title: Urge incontinence and gastrointestinal symptoms in adult patients with pompe disease: a cross-sectional survey.
abstract: OBJECTIVE
  To determine the frequency and impact of gastrointestinal symptoms, and bowel and urinary incontinence, as this is currently unknown in adults with Pompe disease.
  
  
  METHODS
  Adult German Pompe patients and age- and gender-matched controls were asked about symptoms in the upper and lower intestinal tract as well as urinary incontinence using the Gastrointestinal Symptoms Questionnaire and the International Consultation on Incontinence Questionnaires for Bowel Symptoms and Urinary Incontinence.
  
  
  RESULTS
  The overall response rate was 78%; 57 patients and 57 controls participated. The mean age of the patients was 48.3 years ±14.7 (28 female, 29 male). 84% of patients were receiving enzyme replacement therapy. Stool urgency, diarrhoea, and urinary urge incontinence were reported significantly more frequently in patients compared to the age- and gender-matched controls (55%, 56%, 33% vs. 20%, 18%, 7%). 20% of Pompe patients used loperamide daily against diarrhoea. No other gastrointestinal tract-related symptoms were reported to occur more frequently in Pompe patients than in controls.
  
  
  CONCLUSIONS
  Compared to age- and gender-matched controls, both urinary and bowel incontinence occur in a higher frequency in adults with Pompe disease and have a major impact on daily life.
authors.affiliation: Villa Metabolica, Centre for Pediatric and Adolescent Medicine, Langenbeckstr. 2, 55131, Mainz, Germany.
authors.forename: Nesrin
authors.initials: N
authors.lastname: Karabul
authors.forename: Anika
authors.initials: A
authors.lastname: Skudlarek
authors.forename: Janine
authors.initials: J
authors.lastname: Berndt
authors.forename: Cornelia
authors.initials: C
authors.lastname: Kornblum
authors.forename: Rudolf A
authors.initials: RA
authors.lastname: Kley
authors.forename: Stephan
authors.initials: S
authors.lastname: Wenninger
authors.forename: Nikolaus
authors.initials: N
authors.lastname: Tiling
authors.forename: Eugen
authors.initials: E
authors.lastname: Mengel
authors.forename: Ursula
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authors.lastname: Plöckinger
authors.forename: Matthias
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authors.lastname: Vorgerd
authors.forename: Marcus
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authors.lastname: Deschauer
authors.forename: Benedikt
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authors.lastname: Schoser
authors.forename: Frank
authors.initials: F
authors.lastname: Hanisch