caa a22 4500 467933065 CHVBK 20180406152949.0 cr unu---uuuuu 170328e20060401xx s 000 0 eng 10.1007/s10545-005-0255-y doi (NATIONALLICENCE)springer-10.1007/s10545-005-0255-y Breast feeding in organic acidaemias [Elektronische Daten] [G. Gokcay, T. Baykal, Y. Gokdemir, M. Demirkol] Summary: Breast feeding has been recommended for the dietary treatment of infants with organic acidaemias, but studies documenting clinical experience are still very few. Nine infants, diagnosed with methylmalonic acidaemia (n = 4), propionic acidaemia (n = 1), isovaleric acidaemia (n = 2) and glutaric acidaemia type I (n = 2) were breast fed after diagnosis. The age of the patients was 28.9± 13.4 months (mean ± SD) (range 10-57 months). Eight patients were diagnosed with clinical symptoms and one because of an affected sibling. After the control of acute metabolic problems, an initial period with a measured volume of expressed breast milk was continued with on-demand breast feeding with the addition of a special essential amino acid mixture and energy supplements. Breast feeding was well tolerated in seven infants with good growth, metabolic control and neurological outcome. The duration of breast feeding was 12.3± 7.4 months (mean ± SD) (range 4-24 months) in these patients. Breast feeding was terminated in the patient with propionic acidaemia because of two acute metabolic episodes requiring hospitalization, and could not be continued in one of the patients with isovaleric acidaemia owing to shortage of breast milk. A decrease in the frequency of infections, acute metabolic episodes and hospital admissions was observed in breast-fed infants. Breast feeding of infants with organic acidaemias is feasible with close monitoring of clinical parameters such as growth, development and biochemistry, including amino acids, organic acids and ammonia. SSIEM and Springer, 2006 Gokcay G. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut Baykal T. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut Gokdemir Y. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut Demirkol M. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/2-3(2006-04-01), 304-310 0141-8955 29:2-3<304 2006 29 10545 https://doi.org/10.1007/s10545-005-0255-y text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-005-0255-y text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Gokcay G. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut NATIONALLICENCE 700 1- Baykal T. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut NATIONALLICENCE 700 1- Gokdemir Y. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut NATIONALLICENCE 700 1- Demirkol M. Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Capa, 34093, Istanbul, Turkey aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/2-3(2006-04-01), 304-310 0141-8955 29:2-3<304 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer