Expanding our understanding of lower urinary tract symptoms and incontinence in adults with pompe disease.

Journal Article (Journal Article)

OBJECTIVE: To study the prevalence of lower urinary tract symptoms (LUTS) and incontinence in late-onset Pompe disease (LOPD) Methods: Adult LOPD patients seen at the Duke Pompe Clinic were prospectively recruited and asked to complete validated questionnaires on LUTS and incontinence as part of an IRB-approved study. Patient demographics as well as previous urologic history were reviewed. RESULTS: 35 patients with LOPD were included in the study (17 males and 18 females). The median age was 51.8 (range 18-72 years of age). Of these patients, 27/35 were receiving enzyme replacement therapy (ERT) with median duration of 54 months (range 5-88 months). In the male patients, 9/17 (53%) described their stream as dribbling, weak, or intermittent, and 9/17 (53%) complained of post-void dribbling. In addition 38% of the men were unable to stop their urination midstream. In the female patients, the most common complaint was urinary incontinence, reported in 14/18 (78%). In addition, 7/18 (39%) complained of post-void dribbling, and 47% were unable to stop their urination midstream. Bowel incontinence was reported in 45% of patients. There was a significant association between urinary symptoms and lower extremity function scores and duration of ERT (p = 0.005 and p = 0.04, respectively) CONCLUSIONS: This is the first study in a large cohort of LOPD patients that demonstrates LUTS and incontinence occur at a high rate. This study emphasizes the spectrum of LOPD is beyond isolated gross motor and pulmonary involvement and has a significant effect on the lower urinary tract.

Full Text

Duke Authors

Cited Authors

  • McNamara, ER; Austin, S; Case, L; Wiener, JS; Peterson, AC; Kishnani, PS

Published Date

  • 2015

Published In

Volume / Issue

  • 20 /

Start / End Page

  • 5 - 10

PubMed ID

  • 25614307

Pubmed Central ID

  • PMC4375115

International Standard Serial Number (ISSN)

  • 2192-8304

Digital Object Identifier (DOI)

  • 10.1007/8904_2014_381


  • eng

Conference Location

  • United States