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J Pediatr Gastroenterol Nutr. 2014 Feb;58(2):237-44. doi: 10.1097/MPG.0000000000000200.

Gastrointestinal dysmotility in Rett syndrome.

Author information

1
*Department of Developmental Medicine, Royal Children's Hospital, Murdoch Children's Research Institute, Melbourne †Department of Gastroenterology, Princess Margaret Hospital for Children ‡Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, Perth, Australia §Department of Pediatrics ||Civitan International Research Centre, University of Alabama, Birmingham, AL ¶Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Edmond and Lily Safra Children's Hospital, Tel-Hashomer, Israel #Western Sydney Genetics Program, Children's Hospital at Westmead, Discipline of Paediatrics and Genetic Medicine, University of Sydney, Sydney, Australia.

Abstract

OBJECTIVES:

Through evidence review and the consensus of an expert panel, we developed recommendations for the clinical management of gastroesophageal reflux disease, constipation, and abdominal bloating in Rett syndrome.

METHODS:

Based on review of the literature and family concerns expressed on RettNet, initial draft recommendations were created. Wherein the literature was lacking, 25 open-ended questions were included. Input from an international, multidisciplinary panel of clinicians was sought using a 2-stage modified Delphi process to reach consensus agreement. Items related to the clinical assessment and management of gastroesophageal reflux disease, constipation, and abdominal bloating.

RESULTS:

Consensus was achieved on 78 of 85 statements. A comprehensive approach to the assessment of gastroesophageal reflux and reflux disease, constipation, and abdominal bloating was recommended, taking into account impairment of communication skills in Rett syndrome. A stepwise approach to the management was identified with initial use of conservative strategies, escalating to pharmacological measures and surgery, if necessary.

CONCLUSIONS:

Gastrointestinal dysmotility occurs commonly in Rett syndrome. These evidence- and consensus-based recommendations have the potential to improve care of dysmotility issues in a rare condition and stimulate research to improve the present limited evidence base.

PMID:
24121144
DOI:
10.1097/MPG.0000000000000200
[Indexed for MEDLINE]
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