Hemolytic-uremic syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

HUS may be classified according etiology into 4 subtypes including diarrhoea-associated HUS, non-diarrhea-associated HUS (D−HUS), Streptococcus pneumoniae‐associated HUS, diacylglycerol kinase‐ε (DGKE)‐associated HUS.

Classification

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

Typical Or Diarrhea Related

Shiga-Toxin producing E.Coli/ Shigella infection

Atypical or Non Diarrheal

Primary Causes

Complement Factor abnormalities
- Complement factor H (CFH) mutation/ Factor H Defeciency (Autosomal Dominant)
- Complement Factor I(CFI) Defeciency (Acquired antibody mediated)
- Membrane co-factor protein Defeciency (MCP; CD46)^[1]
- Factor B Overactivity (Complement Factor B mutation)^[2]

References

↑ 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.
↑ 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.

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[pmid17914026-1] 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.

[pmid24652797-2] 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.

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