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Breast feeding in IMD

  • SSIEM SYMPOSIUM 2005
  • Published:
Journal of Inherited Metabolic Disease

Summary

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.

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Abbreviations

ASA:

argininosuccinic acid lyase deficiency

CPS:

carbamoyl phosphate synthetase deficiency

HCU:

homocystinuria

IMD:

inherited metabolic disorder

IVA:

isovaleric acidaemia

MMA:

methylmalonic acidaemia

MSUD:

maple syrup urine disease

OTC:

ornithine transcarbamylase (ornithine carbamoyltransferase)

PKU:

phenylketonuria

PPA:

propionic acidaemia

UCD:

urea cycle disorder

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Author information

Authors and Affiliations

  1. Birmingham Children's Hospital, Birmingham, UK

    A. MacDonald, S. Evans, A. Daly, C. Hendriksz & A. Chakrapani A

  2. Pédiatrie neurométabolique, Hôpital Necker Enfants-Malades, Paris, France

    E. Depondt & J.-M. Saudubray

  3. Department of Dietetics, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK

    A. MacDonald

Authors
  1. A. MacDonald
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  2. E. Depondt
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  3. S. Evans
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  4. A. Daly
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  5. C. Hendriksz
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  6. A. Chakrapani A
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  7. J.-M. Saudubray
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Corresponding author

Correspondence to A. MacDonald.

Additional information

Communicating editor: Jean-Marie Saudubray

Competing interests: None declared

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MacDonald, A., Depondt, E., Evans, S. et al. Breast feeding in IMD. J Inherit Metab Dis 29, 299–303 (2006). https://doi.org/10.1007/s10545-006-0332-x

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  • DOI: https://doi.org/10.1007/s10545-006-0332-x

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