2020 ICD-10-CM Diagnosis Code D81.819

Biotin-dependent carboxylase deficiency, unspecified

    2016 2017 2018 2019 2020 Billable/Specific Code
  • D81.819 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM D81.819 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of D81.819 - other international versions of ICD-10 D81.819 may differ.
Applicable To
  • Multiple carboxylase deficiency, unspecified
The following code(s) above D81.819 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to D81.819:
  • D50-D89
    2020 ICD-10-CM Range D50-D89

    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

    Type 2 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • certain conditions originating in the perinatal period (P00-P96)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • human immunodeficiency virus [HIV] disease (B20)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
  • D80-D89
    2020 ICD-10-CM Range D80-D89

    Certain disorders involving the immune mechanism

    Includes
    • defects in the complement system
    • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
    • sarcoidosis
    Type 1 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • functional disorders of polymorphonuclear neutrophils (D71)
    • human immunodeficiency virus [HIV] disease (B20)
    Certain disorders involving the immune mechanism
  • D81
    ICD-10-CM Diagnosis Code D81

    Combined immunodeficiencies

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
    Combined immunodeficiencies
  • D81.81
    ICD-10-CM Diagnosis Code D81.81

    Biotin-dependent carboxylase deficiency

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Applicable To
    • Multiple carboxylase deficiency
    Type 1 Excludes
    • biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
    Biotin-dependent carboxylase deficiency
Approximate Synonyms
  • Biotin dependent carboxylase deficiency
Clinical Information
  • 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.
ICD-10-CM D81.819 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 640 Miscellaneous disorders of nutrition, metabolism, fluids and electrolytes with mcc
  • 641 Miscellaneous disorders of nutrition, metabolism, fluids and electrolytes without mcc

Convert D81.819 to ICD-9-CM

Code History
  • 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
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to D81.819:
  • Deficiency, deficient
    • biotin-dependent carboxylase D81.819
  • Immunodeficiency D84.9
    ICD-10-CM Diagnosis Code D84.9

    Immunodeficiency, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    • combined D81.9
      ICD-10-CM Diagnosis Code D81.9

      Combined immunodeficiency, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Severe combined immunodeficiency disorder [SCID] NOS

ICD-10-CM Codes Adjacent To D81.819
D81.32 Adenosine deaminase 2 deficiency
D81.39 Other adenosine deaminase deficiency
D81.4 Nezelof's syndrome
D81.5 Purine nucleoside phosphorylase [PNP] deficiency
D81.6 Major histocompatibility complex class I deficiency
D81.7 Major histocompatibility complex class II deficiency
D81.8 Other combined immunodeficiencies
D81.81 Biotin-dependent carboxylase deficiency
D81.810 Biotinidase deficiency
D81.818 Other biotin-dependent carboxylase deficiency
D81.819 …… unspecified
D81.89 Other combined immunodeficiencies
D81.9 Combined immunodeficiency, unspecified
D82 Immunodeficiency associated with other major defects
D82.0 Wiskott-Aldrich syndrome
D82.1 Di George's syndrome
D82.2 Immunodeficiency with short-limbed stature
D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus
D82.4 Hyperimmunoglobulin E [IgE] syndrome
D82.8 Immunodeficiency associated with other specified major defects
D82.9 Immunodeficiency associated with major defect, unspecified

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