Abstract
Wiskott-Aldrich syndrome (WAS) is a rare immune deficiency disease. Sialophorin glycosylation is defective in WAS. Although it is not very common, renal involvement including IgA nephropathy (IgAN) was reported. Abnormal glycosylation plays a key role in the pathogenesis of IgAN. We present an 8-year-old boy with WAS who had recurrent episodes of Henoch-Schönlein purpura with renal involvement following upper respiratory tract infections. His renal function did not deteriorate. Both IgAN and WAS have glycosylation defects, but there must be some other factors (genetic and environmental) to explain their rare association.
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Received: 13 July 2000 / Revised: 10 January 2001 / Accepted: 17 January 2001
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Duzova, A., Topaloglu, R., Sanal, O. et al. Henoch-Schönlein purpura in Wiskott-Aldrich syndrome. Pediatr Nephrol 16, 500–502 (2001). https://doi.org/10.1007/s004670100583
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DOI: https://doi.org/10.1007/s004670100583