Detailed assessment of incontinence in boys with fragile-X-syndrome in a home setting

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• 作者：Justine Niemczyk ; Alexander von Gontard ; Monika Equit…
• 关键词：Fragile ; X ; syndrome ; Urinary incontinence ; Nocturnal enuresis ; Faecal incontinence ; Constipation ; Psychological disorders
• 刊名：European Journal of Pediatrics
• 出版年：2016
• 出版时间：October 2016
• 年：2016
• 卷：175
• 期：10
• 页码：1325-1334
• 全文大小：349 KB
• 刊物主题：Pediatrics;
• 出版者：Springer Berlin Heidelberg
• ISSN：1432-1076
• 卷排序：175

文摘

Fragile-X-syndrome (FXS) is caused by a mutation on the X chromosome (Xq27.3). Males with a full mutation have typical dysmorphic signs, moderate intellectual disability and psychological disorders. Twenty-five to fifty percent are affected by incontinence. The aim of the study was to assess subtypes of incontinence and psychological problems in children with FXS in their home environments. Twenty-two boys with FXS (mean age 11.0 years) and 22 healthy controls (mean age 11.1 years) were examined with sonography, uroflowmetry, 48-h bladder diary, physical examination, IQ test, parental psychiatric interview and questionnaires regarding incontinence and psychological symptoms in a home setting. Boys with FXS had higher rates of incontinence than controls (59.1 vs. 4.8 %). The most common subtypes in FXS boys were primary non-monosymptomatic nocturnal enuresis, urge incontinence and nonretentive faecal incontinence. 90.9 % boys with FXS had a psychological comorbidity. Incontinence and behavioural symptoms were not associated.