What are Primary Immunodeficiency Diseases?

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The complex function of the immune system requires the cooperation of various organs, tissues, cells and proteins.

A heritable abnormality in any of these factors of the complex immune system can lead to primary immune deficiency.

The abnormality may be reflected in many forms such as, stem cell deficiency (defects in hemopoisis), B cell deficiency, T- cell deficiency, combined B and T cell deficiency, deficiency of phagocytes, complement deficiency etc. Immunodeficiency’s range from very serious to very mild.

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The serious forms are often noticeable at birth or shortly thereafter.

However, very mild forms may not be diagnosed until later in life, during adolescence or young adulthood. Since immune deficiency diseases are caused by hereditary or genetic defects they are not contagious, and can affect anyone, regardless of age or sex.

The World Health Organization (WHO) has identified nearly 150 primary immune deficiency diseases. The overall incidence of symptomatic primary immunodeficiency is estimated to be 1/10,000.

Patients with primary immunodeficiency diseases commonly have an increased susceptibility to infection and require life-long therapies, such as intravenous immune globulin infusions, antibiotic therapies, bone marrow transplantations etc.

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Primary immune deficiency diseases are generally grouped by the part of the immune system that is malfunctioning. They are sometimes classified according to which component is faulty (T cells, B cells, phagocytic cells or complement) or according to individual clinical syndromes.

Four commonest primary immunodeficiencies seen apart from physiologic at hypogammaglobulinaemia of infancy, are transient hypogammaglobulinaemia of infancy (THI), IgG subclass deficiency, partial antibody deficiency with impaired polysaccharide responsiveness (IPR) and selective IgA deficiency.

Symptoms of primary immunodeficiency:

The most common problem for people with primary immunodeficiency disease is that they are more likely to get infections more often and the infections are more severe, longer lasting, and harder to cure than people with normal immune systems.

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In addition to opportunistic infections autoimmune problems are more common in primary immune deficiency.

Treatment for primary immunodeficiency

Even though no single treatment works in all cases of primary immunodeficiency, there are many treatment options that can even be combined to make it more likely to treat primary immunodeficiency. The basic treatment falls into three stages:

1. Clearing the current infection.

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2. Avoiding germs and preventing exposure to new infections.

3. Correcting the immunodeficiency. Prevention of primary immunodeficiency syndromes

Prevention of primary immunodeficiency depends on the identification and genetic counseling of likely carriers in families with a positive history. Prenatal diagnosis using such techniques as cultured amniotic cells or foetal blood may be possible for some disorders such as severe combined immunodeficiency. X-linked disorders may be excluded by sex determination.

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