Hemolytic-uremic syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2], Anila Hussain, MD [3]


HUS may be classified as typical (caused by Shiga-toxin producing E. coli/ Shigella infection) or atypical (caused by complement factor abnormalities, other viral or bacterial infections, HIV, malignancy, organ transplantation, and rarely SLE and pregnancy related).


Hemolytic-uremic syndrome (HUS) may be classified as follows:

Typical Or Diarrhea Related

Atypical or Non Diarrheal

Primary Causes

Secondary Causes


  1. Dragon-Durey MA, Frémeaux-Bacchi V, Loirat C, Blouin J, Niaudet P, Deschenes G; et al. (2004). "Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases". J Am Soc Nephrol. 15 (3): 787–95. PMID 14978182.
  2. Fremeaux-Bacchi V, Dragon-Durey MA, Blouin J, Vigneau C, Kuypers D, Boudailliez B; et al. (2004). "Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome". J Med Genet. 41 (6): e84. PMC 1735822. PMID 15173250.
  3. Kavanagh D, Kemp EJ, Mayland E, Winney RJ, Duffield JS, Warwick G; et al. (2005). "Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome". J Am Soc Nephrol. 16 (7): 2150–5. doi:10.1681/ASN.2005010103. PMID 15917334.
  4. Fang CJ, Fremeaux-Bacchi V, Liszewski MK, Pianetti G, Noris M, Goodship TH; et al. (2008). "Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome". Blood. 111 (2): 624–32. doi:10.1182/blood-2007-04-084533. PMC 2200836. PMID 17914026.
  5. Fremeaux-Bacchi V, Moulton EA, Kavanagh D, Dragon-Durey MA, Blouin J, Caudy A; et al. (2006). "Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome". J Am Soc Nephrol. 17 (7): 2017–25. doi:10.1681/ASN.2005101051. PMID 16762990.
  6. Marinozzi MC, Vergoz L, Rybkine T, Ngo S, Bettoni S, Pashov A; et al. (2014). "Complement factor B mutations in atypical hemolytic uremic syndrome-disease-relevant or benign?". J Am Soc Nephrol. 25 (9): 2053–65. doi:10.1681/ASN.2013070796. PMC 4147975. PMID 24652797.
  7. Zhu J, Chaki M, Lu D, Ren C, Wang SS, Rauhauser A; et al. (2016). "Loss of diacylglycerol kinase epsilon in mice causes endothelial distress and impairs glomerular Cox-2 and PGE2 production". Am J Physiol Renal Physiol. 310 (9): F895–908. doi:10.1152/ajprenal.00431.2015. PMC 4867310. PMID 26887830.
  8. Copelovitch, L. & Kaplan, B.S. Pediatr Nephrol (2008) 23: 1951. https://doi.org/10.1007/s00467-007-0518-y
  9. Fakhouri F, Roumenina L, Provot F, Sallée M, Caillard S, Couzi L; et al. (2010). "Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations". J Am Soc Nephrol. 21 (5): 859–67. doi:10.1681/ASN.2009070706. PMC 2865741. PMID 20203157.

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