Incontinence in persons with Angelman syndrome

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• 作者：C Wagner ; J Niemczyk ; M Equit ; L Curfs ; A von Gontard
• 关键词：Angelman syndrome ; Nocturnal enuresis ; Daytime urinary incontinence ; Fecal incontinence ; Behavior ; Developmental Behaviour Checklist
• 刊名：European Journal of Pediatrics
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：176
• 期：2
• 页码：225-232
• 全文大小：
• 刊物主题：Pediatrics;
• 出版者：Springer Berlin Heidelberg
• ISSN：1432-1076
• 卷排序：176

文摘

Angelman syndrome (AS) is a congenital syndrome with a prevalence of 1:15,000. Individuals with AS often have severe intellectual disability, typical dysmorphic signs, and behavioral problems. The aim of the study was to investigate the rate of incontinence and associated psychological problems in children and adults with AS. Ninety children (4–18 years) and 54 adults (18–31 years) with AS were recruited through a parent support group (55.6% male, mean age 15.1 years). The Parental Questionnaire: Enuresis/Urinary Incontinence, the Incontinence Questionnaire-Pediatric Lower Urinary Tract Symptoms (ICIQ-CLUTS), as well as the Developmental Behaviour Checklist for parents (DBC-P) or for adults (DBC-A) were filled out by parents or caregivers. 85.6% of individuals with AS were affected by at least one subtype of incontinence (82.7% nocturnal enuresis (NE), 64.7% daytime urinary incontinence (DUI), and 57.1% fecal incontinence (FI)). 52.5% of the children and 32.6% of adults reached a clinically relevant DBC score. Incontinence was not associated with behavioral problems. NE and DUI were associated with genotype and epilepsy.