牛乳蛋白アレルギー児に発症したアミノ酸調整粉末哺育によるビオチン欠乏症  [in Japanese] BIOTIN DEFICIENCY BY AMINO ACID FORMULA NUTRITION FOR AN INFANT WITH MILK PROTEIN ALLERGY  [in Japanese]

アミノ酸調整粉末単独で哺育されていたため,ビオチン欠乏をきたした4ヵ月女児例を報告した.患児は生後2週に牛乳蛋白アレルギーと診断され,アミノ酸調整粉末治療により症状は改善した.治療開始の約3週間後に発症した皮膚のびらんは,眼周囲,口唇,頸部,臀部に認められ,軟膏などの局所治療で改善しなかった.血清ビオチン値の低下と尿中有機酸排泄の増加を認めたため,ビオチン欠乏によるマルチプルカルボキシラーゼ欠損症と診断した.血清ビオチニダーゼ活性は正常範囲内だった.皮膚症状,血清ビオチン値,有機酸尿はビオチン1日1mgの内服により劇的に正常化した.アミノ酸調整粉末にはほとんどビオチンが含まれていないことから,これのみで哺育されている難治性皮膚炎の症例ではビオチン欠乏を考慮する必要がある.

We reported a 4-month-old girl with biotin deficiency caused by amino acid formula. Two weeks after birth, she was diagnosed as having a milk protein allergy. After switching to amino acid formula from usual formula, her symptoms and laboratory findings became normal. About three weeks after the beginning of amino acid formula, she developed intractable skin erosions around the eyes, mouth, neck, and anogenital area. By measuring concentrations of some trace elements, she was diagnosed as having a biotin deficit, because of the organic aciduria and undetectable serum biotin concentration. Her serum biotinidase level was normal. Upon administration of oral biotin supplementation, all her symptoms and laboratory findings were dramatically improved. Since amino acid formula contains very few biotin, we should pay attention to biotin deficiency when infants receiving amino acid formula.