title: Incontinence in children, adolescents and adults with Williams syndrome.
abstract: Williams Syndrome (WS) is a microdeletion syndrome (chromosome 7q11.23) characterized by typical facial features, cardiovascular disease, behavioural symptoms, and mild intellectual disability (ID). The aim of this study was to assess the rates of incontinence and psychological problems in persons with WS.
  
  
  
  231 individuals with WS were recruited through the German parent support group (52.0% male, mean age 19.4 years). Faecal incontinence (FI) was diagnosed from the age of 4 years and nocturnal enuresis (NE) and daytime urinary incontinence (DUI) of 5 years onwards. The Parental Questionnaire: Enuresis/Urinary Incontinence, the International-Consultation-on-Incontinence-Questionnaire-Pediatric LUTS (ICIQ-CLUTS), as well as the Developmental Behavior Checklist for parents (DBC-P) or for adults (DBC-A) were filled out by parents or caregivers.
  
  
  
  17.8% of the sample had NE, 5.9% DUI and 7.6% FI. NE was present in 44.9% of children (4-12 years), 13.5% of teens (13-17y), 3.3% of young adults (18-30y) and in 3.6% of adults (> 30y). DUI (and FI) decreased from 17.9% (21.4%) in children to 0% in adults. 3.5% of the sample had an ICIQ-CLUTS score in the clinical range. 30.5% of children and 22.1% of adults had a clinical DBC score. Children and teens with clinically relevant DBC-P-scores had significantly higher DUI rates.
  
  
  
  Children with WS have high rates of incontinence and LUTS, which decrease with age. Most adults are continent. NE is the most common subtype. Except for DUI in children, incontinence is not associated with behavioural problems. Screening, assessment and treatment of incontinence in individuals with WS is recommended. Neurourol. Urodynam. 35:1000-1005, 2016. © 2015 Wiley Periodicals, Inc.
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg, Germany. alexander.von.gontard@uks.eu.
authors.forename: Alexander
authors.initials: A
authors.lastname: von Gontard
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg, Germany.
authors.forename: Justine
authors.initials: J
authors.lastname: Niemczyk
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg, Germany.
authors.forename: Sorina
authors.initials: S
authors.lastname: Borggrefe-Moussavian
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg, Germany.
authors.forename: Catharina
authors.initials: C
authors.lastname: Wagner
authors.affiliation: Department of Clinical Genetics, Governor Kremers Centre, Maastricht University Medical Centre, Maastricht, The Netherlands.
authors.forename: Leopold
authors.initials: L
authors.lastname: Curfs
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Equit
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002653:Child Behavior Disorders / Q000150:complications / Q000209:etiology / Q000523:psychology; D002675:Child, Preschool; D003248:Constipation / Q000453:epidemiology / Q000209:etiology; D005242:Fecal Incontinence / Q000453:epidemiology / Q000209:etiology* / Q000523:psychology; D005260:Female; D006801:Humans; D008297:Male; D053206:Nocturnal Enuresis / Q000209:etiology / Q000523:psychology; D010290:Parents; D015995:Prevalence; D011795:Surveys and Questionnaires; D014549:Urinary Incontinence / Q000453:epidemiology / Q000209:etiology* / Q000523:psychology; D018980:Williams Syndrome / Q000150:complications* / Q000453:epidemiology / Q000523:psychology; D055815:Young Adult
keywords: DBC; LUTS; Williams syndrome; behavioural symptoms; daytime urinary; faecal incontinence; incontinence; nocturnal enuresis