Increased Risk of Injury in Patients with Fabry Disease: A Nationwide Population-Based Cohort Study in Taiwan.

BACKGROUND

Fabry disease (FD) is a lysosomal storage disorder leading to α-galactosidase A deficiency and glycosphingolipid accumulation. Neurological manifestations of FD, such as central nervous system involvement, peripheral neuropathy, and vestibular dysfunction that results in postural and cognitive impairment, may predispose individuals to injuries. However, the associations between the risk of injury and FD remains underexplored.

METHODS

Using the Taiwanese National Health Insurance Research Database, we conducted a nationwide cohort study of adult FD patients (International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code 272.7)) who were diagnosed between 2001 and 2015. The comparison group was matched at a 1:4 ratio by age, sex, and index date. Injuries were identified via ICD-9-CM codes 800-999. Multivariate Cox proportional hazards models were applied to study the risk of injury, adjusting for sociodemographic factors and comorbidities.

RESULTS

The injury incidence rate was higher in the FD cohort (32.97/1,000 person-years) than in the control cohort (22.52/1,000 person-years). FD was significantly related to an increased risk of injury (adjusted hazard ratio: 1.642; 95% CI: 1.375-1.980; p < 0.001). FD patients had an 86% greater risk of motor vehicle traffic accidents and a 56% greater risk of falls than the control group. Higher risks of injury were also associated with the winter season, more complicated comorbidities, greater urbanization, and treatment at higher-level hospitals.

CONCLUSION

This is the first large-scale study to demonstrate a significantly increased risk of injury among FD patients. These findings highlight the need for proactive injury risk assessments and tailored prevention strategies for this population. Future research should investigate the role of FD-related clinical phenotypes in injury susceptibility to enhance personalized care and improve outcomes.