Ross Benjamin, Kumar Manish, Srinivasan Hema, Ekbote Alka V
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.
Indian Pediatr. 2016 Aug 8;53(8):738-40. doi: 10.1007/s13312-016-0922-0.
Special diet with restricted branched-chain-amino-acids used for treating maple syrup urine disease can lead to specific amino acid deficiencies.
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.
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.
用于治疗枫糖尿症的限制支链氨基酸特殊饮食可导致特定氨基酸缺乏。
我们报告了一名新生儿，在使用特殊配方奶粉时因异亮氨酸缺乏而出现皮肤病变。
干预措施/结果：通过补充挤出来的母乳进行喂养。这使生化指标和临床症状得到改善，皮肤病变消退。
母乳是枫糖尿症特殊配方奶粉的重要且必要的辅助品，可预防新生儿期特定氨基酸缺乏。
