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Wiskott-Aldrich syndrome (Robert Anderson Aldrich)
Also known as:
Aldrich’s syndrome
Aldrich-Huntley syndrome
Aldrich-Dees syndrome
Aldrich-Wiskott syndrome
Wiskott's syndrome
Wiskott-Aldrich-Huntley syndrome
Wiskott-Aldrich-Dees syndrome
Associated persons:
Robert Anderson Aldrich
Susan C. Dees
Carolyn Coker Huntley
Alfred Wiskott
Description:
A syndrome occurring only in males, characterised by a triad of eczema, profound thrombocytopenia and frequent infections due to immunological deficiency. It is a sex-linked recessive disorder with a defect in both T and B cell function. Because of the variable severity of the disease some of the patients have lived into their teens, but death may occur at an earlier age. Without therapy, most affected boys seldom survive past 10 years of age. The condition is estimated to be present in about 4.0 per million live births in the USA.
Bibliography:
- A. Wiskott:
Familiärer, angeborener Morbus Werlhoffi?
Monatsschrift für Kinderheilkunde, Berlin, 1937, 68: 212.
- R. A. Aldrich, Arthur G. Steinberg, Donald C. Campbell:
Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea.
Pediatrics, Evanston, Illinois, 1954, 13(2): 133-139.
- C. C. Huntley, S. C. Dees:
Eczema associated with thrombocytopenic purpura and purulent otitis media.
Pediatrics, Evanston, Illinois, 1957, 19: 351-361.
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