Assessment and Management of Nutrition and Growth in Rett Syndrome
Assessment and Management of Nutrition and Growth in Rett Syndrome
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DOI：
10.1097/mpg.0b013e31829e0b65
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发表时间：
2013-10-01
影响因子：
2.9
通讯作者：
Downs, Jenny
中科院分区：
文献类型：
--
作者：
Leonard, Helen;Ravikumar, Madhur;Downs, Jenny
目的：我们通过证据审查和临床医生专家小组的共识，制定了Rett综合征生长不良和体重增加的临床管理建议。方法：根据文献综述和34个文献缺失的开放式问题，制定初步建议草案。声明和问题以在线格式提供给国际多学科临床医生小组，并通过电子邮件提供微软word格式的草案版本。采用两阶段改进的德尔菲过程寻求意见以达成共识。项目包括临床生长评估、人体测量、喂养困难和增加能量摄入、减少喂养困难的管理，以及考虑胃造口术。结果：112条表述中有101条表述一致。建议对Rett综合征生长不良的管理采取综合办法，考虑到喂养困难和营养需求等因素。在临床实践中，大约25百分位的体重指数可以被认为是一个合理的目标。胃造口术适用于生长极差，如果有误吸的危险，如果喂养时间延长。结论：这些基于证据和共识的建议有可能改善罕见疾病患者的营养和生长护理，并刺激研究以改善目前有限的证据基础。
Objectives: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.Methods: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.Results: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.Conclusions: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.