Chediak-Higashi syndrome

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Chediak-Higashi syndrome

Immune system

General infections

Sepsis

Neonatal sepsis

Abscesses

Hypersensitivity reactions

Type I hypersensitivity

Food allergy

Anaphylaxis

Asthma

Type II hypersensitivity

Immune thrombocytopenic purpura

Autoimmune hemolytic anemia

Hemolytic disease of the newborn

Goodpasture syndrome

Rheumatic heart disease

Myasthenia gravis

Graves disease

Pemphigus vulgaris

Type III hypersensitivity

Serum sickness

Systemic lupus erythematosus

Poststreptococcal glomerulonephritis

Type IV hypersensitivity

Graft-versus-host disease

Contact dermatitis

Transplants

Transplant rejection

Graft-versus-host disease

Cytomegalovirus infection after transplant (NORD)

Post-transplant lymphoproliferative disorders (NORD)

Immunodeficiences

X-linked agammaglobulinemia

Selective immunoglobulin A deficiency

Common variable immunodeficiency

IgG subclass deficiency

Hyperimmunoglobulin E syndrome

Isolated primary immunoglobulin M deficiency

Thymic aplasia

DiGeorge syndrome

Severe combined immunodeficiency

Adenosine deaminase deficiency

Ataxia-telangiectasia

Hyper IgM syndrome

Wiskott-Aldrich syndrome

Leukocyte adhesion deficiency

Chediak-Higashi syndrome

Chronic granulomatous disease

Complement deficiency

Hereditary angioedema

Asplenia

Immune system organ disorders

Thymoma

Ruptured spleen

Immune system pathology review

Blood transfusion reactions and transplant rejection: Pathology review

Immunodeficiencies: T-cell and B-cell disorders: Pathology review

Immunodeficiencies: Combined T-cell and B-cell disorders: Pathology review

Immunodeficiencies: Phagocyte and complement dysfunction: Pathology review

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Chediak-Higashi syndrome

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Summary

Chediak-Higashi syndrome is a rare, autosomal recessive genetic disorder in which a lysosomal trafficking regulator protein mutation leads to a decrease in phagocytosis. This predisposes individuals to recurrent pyogenic infections. People with Chediak-Higashi syndrome also have partial albinism and peripheral neuropathy. They can present with light skin color and hair that is blond, golden brown, or white. Other symptoms include poor vision and hearing, easy bruising and bleeding, and a heightened sensitivity to cold.

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