D81.818

Billable
ChapterD50–D89
Ranksubcategory
Official Description

Other biotin-dependent carboxylase deficiency

Metadata & Insights
  • •2026 ICD-10-CM Edition - Effective October 1, 2025.
  • •Billable/specific code (valid for reimbursement).
  • •Chapter 3: Diseases of the Blood and Blood-Forming Organs (D50-D89).

Instructional Notes

Includes
  • Holocarboxylase synthetase deficiency
  • Other multiple carboxylase deficiency
General instructions are available for the parent sections:
View CH3 NotesView D80-D89 NotesView D81 NotesView D81.81 Notes

Clinical Hierarchy

CH3Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
D80-D89Certain disorders involving the immune mechanism (D80-D89)
D81Combined immunodeficiencies
D81.8Other combined immunodeficiencies
D81.81Biotin-dependent carboxylase deficiency
D81.818Other biotin-dependent carboxylase deficiency

Applicable Ancestor Context

The following parent sections contain instructions or notes that apply to D81.818:

subcategoryD81.81
Biotin-dependent carboxylase deficiency
View context instructions
categoryD81
Combined immunodeficiencies
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blockD80-D89
Certain disorders involving the immune mechanism (D80-D89)
View context instructions
chapterCH3
Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
View context instructions

Alphabetic Index Mentions

These are Diagnosis Index lookup paths that route to D81.818. Use them as entry terms for search, then confirm final coding instructions in the Tabular List and notes above.

2 raw paths
Immunodeficiency2 unique routes
combined(2 variants)
biotin-dependent carboxylase(2 variants)
holocarboxylase synthetase→D81.818
specified type NEC→D81.818

MS-DRG Classification

2026 v43.1

This code is grouped within the following Diagnosis Related Group(s) for clinical reimbursement:

DRG 640
Miscellaneous Disorders of Nutrition, Metabolism, Fluids and Electrolytes with MCC
DRG 641
Miscellaneous Disorders of Nutrition, Metabolism, Fluids and Electrolytes without MCC

Cross Reference

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