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- Title
Isoleucine deficiency in a neonate treated for maple syrup urine disease masquerading as acrodermatitis enteropathica.
- Authors
Ross, Benjamin; Kumar, Manish; Srinivasan, Hema; Ekbote, Alka
- Abstract
Background: Special diet with restricted branched-chain-amino-acids used for treating maple syrup urine disease can lead to specific amino acid deficiencies. Case characteristics: We report a neonate who developed skin lesions due to isoleucine deficiency while using specialised formula. Intervention/Outcome: Feeds were supplemented with expressed breast milk. This caused biochemical and clinical improvement with resolution of skin lesions. Message: Breast milk is a valuable and necessary adjunct to specialized formula in maple syrup urine disease to prevent specific amino acid deficiency in the neonatal period.
- Subjects
MAPLE syrup urine disease; ACRODERMATITIS enteropathica; ISOLEUCINE; NEWBORN infants; BRANCHED chain amino acids; THERAPEUTICS
- Publication
Indian Pediatrics, 2016, Vol 53, Issue 8, p738
- ISSN
0019-6061
- Publication type
Article
- DOI
10.1007/s13312-016-0922-0