Primary immunodeficiency

Introduction

Primary immunodeficiency disorders — also called primary immune disorders — are immune system defects that can permit recurrent infections and other problems. Some of these disorders are passed down from parents to children (inherited). Many people with primary immunodeficiency are born missing some of the body's immune defenses, making them more susceptible to germs that cause infections. Between 25,000 and 50,000 people in the Unites States live with a primary immunodeficiency disorder.

According to the World Health Organization, there are over 100 different types of primary immunodeficiency. What type you have depends on which genetic defect is present. Examples include:

• Common variable immune deficiency (hypogammaglobulinemia)
• Selective IgA deficiency
• X-linked agammaglobulinemia (Bruton's disease)
• Severe combined immune deficiency

In some cases, untreated primary immunodeficiency can lead to long-term health problems, including permanent damage to organs such as the ears or lungs, or physical disability.

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