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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
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<a href="http://doi.org/10.1016/j.ridd.2017.01.006" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ridd.2017.01.006</a>
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Title
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Incontinence and psychological symptoms in individuals with Mowat-Wilson Syndrome
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Research in Developmental Disabilities
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2017
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children; Rehabilitation; Enuresis; Education & Educational Research; phenotype; features; urinary-incontinence; angelman-syndrome; Fecal incontinence; hirschsprungs-disease; Mowat-Wilson Syndrome; Psychopathology; Urinary incontinence; constipation; behavioral problems; bowel incontinence; trajectory; characteristics; fecal incontinence
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Niemczyk J; Einfeld S; Mowat D; Equit M; Wagner C; Curfs L; von Gontard A
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Background: Mowat-Wilson Syndrome (MWS) is caused by deletion/mutation of the ZEB2 gene on chromosome 2q22. MWS is characterized by a distinctive facial appearance, severe intellectual disability and other anomalies, e.g. seizures and/or Hirschsprung disease (HSCR). Most individuals have a sociable demeanor, but one third show psychological problems. Aims: The aim was to investigate incontinence and psychological problems in MWS. Methods and procedures: 26 children (4-12 years), 13 teens (13-17 years) and 8 adults (>18 years) were recruited through a MWS support group. The Parental Questionnaire: Enuresis/Urinary Incontinence, as well as the Developmental Behaviour Checklist (DBC) were completed by parents or care-givers. Outcomes and results: 97.7% of persons with MWS had incontinence (nocturnal enuresis 74.4%; daytime urinary incontinence 76.2%; fecal incontinence 81.4%). Incontinence remained high over age groups (children 95.8%, teens 100%, adults 100%). 46.2% of children, 25% of teens and 37.5% of adults exceeded the clinical cut-off on the DBC. The ability to use the toilet for micturition improved with age. Conclusions and implications: MWS incontinence rates are very high. All had physical disabilities including anomalies of the genitourinary and gastrointestinal tract. Due to the high prevalence rates, a screening for incontinence and psychological problems in MWS is recommended. (C) 2017 Elsevier Ltd. All rights reserved.
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<a href="http://doi.org/10.1016/j.ridd.2017.01.006" target="_blank" rel="noreferrer noopener">10.1016/j.ridd.2017.01.006</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2017
angelman-syndrome
behavioral problems
bowel incontinence
characteristics
Children
Constipation
Curfs L
Education & Educational Research
Einfeld S
enuresis
Equit M
features
fecal incontinence
hirschsprungs-disease
Mowat D
Mowat-Wilson syndrome
Niemczyk J
Phenotype
psychopathology
Rehabilitation
Research in Developmental Disabilities
Trajectory
urinary incontinence
urinary-incontinence
von Gontard A
Wagner C