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Diet For Inborn Error Of Metabolism

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ManuscriptsPMC3444638 Mol Genet Metab. Author manuscript; available in PMC 2013 Sep inborn error of metabolism examples 1.Published in final edited form as:Mol Genet Metab. 2012 Sep; 107(1-2): 3–9. Published online 2012 Jul 16. inborn error of metabolism ppt doi:  10.1016/j.ymgme.2012.07.005PMCID: PMC3444638NIHMSID: NIHMS394682Nutritional Treatment for Inborn Errors of Metabolism: Indications, Regulations, and Availability of Medical Foods and Dietary Supplements Using Phenylketonuria as an ExampleKathryn M. Camp,a,*

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Michele A. Lloyd-Puryear,b and Kathleen L. HuntingtoncaOffice of Dietary Supplements, National Institutes of Health, Bethesda, MD 20892, USAbEunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USAcOregon Health and Sciences University, Portland, OR 97239, USAKathryn M. Camp: vog.hin.do@mkpmac; Michele A. Lloyd-Puryear: vog.hin.liam@amraeyrupdyoll; Kathleen L. Huntington: ude.usho@tgnitnuh

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*Corresponding author at: 6100 Executive Blvd, Rockville, MD 20892, USA. Phone: +1 301 435 3608. FAX: +1 301 480 1845Author information ► Copyright and License information ►Copyright notice and DisclaimerPublisher's DisclaimerThe publisher's final edited version of this article is available at Mol Genet MetabSee other articles in PMC that cite the published article.AbstractMedical foods and dietary supplements are used to treat rare inborn errors of metabolism (IEM) identified through state-based universal newborn screening. These products are regulated under Food and Drug Administration (FDA) food and dietary supplement statutes. The lack of harmony in terminology used to refer to medical foods and dietary supplements and the misuse of words that imply that FDA regulates these products as drugs have led to confusion. These products are expensive and, although they are used for medical treatment of IEM, third-party payer coverage of these products is inconsistent across the United States. Clinicians and families report termination of coverage in late adolescence, failure to cover treatment during pregnancy, covera

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Close ScienceDirectSign inSign in using your ScienceDirect credentialsUsernamePasswordRemember meForgotten username or password?Sign in via your an inborn error of metabolism is caused by institutionOpenAthens loginOther institution loginHelpJournalsBooksRegisterJournalsBooksRegisterSign inHelpcloseSign in using your ScienceDirect credentialsUsernamePasswordRemember meForgotten username or password?Sign in via your institutionOpenAthens loginOther institution login Download PDF Opens in a https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3444638/ new window. Article suggestions will be shown in a dialog on return to ScienceDirect. Help Direct export Export file RIS(for EndNote, Reference Manager, ProCite) BibTeX Text RefWorks Direct Export Content Citation Only Citation and Abstract Advanced search JavaScript is disabled on your browser. Please enable http://www.sciencedirect.com/science/article/pii/S1096719215300913 JavaScript to use all the features on this page. JavaScript is disabled on your browser. Please enable JavaScript to use all the features on this page. This page uses JavaScript to progressively load the article content as a user scrolls. Click the View full text link to bypass dynamically loaded article content. View full text Molecular Genetics and MetabolismVolume 117, Issue 3, March 2016, Pages 322–327 Metabolic Diet App Suite for inborn errors of amino acid metabolismGloria Hoa, Keiko Uedaa, Roderick F.A. Houbenc, Jeff Joac, Alette Giezena, Barbara Chenga, Clara D.M. van Karnebeeka, b, , a Division of Biochemical Diseases, BC Children's Hospital, University of British Columbia, Vancouver, Canadab Department of Pediatrics, Centre for Molecular Medicine & Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, Canadac Health2Media, Vancouver, CanadaReceived 6 November 2015, Revised 21 December 2015, Accepted 21 December 2015, Available online 23 December 2015Highlights•The Metaboli

Errors of Metabolism The past four decades of the http://www.solacenutrition.com/products/inborn-errors-of-metabolism/inborn-errors-of-metabolism.html dietary management of Inborn Errors of Metabolism have focused on eliminating offending amino acids and restricting intake of dietary protein. http://link.springer.com/article/10.1007/BF02722635 The traditional dietary approach has been effective but not without limitations. Traditional medical foods, with their characteristic taste and inborn error low protein content, result in many people reducing their adherence or abandoning their diet programs altogether. Mitochondrial Diseases Mitochondrial disease research is one of the fastest growing sectors of genetics, not only in rare childhood diseases but also inborn error of in many common diseases such as heart disease, Parkinson disease and dementia. Solace Nutrition has partnered with the leading Coenzyme Q10 manufacturer to market specific Orphan Drug designated formulas as medical foods for mitochondrial cytopathies. Cyto Q – Medical Food - Liposomal Ubiquinol for the dietary management of Mitochondrial Cytopathies. Cyto-Q MAX - Medical Food - Unflavored, highly concentrated form of Cyto-Q (100mg/1ml). Cytotine - Medical Food - patented, stabilized creatine monohydrate-based Medical Food that improves Adenosine Triphosphate (ATP) production / energy production in the human body. Cytose - is a patented, Medical Food formulated from GRAS approved D-Ribose Read more phone: 888-8-SOLACE or 888-876-5223fax: 401-633-6066info@solacenutrition.com © Solace Nutrition Site maintained and optimized by RobiDigital

feedback return to old SpringerLink The Indian Journal of PediatricsMay 2002, Volume 69, Issue 5, pp 421–426Dietary management of inborn errors of metabolismAuthorsAuthors and affiliationsMadhulika KabraEmail authorSymposium on Hospital-Based NutritionDOI: 10.1007/BF02722635Cite this article as: Kabra, M. Indian J Pediatr (2002) 69: 421. doi:10.1007/BF02722635 8 Citations 287 Views AbstractInborn errors of metabolism are individually rare but are an important cause of mortality and morbidity in infants and children. Dietary therapy is the mainstay of treatment in phenylketonuria, maple syrup urine disease, homocystinuria, galactossemia and glycogen storage disease (Type I/III). Some disorders like urea cycle disorders and organic acidurias require dietary modification in addition to other modalities. Certain basic principles of dietary management should be clearly understood for proper management of these disorders. Commercially available diets are very expensive and modification in routine Indian diet may be tried based on content of different nutrients but the desirable fine control is not achieved.Key wordsInborn errors of metabolismDietary managementIndian modificationsReferences1.Council of Europe report 1973.2.ICMR Collaborating Centres and Central Co-ordinating Unit. Multricentric study on genetic causes of mental retardation in India.Indian J Med Res 1991; 94: 161–169.3.Ambani LM, Patel ZM, Dhareshwar SSet al. Clinical, biochemical and cytogenetic studies in mental retardation.Indian J Med Res 1984; 79: 384–387.PubMed4.Kaur M, Das GP, Verma IC. Inborn errors of aminoacid metabolism in North India.J Inherit Metab Dis 1994; 17: 1–4.CrossRef5.Dietary management of disorders of aminoacid metabolism, organic acduria and urea cycle defects.Diets for Sick Children. 1987. 263–312, Dorothy Em Francis 4th edn. Blackwell Scientific Publications.6.Phenylketonuria in dietary management of disorders of aminoa

 

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