BIOTIN DEFICIENCY IN AN INFANT FED WITH AMINO-ACID FORMULA AND HYPOALLERGENIC RICE

An amino acid formula produced in Japan is not supplemented with bioti n since biotin is not permitted as a food additive. Biotin deficiency developed in an Ii-month-old Japanese infant who had been diagnosed as a neonate with cow milk and soy bean allergy and fed with an amino ac id formula and hypoallergenic rice processed by protease. Serum levels of zinc, essential fatty acids and biotinidase were within the normal range while that of biotin was below the normal range. Urinary 3-hydr oxy-isovalerate and slightly elevated levels of plasma branched-chain amino acids disappeared 1 week after oral supplementation with I mg: d ay(-1) of biotin as did the symptoms of orificial skin lesions, lethar gy, hypotonia and alopecia later. In summary, to prevent biotin defici ency, biotin should be added to the Japanese amino acid formula.