Effects of medical food leucine content in the management of methylmalonic and propionic acidemias (original) (raw)
A critical reappraisal of dietary practices in methylmalonic acidemia raises concerns about the safety of medical foods. Part 1: isolated methylmalonic acidemias
Irini Manoli
Genetics in Medicine, 2015
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Clinical Course and Nutritional Management of Propionic and Methylmalonic Acidemias
Heba Dawoud
Journal of Nutrition and Metabolism
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Methylmalonic and propionic acidemias: clinical management update
Charles P Venditti
Current Opinion in Pediatrics, 2016
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Variable dietary management of methylmalonic acidemia: metabolic and energetic correlations
Charles P Venditti
The American Journal of Clinical Nutrition, 2010
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Dietary practices in methylmalonic acidaemia: a European survey
Carla vasconcelos
Journal of Pediatric Endocrinology and Metabolism, 2019
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Guidelines for the diagnosis and management of methylmalonic acidaemia and propionic acidaemia: First revision
Stephanie Grünewald
Journal of Inherited Metabolic Disease, 2021
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Dietary practices in propionic acidemia: A European survey
Jaime Dalmau
Molecular genetics and metabolism reports, 2017
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Brain Damage by Mild Metabolic Derangements in Methylmalonic Acidemia
Yin-hsiu Chien
Pediatric Neurology, 2008
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Severe anemia in patients with Propionic acidemia is associated with branched-chain amino acid imbalance
Victor Quintero
Orphanet Journal of Rare Diseases, 2021
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Reduction of large neutral amino acid levels in plasma and brain of hyperleucinemic rats
patricia araujo
Neurochemistry International, 2001
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Review Reviewing the Effects of L-Leucine Supplementation in the Regulation of Food Intake, Energy Balance, and
Thais Zampieri
2015
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Nutrition management guideline for propionic acidemia: An evidence- and consensus-based approach
Adrya Stembridge
Molecular Genetics and Metabolism, 2019
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Branched-chain amino acid interactions with reference to amino acid requirements in adult men: valine metabolism at different leucine intakes
Veronique Anne Pelletier
The American Journal of Clinical Nutrition, 1991
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Amino-acid depletion induced by abnormal amino-acid conjugation and protein restriction in isovaleric acidemia
Elardus Erasmus
European Journal of Clinical Nutrition, 2007
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Dietary practices in isovaleric acidemia: A European survey
Jaime Dalmau
Molecular genetics and metabolism reports, 2017
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Neurological outcome of methylmalonic acidaemia
Paola Nicolaides
Archives of Disease in Childhood, 1998
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High Leucine Diets Stimulate Cerebral Branched-Chain Amino Acid Degradation and Modify Serotonin and Ketone Body Concentrations in a Pig Model
Anna Wessels
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Effects of leucine on whole body leucine, valine, and threonine metabolism in humans
Leonard Hoffer
The American journal of physiology
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Large neutral amino acids supplementation in phenylketonuric patients
Fátima Martel
Journal of Inherited Metabolic Disease, 2009
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Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia
Stephanie Grünewald
Orphanet Journal of Rare Diseases, 2014
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Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food
Antonio Velazquez
Molecular Genetics and Metabolism, 2003
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Leucine is not a good choice as an indicator amino acid for determining amino acid requirements in men
Paul Pencharz
The Journal of nutrition, 2006
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Progressive neurologic disability in methylmalonic acidemia despite transplantation of the liver
J. jay Gargus
European Journal of Pediatrics, 2002
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Evaluation of Indicators of Cobalamin Deficiency Defined as Cobalamin-induced Reduction in Increased Serum Methylmalonic Acid
Jörn Schneede
2000
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Glutathione deficiency as a complication of methylmalonic acidemia: Response to high doses of ascorbate
Philippe Chessex
The Journal of Pediatrics, 1996
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Emerging Roles of Branched-Chain Amino Acid Supplementation in Human Diseases
Niaz Mahmood
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Reviewing the Effects of l-Leucine Supplementation in the Regulation of Food Intake, Energy Balance, and Glucose Homeostasis
Thais Zampieri
Nutrients, 2015
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Alterations in protein and amino acid metabolism in rats fed a branched-chain amino acid- or leucine-enriched diet during postprandial and postabsorptive states
Pavel Šiman
Nutrition & Metabolism, 2016
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