Nutrition therapy of organic acidaemias with amino acid-based formulas: emphasis on methylmalonic and propionic acidaemia.
Failure to thrive has been described in patients with organic acidaemias due to a variety of causes, both organic and inorganic. Failure to thrive in patients with methylmalonic acidaemia (MMA) and propionic acidaemia (PA) may be related to inadequate protein and energy intake rather than pathology of disease. Inadequate protein intake can also result in decreased resting energy expenditure, clinical signs and symptoms of amino acid deficiency, increased risk of infection, and developmental delay. Amino acid-based formulas (also referred to as 'medical foods') provide a key source of nitrogen, energy, vitamins and minerals which, when prescribed appropriately, can promote anabolism and growth. Although protein requirements in patients with organic acidaemias have not been elucidated, providing an adequate balance of protein, energy and other nutrients will help promote growth.
Yannicelli
Steven
S
SHS North America, Valencia, California, USA.
D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood / Q000188:drug therapy / Q000378:metabolism / Q000628:therapy*; D000596:Amino Acids / Q000378:metabolism / Q000627:therapeutic use*; D002626:Chemistry, Pharmaceutical; D019587:Dietary Supplements; D004734:Energy Metabolism; D006801:Humans; D008764:Methylmalonic Acid / Q000097:blood* / Q000378:metabolism; D018529:Nutritional Support; D011422:Propionates / Q000097:blood* / Q000378:metabolism; D011506:Proteins / Q000378:metabolism; D016896:Treatment Outcome
D000596:Amino Acids; D011422:Propionates; D011506:Proteins; D008764:Methylmalonic Acid; C029658:propionic acid