Lower Urinary Tract Symptoms and Incontinence in Children with Pompe Disease.

ABSTRACT:

Background

Pompe disease (PD) is a disorder of lysosomal glycogen storage. The introduction of enzyme replacement therapy (ERT) has shifted the focus of care from survival to quality of life. The presence of lower urinary tract symptoms (LUTS) and incontinence has not been previously described in children with PD.

Methods

Children with PD followed in the Duke Lysosomal Storage Disease Clinic completed a validated bladder control symptom score (BCSS) and additional questions regarding urinary tract infections (UTIs), giggle, and stress incontinence. Descriptive statistics were used to discriminate urinary symptoms between gender, age, and different types of PD.

Results

Sixteen of 23 children (aged 4-14 years) seen in our clinic participated. Seven were girls; ten had c

SUBMITTER: Ajay D 

PROVIDER: S-EPMC5059197 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature