Hemolytic-uremic syndrome classification: Difference between revisions

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==== Primary Causes ====
==== Primary Causes ====
* Complement Factor abnormalities
* Complement Factor abnormalities
** Factor H Defeciency (Autosomal Dominant)
** Complement factor H (CFH) mutation/ Factor H Defeciency (Autosomal Dominant)
** Factor I Defeciency (Acquired antibody mediated)
** Complement Factor I(CFI) Defeciency (Acquired antibody mediated)
** Membrane co-factor protein Defeciency<ref name="pmid17914026">{{cite journal| author=Fang CJ, Fremeaux-Bacchi V, Liszewski MK, Pianetti G, Noris M, Goodship TH et al.| title=Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome. | journal=Blood | year= 2008 | volume= 111 | issue= 2 | pages= 624-32 | pmid=17914026 | doi=10.1182/blood-2007-04-084533 | pmc=2200836 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17914026  }} </ref>
** Membrane co-factor protein Defeciency (MCP; CD46)<ref name="pmid17914026">{{cite journal| author=Fang CJ, Fremeaux-Bacchi V, Liszewski MK, Pianetti G, Noris M, Goodship TH et al.| title=Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome. | journal=Blood | year= 2008 | volume= 111 | issue= 2 | pages= 624-32 | pmid=17914026 | doi=10.1182/blood-2007-04-084533 | pmc=2200836 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17914026  }} </ref>
** Factor B Overactivity (Complement Factor B mutation)<ref name="pmid24652797">{{cite journal| author=Marinozzi MC, Vergoz L, Rybkine T, Ngo S, Bettoni S, Pashov A et al.| title=Complement factor B mutations in atypical hemolytic uremic syndrome-disease-relevant or benign? | journal=J Am Soc Nephrol | year= 2014 | volume= 25 | issue= 9 | pages= 2053-65 | pmid=24652797 | doi=10.1681/ASN.2013070796 | pmc=4147975 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24652797  }} </ref>
** Factor B Overactivity (Complement Factor B mutation)<ref name="pmid24652797">{{cite journal| author=Marinozzi MC, Vergoz L, Rybkine T, Ngo S, Bettoni S, Pashov A et al.| title=Complement factor B mutations in atypical hemolytic uremic syndrome-disease-relevant or benign? | journal=J Am Soc Nephrol | year= 2014 | volume= 25 | issue= 9 | pages= 2053-65 | pmid=24652797 | doi=10.1681/ASN.2013070796 | pmc=4147975 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24652797  }} </ref>



Revision as of 18:22, 2 August 2018

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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

  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.
  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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