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Biotin Deficiency in an Infant Fed with Amino Acid Formula
Author(s) -
Fujimoto Wataru,
Inaoki Makoto,
Fukui Toru,
Inoue Yoshito,
Kuhara Tomiko
Publication year - 2005
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2005.tb00758.x
Subject(s) - biotin , biotin deficiency , biotinidase deficiency , excretion , urine , weaning , medicine , urinary system , endocrinology , physiology , dermatology , chemistry , biochemistry
Biotin deficiency is rarely encountered in an infant on weaning from breast and formula feeding. It is characterized by alopecia and scaly, erythematous dermatitis distributed around the body orifices. We report a 5‐month‐old Japanese infant with typical skin lesions who had been diagnosed as a neonate with dyspepsia and fed only an amino acid formula. Serum and urine levels of biotin were below the normal range, but zinc and biotinidase were within normal range. Urinary excretion of 3‐methylcrotonylglycine, 3‐hydroxyisovaleric acid, and methylcitric acid was significantly elevated. Daily oral supplementation with 1 mg of biotin resulted in dramatic improvement of the periorificial dermatitis and hair growth together with a complete disappearance of the organic aciduria. Our case shows that the characteristic skin manifestations are the most important clue to the diagnosis of biotin deficiency and demonstrated that urinary excretion of biotin and organic aciduria, rather than the serum concentration of biotin, are the sensitive indicators for evaluating the patient's status of biotin deficiency.