PMID: 2502611Aug 1, 1989Paper

Parenteral nutrition in propionic and methylmalonic acidemia

The Journal of Pediatrics
S G KahlerC R Roe

Abstract

Although propionic acidemia and methylmalonic acidemia, two disorders of branched-chain amino acid metabolism often complicated by chronic anorexia and vomiting, are not usually treated with parenteral nutrition for fear of amino acid overload and exacerbation of biochemical derangements, we gave long-term parenteral nutrition to two critically ill patients with these disorders. Health and growth were restored, and there was minimal production of abnormal metabolites. The dramatic clinical and biochemical improvement of these patients bolsters the concept that most of the toxic metabolites produced in these diseases are not related to the administered load of nutrient precursors, but rather to endogenous turnover of amino acids, particularly during a chronic catabolic state. Suppression of catabolism can produce striking biochemical and clinical improvement. With appropriate monitoring, parenteral nutrition can be used safely in the management of patients with these disorders.

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Citations

Jan 1, 1994·Journal of Inherited Metabolic Disease·W SperlK Groke
Oct 22, 2003·Molecular Genetics and Metabolism·Steven YannicelliGeorgianne L Arnold
Jul 14, 2012·PloS One·Heidi L PetersNicole E Buck
Mar 1, 1994·The Journal of Pediatrics·L De RaeveE Gerlo
Jun 19, 2002·Seminars in Neonatology : SN·Hélène Ogier de Baulny
Oct 12, 2011·Molecular Genetics and Metabolism·Loren PenaMarshall L Summar
Oct 18, 2011·Molecular Genetics and Metabolism·Kimberly A ChapmanAnupam Chakrapani
Oct 4, 2011·Molecular Genetics and Metabolism·V Reid SuttonAnupam Chakarapani
Jul 1, 1991·Journal of Child Neurology·P T Ozand, G G Gascon
May 6, 2004·Journal of Pediatric Hematology/oncology·Tansu SipahiBetul Tavil
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Sep 4, 1998·Clinical Genetics·M Al EssaP T Ozand
Oct 12, 2010·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Franco Lilliu
Oct 1, 1991·Biochemical Medicine and Metabolic Biology·T MatsuishiK Chrislip

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