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Isoleucine deficiency in a neonate treated for maple syrup urine disease masquerading as acrodermatitis enteropathica

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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.

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Author information

Authors and Affiliations

  1. Departments of Neonatology, Christian Medical College, Vellore, Tamilnadu, India

    Benjamin Ross & Manish Kumar

  2. Departments of Pediatrics, Christian Medical College, Vellore, Tamilnadu, India

    Hema Srinivasan

  3. Clinical Genetics Unit, Christian Medical College, Vellore, Tamilnadu, India

    Alka V. Ekbote

  4. Department of Neonatology, Christian Medical College, Vellore, 632 004, Tamilnadu, India

    Benjamin Ross

Authors
  1. Benjamin Ross
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  2. Manish Kumar
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  3. Hema Srinivasan
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  4. Alka V. Ekbote
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Corresponding author

Correspondence to Benjamin Ross.

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Ross, B., Kumar, M., Srinivasan, H. et al. Isoleucine deficiency in a neonate treated for maple syrup urine disease masquerading as acrodermatitis enteropathica. Indian Pediatr 53, 738–740 (2016). https://doi.org/10.1007/s13312-016-0922-0

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  • DOI: https://doi.org/10.1007/s13312-016-0922-0

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