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ICD-10-CM Codes
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D50-D89
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
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D80-D89
Certain disorders involving the immune mechanism
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D81-
Combined immunodeficiencies
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2021 ICD-10-CM Diagnosis Code D81.819
2021 ICD-10-CM Diagnosis Code D81.819
Biotin-dependent carboxylase deficiency, unspecified
2016 2017 2018 2019 2020 2021 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 2021 edition of ICD-10-CM D81.819 became effective on October 1, 2020.
- 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
2021 ICD-10-CM Range D50-D89
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanismType 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)
- D80-D89
2021 ICD-10-CM Range D80-D89
Certain disorders involving the immune mechanismIncludes- 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)
- D81
ICD-10-CM Diagnosis Code D81
Combined immunodeficiencies
2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
Type 1 Excludes- autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
- D81.81
ICD-10-CM Diagnosis Code D81.81
Biotin-dependent carboxylase deficiency
2016 2017 2018 2019 2020 2021 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
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 v38.0):
- 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
- 2021 (effective 10/1/2020): No change
- Deficiency, deficient
- biotin-dependent carboxylase D81.819
- Immunodeficiency D84.9
ICD-10-CM Diagnosis Code D84.9
Immunodeficiency, unspecified
2016 2017 2018 2019 2020 2021 Billable/Specific Code
Applicable To- Immunocompromised NOS
- Immunodeficient NOS
- Immunosuppressed NOS
- combined D81.9
ICD-10-CM Diagnosis Code D81.9
Combined immunodeficiency, unspecified
2016 2017 2018 2019 2020 2021 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
D81.5 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
D81.818 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.
