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Diseases » Primary Immune Deficiency » Glossary

Glossary for Primary Immune Deficiency

AIDS: A term given to HIV patients who have a low CD4 count (below 200) which means that they have low levels of a type of immune cell called T-cells. AIDS patients tend to develop opportunistic infections and cancers. Opportunistic infections are infections that would not normally affect a person with a healthy immune system. The HIV virus is a virus that attacks the body's immune system.
Ataxia Telangiectasia: A rare inherited childhood disorder involving progressive degeneration of the nervous system.
Bizarre medical conditions: Various unusual and unexpected medical disorders
Candidiasis: Fungal infection of moist areas such as mouth or vagina
Cartilage Hair Hypoplasia: An inherited disorder characterized by short-limb dwarfism as well as hair that is short, fine, brittle and often light colored.
Celiac Disease: Digestive intolerance to gluten in the diet.
Chediak-Higashi Syndrome: A rare genetic disorder where both parents need to be carriers for it to be passed on. Sufferers have reduced skin pigmentation to the point of albinism in some cases. Characteristic white cell abnormalities increase bleeding problems and increase the risk of infections.
Chronic Granulomatous Disease: A very rare inherited blood disorder where certain cells involved with immunity (phagocytes) are unable to destroy bacteria and hence the patient suffers repeated bacterial infections.
Chronic bronchitis: Chronic bronchitis is a chronic inflammation of the bronchi (medium-size airways) in the lungs.
Chronic sinusitis: Chronic form of sinusitis, inflammation of the sinus cavities.
Common Variable Immunodeficiency: An immunodeficiency disorder involving low blood gamma globulin levels which results in an increased susceptibility to infections. The condition may be inherited or can be caused by certain drugs (levamisole, hydantoin and carbamazepine).
Complement deficiencies: A deficiency in the effectiveness of complement in the body
Cystic Fibrosis: Cystic fibrosis is a hereditary disease affecting the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure.
DiGeorge syndrome: 22q11.2 deletion syndrome is a genetic disorder which can result in a vast array of symptoms. Various names have been used to describe different manifestations of the syndrome. Di George Syndrome primarily involves an underdeveloped thymus and parathyroid glands which results in lowered immunity low blood calcium levels respectively. Another primary feature is heart defects. Various other variable features are also present. It is not uncommon for patients to have more than one of the 22q11.2 deletion syndrome subtypes which can make diagnosis confusing - other subtypes include Sphrintzen syndrome, Caylor cardiofacial syndrome and CATCH 22.
Good syndrome: A rare condition characterized by low immunity (due to reduced number of B cells) and thymoma.
Hyper-IgE Syndrome: A condition characterized by an excess of immunoglobulin E
Hyper-IgM Syndrome: A rare inherited immunodeficiency disorder which causes frequent infections involving the ears, eyes, sinuses, lungs, skin, respiratory tract and other areas of the body.
Immune deficiency conditions: Any of various diseases that suppress the immune system.
Immune disorders: Disorders that affect the immune system
Immunoglobulin G subclass deficiency: A deficiency of one or more of the subclasses of immunoglobulin G. The overall IgG level may be normal but the subclass levels will vary (there are four subclasses). Some patients remain healthy despite having some degree of IgG deficiency.
Leukocyte Adhesion Defect: A rare inherited disorder characterized by abnormal neutrophil functioning which reduces the body's immunity. The abnormal neutrophils are unable to be transported to sites of infection due to their inability to adhere to certain blood vessel components which would normally lead them to the infection site. Infections may be life-threatening as the body is unable to destroy bacteria effectively.
Lupus: Autoimmune disease with numerous effects on various organs and linings.
Opportunistic infections: Is defined as an infection that occurs due to an organism that does not usually cause disease but becomes pathogenic under certain conditions
Progeria short stature pigmented nevi: A very rare inherited disorder characterized by premature aging, short stature, and immune system deficiency. The type and severity of symptoms is variable.
Purine nucleoside phosphorylase (PNP) deficiency: A condition which is characterized by a deficiency of purine nucleoside phosphorylate
Respiratory symptoms: Symptoms affecting the breathing systems.
SCID: Major failure of the immune system, usually genetic.
Selective IgA Deficiency: Immune deficiency from lacking immunoglobulin A (IgA) antibodies.
Wiskott-Aldrich Syndrome: An inherited immune system disorder that affects only males and is characterized by recurring infections, eczema and reduced level of blood platelets (thrombocytopenia).
X-Linked Agammaglobulinemia: Immune deficiency from lack of antibodies.