Henoch-Schonlein purpura in Wiskott-Aldrich syndrome

Duzova A., Topaloglu R., Sanal O., Kilic S. Ş., Mazza C., Besbas N., ...More

PEDIATRIC NEPHROLOGY, vol.16, no.6, pp.500-502, 2001 (SCI-Expanded) identifier

  • Publication Type: Article / Article
  • Volume: 16 Issue: 6
  • Publication Date: 2001
  • Doi Number: 10.1007/s004670100583
  • Journal Name: PEDIATRIC NEPHROLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.500-502
  • Keywords: abnormal glycosylation, Henoch-Schonlein purpura, IgA nephropathy, Wiskott-Aldrich syndrome, IGA NEPHROPATHY, SYNDROME PROTEIN, HINGE REGION, GLYCOSYLATION, EXPRESSION, GLYCANS, DISEASE
  • Bursa Uludag University Affiliated: Yes

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-Schonlein 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.