Journal of Inherited Metabolic Disease

, Volume 29, Issue 2–3, pp 304–310 | Cite as

Breast feeding in organic acidaemias



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.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Demirkol M, Hüner G, Kuru N, Dünmez S, Baykal T, Seçkin Y (2001) Feasibility of breast feeding in inborn errors of metabolism: experience in phenylketonuria. Ann Nutr Metab 45(Supplement 1): 497–498.Google Scholar
  2. Dixon M (2000) Breast feeding in metabolic disease: how successful is this? Dietitians' Meeting VIII ICIEM Meeting, Cambridge, Abstract book.Google Scholar
  3. Hüner G, Demirkol M (1996) Breast feeding and phenylketonuria. In: Demirkol M, Shin YS, eds. Diagnosis and Treatment of Inborn Errors of Metabolism: Contributions to an Equal Opportunity for Children in Asia and Europe. Istanbul: Ufuk Press, 101–117.Google Scholar
  4. Hüner G, Baykal T, Demir F, Demirkol M (2005) Breast feeding experience in inborn errors of metabolism other than phenylketonuria. J Inherit Metab Dis 28: 457–465.PubMedCrossRefGoogle Scholar
  5. Leonard J, van Wyk K (2002) Breast feeding in IEOM other than PKU. 34th EMG Meeting Zurich, Publication of Workshop results,6–8.Google Scholar
  6. McCabe L, Ernst AE, Neifert MR, et al (1989) The management of breast feeding among infants with phenylketonuria. J Inherit Metab Dis 12: 467–474.PubMedCrossRefGoogle Scholar
  7. White F (2000) Breast feeding in inherited metabolic disorders. Dietitians' Meeting VIII ICIEM Meeting, Cambridge, Abstractbook.Google Scholar

Copyright information

© SSIEM and Springer 2006

Authors and Affiliations

  • G. Gokcay
    • 1
  • T. Baykal
    • 1
  • Y. Gokdemir
    • 1
  • M. Demirkol
    • 1
  1. 1.Department of Pediatric Nutrition and Metabolism, Istanbul Medical FacultyIstanbul UniversityIstanbulTurkey

Personalised recommendations