1. ICD-10-CM Codes
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2. D50-D89 Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
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3. D80-D89 Certain disorders involving the immune mechanism
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4. D81- Combined immunodeficiencies
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2018 ICD-10-CM Diagnosis Code D81.818

Other biotin-dependent carboxylase deficiency

2016 2017 2018 Billable/Specific Code

• D81.818 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2018 edition of ICD-10-CM D81.818 became effective on October 1, 2017.
• This is the American ICD-10-CM version of D81.818 - other international versions of ICD-10 D81.818 may differ.

• Deficiency, holocarboxylase synthease
• Holocarboxylase synthase deficiency

• A rare autosomal recessive inherited disorder caused by mutations in the hlcs gene. It is characterized by deficiency of the enzyme holocarboxylase synthetase which facilitates the effective use of the vitamin biotin in the body. Signs and symptoms appear early in life and include breathing difficulties, feeding difficulties, alopecia, skin rash, and lethargy. Lifelong administration of biotin supplements is required. If it is not treated properly, it may lead to developmental delays, seizures, and coma.
• A rare autosomal recessive inherited disorder that is manifested in the neonatal period and is caused by mutations in the hlcs gene. It is characterized by deficiency of the enzyme holocarboxylase synthetase which facilitates the effective use of the vitamin biotin in the body. Signs and symptoms include difficulties in feeding and breathing, skin rash, seizures, lethargy, and coma.
• 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).

• 640 Miscellaneous disorders of nutrition, metabolism , fluids and electrolytes with mcc
• 641 Miscellaneous disorders of nutrition, metabolism , fluids and electrolytes without mcc

Convert D81.818 to ICD-9-CM

• 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
• 2017 (effective 10/1/2016): No change
• 2018 (effective 10/1/2017): No change

• Immunodeficiency D84.9
  ICD-10-CM Diagnosis Code D84.9

  Immunodeficiency, unspecified

  2016 2017 2018 Billable/Specific Code
  • combined D81.9
    ICD-10-CM Diagnosis Code D81.9

    Combined immunodeficiency, unspecified

    2016 2017 2018 Billable/Specific Code

    Applicable To

    • Severe combined immunodeficiency disorder [SCID] NOS
    • biotin-dependent carboxylase D81.819
      ICD-10-CM Diagnosis Code D81.819

      Biotin-dependent carboxylase deficiency, unspecified

      2016 2017 2018 Billable/Specific Code

      Applicable To

      • Multiple carboxylase deficiency, unspecified
      • holocarboxylase synthetase D81.818
      • specified type NEC D81.818

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.