Updated, web-based nutrition management guideline for PKU: An evidence and consensus based approach
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- 作者：Rani H. Singha ; b ; rsingh@emory.edu" class="auth_mail" title="E-mail the corresponding author ; Amy C. Cunninghamc ; acunnin@tulane.edu" class="auth_mail" title="E-mail the corresponding author ; Shideh Mofidid ; shideh.mofidi@hotmail.com" class="auth_mail" title="E-mail the corresponding author ; Teresa D. Douglasa ; teresadd@alum.emory.edu" class="auth_mail" title="E-mail the corresponding author ; Dianne M. Fraziere ; Dianne_frazier@med.unc.edu" class="auth_mail" title="E-mail the corresponding author ; Debra Geary Hookf ; debrahook@sbcglobal.net" class="auth_mail" title="E-mail the corresponding author ; Laura Jeffersg ; Jefferl2@ccf.org" class="auth_mail" title="E-mail the corresponding author ; Helen McCuneh ; mccunh@shands.ufl.edu" class="auth_mail" title="E-mail the corresponding author ; Kathryn D. Moseleyi ; kmoseley@usc.edu" class="auth_mail" title="E-mail the corresponding author ; Beth Ogataj ; bogata@uw.edu" class="auth_mail" title="E-mail the corresponding author ; Surekha Pendyale ; surekha_pendyal@med.unc.edu" class="auth_mail" title="E-mail the corresponding author ; Jill Skrabalk ; Jskrabal@unmc.edu" class="auth_mail" title="E-mail the corresponding author ; Patricia L. Splettl ; splet004@umn.edu" class="auth_mail" title="E-mail the corresponding author ; Adrya Stembridgea ; astembr@emory.edu" class="auth_mail" title="E-mail the corresponding author ; Ann Wesselm ; ann.wessel@childrens.harvard.edu" class="auth_mail" title="E-mail the corresponding author ; Frances Rohrm ; frances.rohr@childrens.harvard.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：ACMG ; American College of Medical Genetics ; AGREE II ; Appraisal of Guidelines for Research and Evaluation ; BH4 ; tetrahydrobiopterin ; BMD ; bone mineral disease ; DRI ; dietary reference intake ; DXA ; dual x-ray absorptiometry ; EF ; executive function ; FFM ; fat-free mass ; GMDI ; Genetic Metabolic Dietitians International ; IMD ; inherited metabolic disorder ; LNAA ; large neutral amino acid ; MeSH ; medical subject heading ; MPKU ; maternal PKU syndrome ; MRI ; magnetic resonance imaging ; MSUD ; Maple Syrup Urine
- 刊名：Molecular Genetics and Metabolism
- 出版年：2016
- 出版时间：June 2016
- 年：2016
- 卷：118
- 期：2
- 页码：72-83
- 全文大小：478 K
文摘
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Web-based, open access nutrition management guidelines for Phenylketonuria
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Addresses nutrient intake, updated protein requirements, and blood PHE levels
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Reviews nutrition intervention, monitoring, adjunct therapy, and pregnancy