What is the most severe inherited immunodeficiency disease?

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asked Feb 25 in Diseases Conditions by sezro833 (1,940 points)
What is the most severe inherited immunodeficiency disease?

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answered Mar 25 by atnight (4,540 points)
The most severe inherited immunodeficiency disease is severe combined immunodeficiency which is a very rare genetic disorder which causes life threatening issues with the immune system.

Severe combined immunodeficiency is a type of primary immune deficiency also called an inborn error of immunity.

The most common primary immunodeficiency disease is an IgA deficiency which occurs in around 1 in 300 to 1 in 500 people.

The most common problem in immunodeficient patients with a primary antibody deficiency is recurrent infections.

People with immunodeficiency are often referred to infectious disease specialists prior to them being seen by a immunologist.

The complications of antibody deficiency include.

Recurrent infections.
Autoimmune disorders.
Damage to the heart, lungs, nervous system or digestive tract.
Slowed growth.
Increased risk of cancer.
Death from serious infection.

The most common antibody deficiency disorders are.

X-linked agammaglobulinemia (Bruton disease).
Transient hypogammaglobulinemia of newborn.
Selective Ig immunodeficiencies, for example, IgA selective deficiency.
Super IgM syndrome.
Common variable immunodeficiency disorder.

Recurrent ear infections, sinusitis, bronchitis, and pneumonia are the most frequently observed illnesses in people with SAD. Some individuals show an increased frequency of infection beginning in the first years of life.

In others, the onset of infections may occur later.

The prognosis for patients with specific antibody deficiency is generally reasonable.

Children have been known to outgrow specific antibody deficiency naturally and those who do not are still able to maintain a good quality of life with antibiotic and immunoglobulin therapy.

Immunodeficiency results from a failure or absence of elements of the immune system, including lymphocytes, phagocytes, and the complement system.

These immunodeficiencies can be either primary, such as Bruton disease, or secondary, as the one caused by HIV infection.

Primary Immunodeficiency. B-cell Deficiencies.

Autoimmune diseases that have associated antibodies are.

Anti-CCP: Rheumatoid arthritis.
Anti-ganglioside antibodies: Miller-Fisher syndrome, acute motor axonal neuropathy, multifocal motor neuropathy with conduction block.
Anti-mitochondrial antibody: Primary biliary cirrhosis.

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