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Primary Immunodeficiency Disorders

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Primary Immunodeficiency Disorders

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Primary Immunodeficiency Disorders (PID)

Definition

The immunodeficiency disorders are a diverse group of illnesses that, as a result of one or more abnormalities of the immune system, increase susceptibility to infection.

The PID are not associated with other illnesses that impair the immune system.

Many are genetic disorders with a characteristic inheritance pattern.

International Union of Immunological Societies (IUIS) expert Committee on primary Immunodeficiency in

1973 ,Classification every 2 years

2015

For Dx & better Treament

8 tables:

1 Combined ID

2 Well defined syndrome with ID

3 prominent Ab defect

,4 defect of Immune dysregulation(HLH)

5 phagocytic defect

Innate immunity6

7 Auto-inflammatory condition(FMF)

8 complement

9 phenocopy of ID

Incidence

Estimated occurrence of PID is 1 per 10000 live birth, excluding the asymptomatic Ig A def

First IRPID report: CVID was the most common PID in Iran

Since 1952 more than 200 different PID disorders had been defined

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