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Children with Hirschsprung's Disease and Syndromes with Cognitive Dysfunction : Manifestations, Treatment, and Outcomes

Hedbys, Josefine ; Hasserius, Johan ; Granéli, Christina LU ; Arnbjörnsson, Einar LU ; Hagelsteen, Kristine LU orcid and Stenström, Pernilla LU orcid (2019) In The Surgery Journal 5(3). p.103-109
Abstract

Introduction  To assess differences in initial symptoms, treatments, and bowel function between children with Hirschsprung's disease (HD) with or without a cognitive dysfunction (CD). Materials and Methods  The study included children with HD who underwent transanal endorectal pull-through. A retrospective chart review was performed to collect data on patient characteristics, diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without a colostomy were compiled during a cross-sectional, patient-reported follow-up. Results  Fifty-three children with HD were included; of these, 12 (23%) had CD. The median birth weight was lower, frequency of vomiting as the presenting symptom was lower, and time until the first... (More)

Introduction  To assess differences in initial symptoms, treatments, and bowel function between children with Hirschsprung's disease (HD) with or without a cognitive dysfunction (CD). Materials and Methods  The study included children with HD who underwent transanal endorectal pull-through. A retrospective chart review was performed to collect data on patient characteristics, diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without a colostomy were compiled during a cross-sectional, patient-reported follow-up. Results  Fifty-three children with HD were included; of these, 12 (23%) had CD. The median birth weight was lower, frequency of vomiting as the presenting symptom was lower, and time until the first contact with a pediatric surgeon was higher in children with CD than in those without (3,295 vs. 3,623 g, p  = 0.013; 28 vs. 66%, p  = 0.02; and 4 days vs. 1 day, p  = 0.048, respectively). At follow-up, 5 (15%) of 33 children aged over 4 years had CD. More children without CD had some ability to hold back defecation and sense the urge to defecate than those with CD ( p  = 0.002 and p  = 0.001, respectively). Conclusion  HD children who have CD present with different initial symptoms, have a delay in the first consultation with a pediatric surgeon, and experience poorer bowel function outcomes than HD children without CD. Therefore, HD children with CD should receive special attention in both clinical practice and research.

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author
; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
The Surgery Journal
volume
5
issue
3
pages
103 - 109
publisher
Georg Thieme Verlag
external identifiers
  • pmid:31508491
ISSN
2378-5128
DOI
10.1055/s-0039-1696730
language
English
LU publication?
yes
id
94615fb2-e9c4-42ad-a0a0-e805bc97ab04
date added to LUP
2019-09-16 09:12:58
date last changed
2019-09-19 02:18:22
@article{94615fb2-e9c4-42ad-a0a0-e805bc97ab04,
  abstract     = {{<p>Introduction  To assess differences in initial symptoms, treatments, and bowel function between children with Hirschsprung's disease (HD) with or without a cognitive dysfunction (CD). Materials and Methods  The study included children with HD who underwent transanal endorectal pull-through. A retrospective chart review was performed to collect data on patient characteristics, diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without a colostomy were compiled during a cross-sectional, patient-reported follow-up. Results  Fifty-three children with HD were included; of these, 12 (23%) had CD. The median birth weight was lower, frequency of vomiting as the presenting symptom was lower, and time until the first contact with a pediatric surgeon was higher in children with CD than in those without (3,295 vs. 3,623 g, p  = 0.013; 28 vs. 66%, p  = 0.02; and 4 days vs. 1 day, p  = 0.048, respectively). At follow-up, 5 (15%) of 33 children aged over 4 years had CD. More children without CD had some ability to hold back defecation and sense the urge to defecate than those with CD ( p  = 0.002 and p  = 0.001, respectively). Conclusion  HD children who have CD present with different initial symptoms, have a delay in the first consultation with a pediatric surgeon, and experience poorer bowel function outcomes than HD children without CD. Therefore, HD children with CD should receive special attention in both clinical practice and research.</p>}},
  author       = {{Hedbys, Josefine and Hasserius, Johan and Granéli, Christina and Arnbjörnsson, Einar and Hagelsteen, Kristine and Stenström, Pernilla}},
  issn         = {{2378-5128}},
  language     = {{eng}},
  number       = {{3}},
  pages        = {{103--109}},
  publisher    = {{Georg Thieme Verlag}},
  series       = {{The Surgery Journal}},
  title        = {{Children with Hirschsprung's Disease and Syndromes with Cognitive Dysfunction : Manifestations, Treatment, and Outcomes}},
  url          = {{http://dx.doi.org/10.1055/s-0039-1696730}},
  doi          = {{10.1055/s-0039-1696730}},
  volume       = {{5}},
  year         = {{2019}},
}