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holocarboxylase synthetase deficiency

Medical Definition

The neonatal form of multiple carboxylase deficiency that is caused by a defect or deficiency in holocarboxylase synthetase. hlcs is the enzyme that covalently links biotin to the biotin dependent carboxylases (propionyl-coa-carboxylase, pyruvate carboxylase, and beta-methylcrotonyl-coa carboxylase).
Related Codes (1)
Code
Description
Billable
Details
D81.818Other biotin-dependent carboxylase deficiency

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