Breast feeding in IMD.
Breast feeding has proven benefits for many infants with inherited metabolic disorders (IMDs) but, with the exception of phenylketonuria, there are few reports in other conditions. A questionnaire, completed by dietitians and clinicians from 27 IMD centres from 15 countries (caring for a total of over 8000 patients with IMDs on diet) identified breast feeding experience in IMD. Successful, demand breast feeding (in combination with an infant amino acid formula free of precursor amino acids) was reported in 17 infants with MSUD, 14 with tyrosinaemia type I, and 5 with homocystinuria. Eighty-nine per cent were still breast fed at 16 weeks. Fewer infants with organic acidaemias were demand breast fed (7 with propionic acidaemia; 6 with methylmalonic acidaemia and 13 with isovaleric acidaemia) (usually preceded by complementary feeds of a protein-free infant formula or infant amino acid formula free of precursor amino acids). Only 12 infants with urea cycle disorders were given demand breast feeds, but this was unsuccessful beyond 8 days in CPS deficiency. Further work is needed in developing guidelines for feeding and for clinical and biochemical monitoring for breast-fed infants with IMDs.
MacDonald
A
A
Birmingham Children's Hospital, Birmingham, UK. anita.macdonald@bch.nhs.uk
Depondt
E
E
Evans
S
S
Daly
A
A
Hendriksz
C
C
Chakrapani A
A
A
Saudubray
J-M
JM
D000592:Amino Acid Metabolism, Inborn Errors / Q000178:diet therapy / Q000378:metabolism; D000596:Amino Acids / Q000627:therapeutic use; D001903:Bottle Feeding; D001942:Breast Feeding; D005247:Feeding Behavior; D005260:Female; D006801:Humans; D007223:Infant; D041943:Infant Formula; D007227:Infant Nutritional Physiological Phenomena; D008661:Metabolism, Inborn Errors / Q000178:diet therapy* / Q000378:metabolism; D008895:Milk, Human / Q000737:chemistry; D017410:Practice Guidelines as Topic; D011506:Proteins / Q000378:metabolism; D011795:Surveys and Questionnaires; D013997:Time Factors
D000596:Amino Acids; D011506:Proteins