caa a22 4500 467933030 CHVBK 20180406152948.0 cr unu---uuuuu 170328e20060401xx s 000 0 eng 10.1007/s10545-006-0267-2 doi (NATIONALLICENCE)springer-10.1007/s10545-006-0267-2 Yannicelli Steven SHS North America, Valencia, California, USA aut Nutrition therapy of organic acidaemias with amino acid-based formulas: Emphasis on methylmalonic and propionic acidaemia [Elektronische Daten] [Steven Yannicelli] Summary: 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. SSIEM and Springer, 2006 Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/2-3(2006-04-01), 281-287 0141-8955 29:2-3<281 2006 29 10545 https://doi.org/10.1007/s10545-006-0267-2 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0267-2 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Yannicelli Steven SHS North America, Valencia, California, USA aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/2-3(2006-04-01), 281-287 0141-8955 29:2-3<281 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer