Prevalence and Incidence of Wiskott-Aldrich Syndrome
Wiskott-Aldrich Syndrome: Rare Disease
Wiskott-Aldrich Syndrome is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Wiskott-Aldrich Syndrome, or a subtype of Wiskott-Aldrich Syndrome,
affects less than 200,000 people in the US population.
Wiskott-Aldrich Syndrome Prevalence: Book Excerpts
Prevalence/Incidence of Wiskott-Aldrich Syndrome: Online Medical Books
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Review excerpts from medical books online, free, without registration,
for more information about the prevalence and/or incidence of Wiskott-Aldrich Syndrome.
Wiskott-Aldrich syndrome:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
Because Wiskott-Aldrich syndrome results from an X-linked recessive trait, it affects only males. Children with this genetic defect are born with a normal thymus gland, plasma cells, and lymphoid tissues. However, an inherited defect in both B-cell and T-cell function compromises the child’s immune system response and increases his vulnerability to infection. These children also have a metabolic defect in platelet synthesis that causes them to produce only small, short-lived platelets, resulting in thrombocytopenia.
Wiskott-Aldrich syndrome occurs in 4 neonates per 1 million live births.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Acquired immunodeficiency syndrome:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
AIDS results from infection with HIV, which has two forms: HIV-1 and HIV-2. Both forms of HIV have the same modes of transmission and similar opportunistic infections associated with AIDS, but studies indicate that HIV-2 develops more slowly and presents with milder symptoms than HIV-1.
Transmission occurs through contact with infected blood or body fluids and is associated with identifiable high-risk behaviors. It’s disproportionately represented in:
❑ homosexual and bisexual men
❑ persons who use illicit I.V. drugs
❑ neonates of infected females
❑ recipients of contaminated blood or blood products (incidence dramatically decreased since mid-1985)
❑ heterosexual partners of persons in the former groups.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
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.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Wiskott Aldrich Syndrome:
Wiskott Aldrich Syndrome - epidemiology
(The 5-Minute Pediatric Consult)
- Presents in infancy with serious bleeding episodes secondary to thrombocytopenia (such as circumcision with increased bleeding, bloody diarrhea, ecchymoses)
- Recurrent infections usually start after 6 months of age:
- Bacterial: Otitis media, sinusitis, meningitis, sepsis, and pneumonia
- Viral infections: Herpes simplex virus, varicella with systemic complications
- Milder phenotypes may lack history of recurrent infections.
- Eczema is usually present by 1 year of age. (May be resistant to therapy, sometimes requiring systemic antibiotics)
Wiskott Aldrich Syndrome - incidence
- For WAS/XLT estimate is 10 in 1 million live births.
- Prevalence of XLT equal to WAS.
» READ BOOK EXCERPT ONLINE »
Source: The 5-Minute Pediatric Consult, 2008
About prevalence and incidence statistics:
The term 'prevalence' of Wiskott-Aldrich Syndrome usually refers to the estimated population
of people who are managing Wiskott-Aldrich Syndrome at any given time.
The term 'incidence' of Wiskott-Aldrich Syndrome refers to the annual diagnosis rate,
or the number of new cases of Wiskott-Aldrich Syndrome 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.
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