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Takayasu's arteritis associated with Wiskott‐Aldrich syndrome
Author(s) -
LAU YL,
WONG SN.,
LAWTON W. M.
Publication year - 1992
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1111/j.1440-1754.1992.tb02703.x
Subject(s) - medicine , arteritis , wiskott–aldrich syndrome , takayasu's arteritis , pancytopenia , renovascular hypertension , gastroenterology , immunology , surgery , pathology , bone marrow , vasculitis , disease , kidney , biochemistry , chemistry , gene
A unique case of a Chinese boy with Wiskott‐Aldrich syndrome (WAS) associated with Takayasu's arteritis is reported. He had eczema, epistaxis and recurrent infections since early infancy and was found to have thrombocytopenia, negative delayed‐type skin hypersensitivity, low T cell number and impaired lymphocyte proliferation to phytohaemagglutinin and concanavalin A. He had high normal serum immunoglobulin (Ig)G and IgA with low IgM and isohaemagglutinin. He presented with hypertensive encephalopathy at 5.5 years of age and an aortogram demonstrated abdominal aortic aneurysm with bilateral stenosis of renal arteries resulting in renovascular hypertension. His hypertension was difficult to control medically and autotransplant of his kidneys to the iliac arteries was performed, but he died in the immediate postoperative period. The relationship between immunodeficiency and collagen‐vascular disease was discussed.