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Immunodeficiency is also the hallmark of acquired immunodeficiency syndrome (AIDS), [11] caused by the human immunodeficiency virus (HIV). HIV directly infects a small number of T helper cells, and also impairs other immune system responses indirectly.
The precise symptoms of a primary immunodeficiency depend on the type of defect. Generally, the symptoms and signs that lead to the diagnosis of an immunodeficiency include recurrent or persistent infections or developmental delay as a result of infection. Particular organ problems (e.g. diseases involving the skin, heart, facial development ...
This is a list of primary immunodeficiencies (PID), which are immune deficiencies that are not secondary to another condition.. The International Union of Immunological Societies recognizes nine classes of primary immunodeficiencies, totaling approximately 430 conditions.
Severe combined immunodeficiency (SCID) DiGeorge syndrome; Hyperimmunoglobulin E syndrome (also known as Job's Syndrome) Common variable immunodeficiency (CVID): B cell levels are normal in circulation but with decreased production of IgG throughout the years, so it is the only primary immune disorder that presents onset in the late teens years.
Nuclear factor-kappa B Essential Modulator (NEMO) deficiency syndrome is a rare type of primary immunodeficiency disease that has a highly variable set of symptoms and prognoses. It mainly affects the skin and immune system but has the potential to affect all parts of the body, including the lungs , urinary tract and gastrointestinal tract . [ 1 ]
Adult-onset immunodeficiency syndrome is a type of immunodeficiency. It is linked to vulnerability to disseminated infections brought on by opportunistic pathogens. People with this condition have increased levels of anti-interferon-gamma autoantibodies. These particular immune system proteins mistakenly target an individual's own tissues.
ICF syndrome patients exhibit facial anomalies which include hypertelorism, low-set ears, epicanthal folds and macroglossia. [3] Other frequent symptoms observed in individuals with ICF syndrome include intellectual disability, recurrent and prolonged respiratory infections, and integumentary and digestive system infections.
When a clinical diagnosis of combined immunodeficiency is suspected, preliminary laboratory tests should be ordered. The patient's complete blood count (CBC) reveals immunological changes. The absolute neutrophil and lymphocyte count should be determined based on the patient's age. In all patients, HIV should be ruled out.