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(D82.0) Wiskott-aldrich syndrome
More details coming soon
57 978 in individuals diagnosis wiskott-aldrich syndrome confirmed
2 889 deaths with diagnosis wiskott-aldrich syndrome
5% mortality rate associated with the disease wiskott-aldrich syndrome
Diagnosis wiskott-aldrich syndrome is diagnosed Men are 15.88% more likely than Women
33 592
Men receive the diagnosis wiskott-aldrich syndrome
1 730 (5.2 %)Died from this diagnosis.
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24 386
Women receive the diagnosis wiskott-aldrich syndrome
1 159 (4.8 %)Died from this diagnosis.
Risk Group for the Disease wiskott-aldrich syndrome - Men and Women aged 5-9
In Men diagnosis is most often set at age 0-19, 25-29, 40-44, 50-64, 70-84
Less common in men the disease occurs at Age 20-24, 30-39, 45-49, 65-69, 85-95+Less common in women the disease occurs at Age 40-44, 50-69, 80-95+
In Women diagnosis is most often set at age 0-39, 45-49, 70-79
Disease Features wiskott-aldrich syndrome
Absence or low individual and public risk
Wiskott-aldrich syndrome - what does this mean
Wiskott-aldrich syndrome (was) is an x-linked genetic disorder caused by a mutation in the was gene, which is responsible for producing a protein involved in the regulation of the immune system. this mutation results in the immune system being weakened, leading to increased susceptibility to infections, eczema, and bleeding. was can also lead to other complications, such as an increased risk of developing certain types of cancer.
What happens during the disease - wiskott-aldrich syndrome
Wiskott-aldrich syndrome (was) is an x-linked recessive immunodeficiency disorder caused by a mutation in the was gene. this gene encodes a protein called wasp, which is important for the proper functioning of the immune system. the mutation in the was gene leads to the production of a defective wasp protein, resulting in a weakened immune system, increased susceptibility to infections, and increased risk of developing autoimmune diseases, such as eczema, thrombocytopenia, and anemia.
Clinical Pattern
More details coming soon
How does a doctor diagnose
- Complete blood count (CBC)
- Immunoglobulin levels
- Flow cytometry
- Platelet function tests
- Immunophenotyping
- Genetic testing
- Skin biopsy
Treatment and Medical Assistance
Main goal: Treat the symptoms of Wiskott-Aldrich syndrome.
- Regular monitoring of platelet counts, clotting factors, and immunoglobulin levels.
- Replacement of clotting factors and immunoglobulins to prevent bleeding and infection.
- Treatment of infections with appropriate antibiotics.
- Infusion of bone marrow or stem cells to restore the bone marrow’s ability to produce healthy blood cells.
- Administration of immunosuppressant drugs to reduce the body’s immune response.
- Regular vaccinations to prevent infections.
8 Days of Hospitalization Required
55 Hours Required for Outpatient Treatment
Wiskott-aldrich syndrome - Prevention
Prevention of wiskott-aldrich syndrome involves genetic counselling and preimplantation genetic diagnosis for families with a known history of the disorder. carrier screening of prospective parents is also recommended to reduce the risk of transmitting the disease to offspring.