Prevalence and Incidence of Primary Immune Deficiency

Prevalance of types of Primary Immune Deficiency:

For details see prevalence of types of Primary Immune Deficiency analysis; summary of available prevalence data:

• Selective IgA Deficiency: 1 in 300 (NIDCD); 1 in 600 (NIAID); 1 in 333 Caucasians.
• X-Linked Agammaglobulinemia: 1-in-100,000
• Chronic Granulomatous Disease: 1-in-4 million to 5 million (NIAID)
• SCID: approximately 1-per-500,000 (NIDCD); 1-per-million
• Ataxia Telangiectasia: 1 per 40,000 - 100,000 people worldwide suffer from ataxia-telangiectasia, Genetics Home Reference website
• Chediak-Higashi Syndrome: rare
• more types of Primary Immune Deficiency...»

Prevalence/Incidence of Primary Immune Deficiency: Online Medical Books

16 MEDICAL BOOKS ONLINE! Review excerpts from medical books online, free, without registration, for more information about the prevalence and/or incidence of Primary Immune Deficiency.

Severe combined immunodeficiency disease: Causes and incidence
(Professional Guide to Diseases (Eighth Edition))

SCID is usually transmitted as an autosomal recessive trait, although it may be X-linked. In most cases, the genetic defect seems associated with failure of the stem cell to differentiate into T and B lymphocytes. Many molecular defects such as mutation of the kinase ZAP-70 can cause SCID. X-linked SCID is due to a mutation of a subunit of the interleukin (IL)-2, IL-4, and IL-7 receptors. Less commonly, it results from an enzyme deficiency.

SCID affects more males than females. Its estimated incidence is 1 in every 100,000 to 500,000 births. Most untreated patients die from infection within 1 year of birth.

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Source: Professional Guide to Diseases (Eighth Edition), 2005

Immune Deficiency: Immune Deficiency - epidemiology
(The 5-Minute Pediatric Consult)

Immune Deficiency - prevalence

Most common immunodeficiency is IgA deficiency, with an estimated prevalence of 1:500 people:

• 1:3,000 for chromosome 22q11.2 deletion syndrome (DiGeorge syndrome)
• 1:60,000 for SCID
• 1:200,000 for chronic granulomatous disease

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Source: The 5-Minute Pediatric Consult, 2008

Congenital Immunodeficiencies: Epidemiology
(Pediatric Infectious Disease)

The identification of primary immunodeficiencies in children is critical. A child with an unrecognized immunodeficiency may expire from an overwhelming opportunistic infection. Even when not fatal, multiple pneumonias in such patients can progress to chronic, debilitating pulmonary disease. Children with underlying immunodeficiency are also susceptible to adverse reactions if administered live vaccinations. It is important that the pediatrician consider the possibility of underlying immunodeficiency when managing serious infections in the first year of life.

The average child can have between six and eight respiratory infections per year. This number can increase if there are additional risk factors, such as attendance at day care, sibling exposures, or atopic disease. Although parents may complain that their child is “always sick,” these infections are usually self-limited upper respiratory infections.

Age-related Clinical Patterns for Primary Immunodeficiencies

The immunodeficiencies involving T cells, such as severe combined immunodeficiency (SCID) or DiGeorge ’s syndrome, appear at an earlier age. These defects often present in the first 6 months of life with failure to thrive, diarrhea, pneumonia, and thrush. Significant lymphopenia is often seen. Antibody deficiency disease, such as X-linked agammaglobulinemia (Bruton ’s disease) often clinically manifest as maternal immunoglobulin levels wane after 6 months of age. Immunoglobulin deficiencies typically present with severe recurrent bacterial respiratory infections.

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Source: Pediatric Infectious Disease, 2004

About prevalence and incidence statistics:

The term 'prevalence' of Primary Immune Deficiency usually refers to the estimated population of people who are managing Primary Immune Deficiency at any given time. The term 'incidence' of Primary Immune Deficiency refers to the annual diagnosis rate, or the number of new cases of Primary Immune Deficiency diagnosed each year. Hence, these two statistics types can differ: a short-lived disease like flu can have high annual incidence but low prevalence, but a life-long disease like diabetes has a low annual incidence but high prevalence. For more information see about prevalence and incidence statistics.