasia ossificans progressiva.

Methods

We reviewed comprehensive mortality reports from two large registries of patients with fibrodysplasia ossificans progressiva. Together, these registries comprise >90% of all known patients with this condition in the world. We noted the sex, dates of birth and death, and the cause of death for each individual. We verified the cause of death with extensive medical records

SUBMITTER: Kaplan FS 

PROVIDER: S-EPMC2827822 | biostudies-literature | 2010 Mar

REPOSITORIES: biostudies-literature