ICD-10 Diagnosis Code E70.330

Chediak-Higashi syndrome

Diagnosis Code E70.330

ICD-10: E70.330
Short Description: Chediak-Higashi syndrome
Long Description: Chediak-Higashi syndrome
This is the 2017 version of the ICD-10-CM diagnosis code E70.330

Code Classification
  • Endocrine, nutritional and metabolic diseases
    • Metabolic disorders (E70-E88)
      • Disorders of aromatic amino-acid metabolism (E70)

Information for Medical Professionals

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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.

  • Chédiak-Higashi syndrome
  • Chédiak-Higashi syndrome
  • Chemotactic disorder
  • Chemotactic disorder
  • Combined phagocytic defect
  • Combined phagocytic defect
  • Dense body defect
  • Dense body defect
  • Granulocyte granule deficiency
  • Granulocyte granule deficiency
  • Partial albinism
  • Partial albinism
  • Periodontitis co-occurrent with Chédiak-Higashi syndrome
  • Periodontitis co-occurrent with genetic disorder
  • Platelet storage pool defect
  • Platelet storage pool defect

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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Chediak-Higashi syndrome Chediak-Higashi syndrome is a condition that affects many parts of the body, particularly the immune system. This disease damages immune system cells, leaving them less able to fight off invaders such as viruses and bacteria. As a result, most people with Chediak-Higashi syndrome have repeated and persistent infections starting in infancy or early childhood. These infections tend to be very serious or life-threatening.Chediak-Higashi syndrome is also characterized by a condition called oculocutaneous albinism, which causes abnormally light coloring (pigmentation) of the skin, hair, and eyes. Affected individuals typically have fair skin and light-colored hair, often with a metallic sheen. Oculocutaneous albinism also causes vision problems such as reduced sharpness; rapid, involuntary eye movements (nystagmus); and increased sensitivity to light (photophobia).Many people with Chediak-Higashi syndrome have problems with blood clotting (coagulation) that lead to easy bruising and abnormal bleeding. In adulthood, Chediak-Higashi syndrome can also affect the nervous system, causing weakness, clumsiness, difficulty with walking, and seizures.If the disease is not successfully treated, most children with Chediak-Higashi syndrome reach a stage of the disorder known as the accelerated phase. This severe phase of the disease is thought to be triggered by a viral infection. In the accelerated phase, white blood cells (which normally help fight infection) divide uncontrollably and invade many of the body's organs. The accelerated phase is associated with fever, episodes of abnormal bleeding, overwhelming infections, and organ failure. These medical problems are usually life-threatening in childhood.A small percentage of people with Chediak-Higashi syndrome have a milder form of the condition that appears later in life. People with the adult form of the disorder have less noticeable changes in pigmentation and are less likely to have recurrent, severe infections. They do, however, have a significant risk of progressive neurological problems such as tremors, difficulty with movement and balance (ataxia), reduced sensation and weakness in the arms and legs (peripheral neuropathy), and a decline in intellectual functioning.
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