Causes of Death and Comorbidities in Adult Patients With Late-Onset Pompe Disease: A French Pompe Registry Retrospective Study.

ABSTRACT:

Background and objectives

Mortality in Late-Onset Pompe Disease (LOPD) has been associated with the rapid progression of respiratory and motor impairment. However, an in-depth approach to the exact causes of death in these patients is still lacking.

Methods

In this retrospective cohort study, we analyzed the cause of death and the comorbidities of all deceased patients from the French Late-Onset Pompe Disease registry.

Results

By the time of the last extraction, 60 patients diagnosed with LOPD and monitored were registered as deceased in the French national registry, out of a total of 260 patients included. The median age of death was 70.5 years, while the median age of diagnosis was 58 years. The causes of death were divided into disease-related, accounting for 46.6%

SUBMITTER: Chitimus DM 

PROVIDER: S-EPMC12554947 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature