title: Breast milk feeding in infants with inherited metabolic disorders other than phenylketonuria - a 10-year single-center experience.
abstract: BACKGROUND
  Published data on breast milk feeding in infants suffering from inherited metabolic disorders (IMDs) other than phenylketonuria (PKU) are limited and described outcome is variable.
  
  
  OBJECTIVE
  We aimed to evaluate retrospectively whether breastfeeding and/or breast milk feeding are feasible in infants with IMDs including organic acidemias, fatty acid oxidation disorders, urea cycle disorders, aminoacidopathies or disorders of galactose metabolism.
  
  
  METHODS
  Data on breastfeeding and breast milk feeding as well as monitoring and neurological outcome were collected retrospectively from our database of patients with the mentioned IMD, who were followed in our metabolic center within the last 10 years.
  
  
  RESULTS
  Twenty patients were included in the study, who were either breast fed on demand or received expressed breast milk. All the infants were evaluated clinically and biochemically at 2-4-week intervals, with weight gain as the leading parameter to determine metabolic control. Good metabolic control and adequate neurological development were achieved in all patients but one, who experienced the only metabolic crisis observed within the study period.
  
  
  CONCLUSIONS
  Breast milk feeding with close clinical and biochemical monitoring is feasible in most IMD and should be considered as it offers nutritional and immunological benefits.
authors: Department of Pediatrics, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna.
authors: Karin
authors: K
authors: Pichler
authors: University Hospital Innsbruck, Department of Pediatrics I, Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstrasse. 35, A-6020 Innsbruck.
authors: Miriam
authors: M
authors: Michel
authors: University Hospital Innsbruck, Department of Pediatrics I, Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstrasse. 35, A-6020 Innsbruck.
authors: Manuela
authors: M
authors: Zlamy
authors: University Hospital Innsbruck, Department of Pediatrics I, Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstrasse. 35, A-6020 Innsbruck.
authors: Sabine
authors: S
authors: Scholl-Buergi
authors: University Hospital Innsbruck, Department of Pediatrics II, Neonatology, Medical University of Innsbruck, Anichstrasse. 35, A-6020 Innsbruck.
authors: Elisabeth
authors: E
authors: Ralser
authors: University Hospital Innsbruck, Department of Pediatrics I, Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstrasse. 35, A-6020 Innsbruck.
authors: Monika
authors: M
authors: Jörg-Streller
authors: University Hospital Innsbruck, Department of Pediatrics I, Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstrasse. 35, A-6020 Innsbruck.
authors: Daniela
authors: D
authors: Karall
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000178:diet therapy / Q000378:metabolism; D001942:Breast Feeding; D002239:Carbohydrate Metabolism, Inborn Errors / Q000178:diet therapy / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008052:Lipid Metabolism, Inborn Errors / Q000178:diet therapy / Q000378:metabolism; D008297:Male; D008661:Metabolism, Inborn Errors / Q000178:diet therapy* / Q000378:metabolism; D008895:Milk, Human; D012189:Retrospective Studies; D056806:Urea Cycle Disorders, Inborn / Q000178:diet therapy / Q000378:metabolism; D015430:Weight Gain
keywords: Breastfeeding; breast milk feeding; inherited metabolic disorders (IMDs); metabolic control