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Controlled outcome of Hirschsprung's disease beyond adolescence: a single center experience
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Research group (Dept. of women´s and children´s health), Pediatric Surgery.
2019 (English)In: Pediatric surgery international (Print), ISSN 0179-0358, E-ISSN 1437-9813, Vol. 35, no 2, p. 181-185Article in journal (Refereed) Published
Abstract [en]

PurposeThe aim of this study was to assess the function and quality of life of Hirschsprung's Disease (HD) beyond adolescence and relate it to matched controls.MethodsAll 203 patients diagnosed with HD at our department from 1961 to 1995 were identified. 21 had died, 43 had unclear diagnosis and 16 could not be traced. The remaining 123 patients were sent bowel function and SF-36 quality of life questionnaires. 69 patients (mean age 37.8, range 22-58, 13 female) responded and were matched with 138 age and sex-matched controls.ResultsFunction: HD-patients had significantly higher number of bowel movements per week, higher incidence of soiling, urgency, permanent stomas, use of laxatives, enemas and loperamide. HD-patients also scored significantly lower in their satisfaction with their bowel function. There was, however, no significant difference in Miller Incontinence score.QOL: HD-patients reported a significantly higher incidence of negative impact by their bowel function on daily life, social interaction and ability to go on vacation. There were no significant differences in SF-36-scores.ConclusionsBowel function has a lifelong negative impact on the lives of patients with HD. This strongly indicates a need for structured follow-up beyond adolescence.

Place, publisher, year, edition, pages
Springer, 2019. Vol. 35, no 2, p. 181-185
Keywords [en]
Hirschsprung's disease, Aganglionosis, Long-term, Bowel function, Quality of life
National Category
Gastroenterology and Hepatology
Identifiers
URN: urn:nbn:se:uu:diva-376730DOI: 10.1007/s00383-018-4391-5ISI: 000456316100002PubMedID: 30460379OAI: oai:DiVA.org:uu-376730DiVA, id: diva2:1287181
Funder
Fredrik och Ingrid Thurings StiftelseAvailable from: 2019-02-08 Created: 2019-02-08 Last updated: 2019-02-08Bibliographically approved

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Gustafson, Elisabet K.Danielson, Johan

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