Acrodermatitis enteropathica-like syndrome secondary to isoleucine deficiency during treatment of maple syrup urine disease
| Autor: | Gerald T. Berry, George P. Giacoia |
|---|---|
| Rok vydání: | 1993 |
| Předmět: |
Male
medicine.medical_specialty Iatrogenic Disease Maple Syrup Urine Disease Internal medicine medicine Iatrogenic disease Humans Isoleucine chemistry.chemical_classification business.industry Maple syrup urine disease Acrodermatitis Acrodermatitis enteropathica Infant Newborn Syndrome medicine.disease Amino acid Endocrinology chemistry Pediatrics Perinatology and Child Health Plasma concentration Infant Food Leucine business |
| Zdroj: | American journal of diseases of children (1960). 147(9) |
| ISSN: | 0002-922X |
| Popis: | • We describe a patient with maple syrup urine disease in whom an acrodermatitis enteropathica–like syndrome developed while he was receiving a branched-chain amino acid–free formula. latrogenically induced isoleucine deficiency developed and resulted in a decreased protein accretion and persistent increase in the plasma concentrations of leucine. A rapid clinical response to isoleucine supplementation was noted. This observation underscores the risks of using amino acid–free formulas without adequate supplementation of deficient amino acids. (AJDC. 1993;147:954-956) |
| Databáze: | OpenAIRE |
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