Chapter 3 - Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)»Certain disorders involving the immune mechanism (D80-D89)»Biotin-dependent carboxylase deficiency (D81.81)
D81.81
Biotin-dependent carboxylase deficiency
Sub-codes (3)
Related indications (MeSH) (1)
A deficiency in the activities of biotin-dependent enzymes (propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due to one of two defects in BIOTIN metabolism. The neonatal form is due to HOLOCARBOXYLASE SYNTHETASE DEFICIENCY. The late-onset form is due to BIOTINIDASE DEFICIENCY.