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J Pediatr Gastroenterol Nutr. 2013 Oct;57(4):451-60. doi: 10.1097/MPG.0b013e31829e0b65.

Assessment and management of nutrition and growth in Rett syndrome.

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*Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia †Department of Gastroenterology, Princess Margaret Hospital for Children, Perth ‡Department of Developmental Medicine, Royal Children's Hospital, Murdoch Children's Research Institute, Melbourne §Western Sydney Genetics Program, Children's Hospital at Westmead, Discipline of Paediatrics and Genetic Medicine, University of Sydney, Sydney, Australia ||Civitan International Research Centre, University of Alabama, Birmingham, AL ¶Department of Otolaryngology, Head Neck Surgery, and Radiology, Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY #Katie's Clinic for Rett Syndrome, Children's Hospital & Research Center, Oakland, CA.



We developed recommendations for the clinical management of poor growth and weight gain in Rett syndrome through evidence review and the consensus of an expert panel of clinicians.


Initial draft recommendations were created based upon literature review and 34 open-ended questions in which the literature was lacking. Statements and questions were made available to an international, multidisciplinary panel of clinicians in an online format and a Microsoft Word-formatted version of the draft via e-mail. Input was sought using a 2-stage modified Delphi process to reach consensus. Items included clinical assessment of growth, anthropometry, feeding difficulties and management to increase energy intake, decrease feeding difficulties, and consideration of gastrostomy.


Agreement was achieved on 101 of 112 statements. A comprehensive approach to the management of poor growth in Rett syndrome is recommended that takes into account factors such as feeding difficulties and nutritional needs. A body mass index of approximately the 25th centile can be considered as a reasonable target in clinical practice. Gastrostomy is indicated for extremely poor growth, if there is risk of aspiration and if feeding times are prolonged.


These evidence- and consensus-based recommendations have the potential to improve care of nutrition and growth in a rare condition and stimulate research to improve the present limited evidence base.

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