title: Isoleucine Deficiency in a Neonate Treated for Maple Syrup Urine Disease Masquerading as Acrodermatitis Enteropathica.
abstract: BACKGROUND
  Special diet with restricted branched-chain-amino-acids used for treating maple syrup urine disease can lead to specific amino acid deficiencies.
  
  
  METHODS
  We report a neonate who developed skin lesions due to isoleucine deficiency while using specialised formula.
  
  
  RESULTS
  Feeds were supplemented with expressed breast milk. This caused biochemical and clinical improvement with resolution of skin lesions.
  
  
  CONCLUSIONS
  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.
authors: Departments of Neonatology, *Pediatrics and #Clinical Genetics Unit,Christian Medical College, Vellore, Tamilnadu, India. Correspondence to: Dr Benjamin Ross, Department of Neonatology, Christian Medical College, Vellore 632 004, Tamilnadu, India. benjaminross@cmcvellore.ac.in.
authors: Benjamin
authors: B
authors: Ross
authors: Manish
authors: M
authors: Kumar
authors: Hema
authors: H
authors: Srinivasan
authors: Alka V
authors: AV
authors: Ekbote
mesh_terms: D000169:Acrodermatitis; D001942:Breast Feeding; D003937:Diagnosis, Differential; D006801:Humans; D041943:Infant Formula; D007231:Infant, Newborn; D007532:Isoleucine / Q000008:administration & dosage / Q000172:deficiency; D008375:Maple Syrup Urine Disease / Q000178:diet therapy / Q000235:genetics / Q000503:physiopathology; D015032:Zinc / Q000172:deficiency
chemical_list: D007532:Isoleucine; D015032:Zinc