scholarly journals Two cases of atypical hemolytic uremic syndrome due to anti-factor H autoantibodies successfully treated by plasma exchange, corticosteroids and mizoribine

Immunobiology ◽  
2016 ◽  
Vol 221 (10) ◽  
pp. 1145
Author(s):  
Toshihiro Sawai ◽  
Yusuke Okuda ◽  
Toshiki Masuda ◽  
Tomoyuki Sakai ◽  
Toshinaga Maeda ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Plasma Exchange ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Uremic Syndrome ◽  
Factor H Autoantibodies

Factor H-autoantibodies in atypical hemolytic uremic syndrome

2007 ◽  
Vol 44 (16) ◽  
pp. 3974
Author(s):  
Stefanie Strobel ◽  
Hans-Martin Dahse ◽  
Wei-shih Liu ◽  
Peter F. Hoyer ◽  
Martin Oppermann ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Uremic Syndrome ◽  
Factor H Autoantibodies

scholarly journals Anti–Factor H Autoantibodies Associated with Atypical Hemolytic Uremic Syndrome

2004 ◽  
Vol 16 (2) ◽  
pp. 555-563 ◽  
Author(s):  
Marie-Agnès Dragon-Durey ◽  
Chantal Loirat ◽  
Sylvie Cloarec ◽  
Marie-Alice Macher ◽  
Jacques Blouin ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Uremic Syndrome ◽  
Factor H Autoantibodies

scholarly journals Heterogeneity but individual constancy of epitopes, isotypes and avidity of factor H autoantibodies in atypical hemolytic uremic syndrome

2016 ◽  
Vol 70 ◽  
pp. 47-55 ◽  
Author(s):  
Pilar Nozal ◽  
Maria E. Bernabéu-Herrero ◽  
Barbara Uzonyi ◽  
Ágnes Szilágyi ◽  
Satu Hyvärinen ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Uremic Syndrome ◽  
Factor H Autoantibodies

Factor H autoantibodies associated with atypical hemolytic uremic syndrome crossreact with factor H-related protein 1

2009 ◽  
Vol 46 (14) ◽  
pp. 2869
Author(s):  
Stefanie Strobel ◽  
Pilar Sánchez-Corral ◽  
Cynthia Abarrategui-Garrido ◽  
Elena Fariza Requejo ◽  
Peter F. Zipfel ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Related Protein ◽  
Uremic Syndrome ◽  
Factor H Autoantibodies

scholarly journals Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome

Blood ◽  
2010 ◽  
Vol 115 (2) ◽  
pp. 379-387 ◽  
Author(s):  
Iain Moore ◽  
Lisa Strain ◽  
Isabel Pappworth ◽  
David Kavanagh ◽  
Paul N. Barlow ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Complement Factor ◽  
Disease Manifestation ◽  
Genes Encoding ◽  
Uremic Syndrome ◽  
Complement Regulator ◽  
Related Proteins ◽  
Factor H Autoantibodies

Abstract Factor H autoantibodies have been reported in approximately 10% of patients with atypical hemolytic uremic syndrome (aHUS) and are associated with deficiency of factor H–related proteins 1 and 3. In this study we examined the prevalence of factor H autoantibodies in the Newcastle cohort of aHUS patients, determined whether the presence of such autoantibodies is always associated with deficiency of factor H–related proteins 1 and 3, and examined whether such patients have additional susceptibility factors and/or mutations in the genes encoding complement regulator/activators. We screened 142 patients with aHUS and found factor H autoantibodies in 13 individuals (age 1-11 years). The presence of the autoantibodies was confirmed by Western blotting. By using multiplex ligation-dependent probe amplification we measured complement factor H–related (CFHR)1 and CFHR3 copy number. In 10 of the 13 patients there were 0 copies of CFHR1, and in 3 patients there were 2. In 3 of the patients with 0 copies of CFHR1 there was 1 copy of CFHR3, and these individuals exhibited a novel deletion incorporating CFHR1 and CFHR4. In 5 patients mutations were identified: 1 in CFH, 1 in CFI, 1 in CD46, and 2 in C3. The latter observation emphasizes that multiple concurrent factors may be necessary in individual patients for disease manifestation.

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