Combined immunodeficiencies (D81)
ICD-10 Index
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)
- D81 - Combined immunodeficiencies NON-BILLABLE CODE
- D81.0 - Severe combined immunodeficiency with reticular dysgenesis BILLABLE CODE
- D81.1 - Severe combined immunodeficiency w low T- and B-cell numbers BILLABLE CODE
- D81.2 - Severe combined immunodef w low or normal B-cell numbers BILLABLE CODE
- D81.3 - Adenosine deaminase [ADA] deficiency NON-BILLABLE CODE
- D81.30 - Adenosine deaminase deficiency, unspecified BILLABLE CODE
- D81.31 - Severe combined immunodef due to adenosine deaminase defic BILLABLE CODE
- D81.32 - Adenosine deaminase 2 deficiency BILLABLE CODE
- D81.39 - Other adenosine deaminase deficiency BILLABLE CODE
- D81.4 - Nezelof's syndrome BILLABLE CODE
- D81.5 - Purine nucleoside phosphorylase [PNP] deficiency BILLABLE CODE
- D81.6 - Major histocompatibility complex class I deficiency BILLABLE CODE
- D81.7 - Major histocompatibility complex class II deficiency BILLABLE CODE
- D81.8 - Other combined immunodeficiencies NON-BILLABLE CODE
- D81.81 - Biotin-dependent carboxylase deficiency NON-BILLABLE CODE
- D81.810 - Biotinidase deficiency BILLABLE CODE
- D81.818 - Other biotin-dependent carboxylase deficiency BILLABLE CODE
- D81.819 - Biotin-dependent carboxylase deficiency, unspecified BILLABLE CODE
- D81.82 - Activated Phosphoinositide 3-kinase Delta Syndrome [APDS] BILLABLE CODE NEW CODE
- D81.89 - Other combined immunodeficiencies BILLABLE CODE
- D81.9 - Combined immunodeficiency, unspecified BILLABLE CODE
Combined immunodeficiencies (D81)
Clinical Information for Combined immunodeficiencies (D81)
Biotinidase Deficiency - The late onset form of MULTIPLE CARBOXYLASE DEFICIENCY (deficiency of the activities of biotin-dependent enzymes propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due to a defect or deficiency in biotinidase which is essential for recycling BIOTIN.
Holocarboxylase Synthetase Deficiency - 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).
Multiple Carboxylase Deficiency - 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.
Biotinidase Deficiency - The late onset form of MULTIPLE CARBOXYLASE DEFICIENCY (deficiency of the activities of biotin-dependent enzymes propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due to a defect or deficiency in biotinidase which is essential for recycling BIOTIN.
Biotinidase Deficiency - The late onset form of MULTIPLE CARBOXYLASE DEFICIENCY (deficiency of the activities of biotin-dependent enzymes propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due to a defect or deficiency in biotinidase which is essential for recycling BIOTIN.
Holocarboxylase Synthetase Deficiency - 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).
Multiple Carboxylase Deficiency - 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.
Holocarboxylase Synthetase Deficiency - 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).
Multiple Carboxylase Deficiency - 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.
Instructional Notations
Type 1 Excludes Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- autosomal recessive agammaglobulinemia Swiss type D80.0
