ICD-10 Diagnosis Code D81.818

Other biotin-dependent carboxylase deficiency

Diagnosis Code D81.818

ICD-10: D81.818
Short Description: Other biotin-dependent carboxylase deficiency
Long Description: Other biotin-dependent carboxylase deficiency
This is the 2017 version of the ICD-10-CM diagnosis code D81.818

Code Classification
  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
    • Certain disorders involving the immune mechanism (D80-D89)
      • Combined immunodeficiencies (D81)

Information for Medical Professionals

Diagnostic Related Groups
The diagnosis code D81.818 is grouped in the following Diagnostic Related Group(s) (MS-DRG v33.0)


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The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

  • Biotin-
  • Holocarboxylase synthase deficiency
  • Propionic acidemia

Index of Diseases and Injuries
References found for the code D81.818 in the Index of Diseases and Injuries:

Information for Patients

Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs that work together to defend against germs. It helps your body to recognize these "foreign" invaders. Then its job is to keep them out, or if it can't, to find and destroy them.

If your immune system cannot do its job, the results can be serious. Disorders of the immune system include

  • Allergy and asthma - immune responses to substances that are usually not harmful
  • Immune deficiency diseases - disorders in which the immune system is missing one or more of its parts
  • Autoimmune diseases - diseases causing your immune system to attack your own body's cells and tissues by mistake

NIH: National Institute of Allergy and Infectious Diseases

  • Agammaglobulinemia
  • Aging changes in immunity
  • Chronic granulomatous disease
  • Graft-versus-host disease
  • Histiocytosis
  • Hyperimmunoglobulin E syndrome
  • Immune response
  • Immunodeficiency disorders
  • Selective deficiency of IgA

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Holocarboxylase synthetase deficiency Holocarboxylase synthetase deficiency is an inherited disorder in which the body is unable to use the vitamin biotin effectively. This disorder is classified as a multiple carboxylase deficiency, a group of disorders characterized by impaired activity of certain enzymes that depend on biotin.The signs and symptoms of holocarboxylase synthetase deficiency typically appear within the first few months of life, but the age of onset varies. Affected infants often have difficulty feeding, breathing problems, a skin rash, hair loss (alopecia), and a lack of energy (lethargy). Immediate treatment and lifelong management with biotin supplements may prevent many of these complications. If left untreated, the disorder can lead to delayed development, seizures, and coma. These medical problems may be life-threatening in some cases.
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