Hemolytic-uremic syndrome classification: Difference between revisions

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** 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><ref name="pmid16762990">{{cite journal| author=Fremeaux-Bacchi V, Moulton EA, Kavanagh D, Dragon-Durey MA, Blouin J, Caudy A et al.| title=Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome. | journal=J Am Soc Nephrol | year= 2006 | volume= 17 | issue= 7 | pages= 2017-25 | pmid=16762990 | doi=10.1681/ASN.2005101051 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16762990  }} </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><ref name="pmid16762990">{{cite journal| author=Fremeaux-Bacchi V, Moulton EA, Kavanagh D, Dragon-Durey MA, Blouin J, Caudy A et al.| title=Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome. | journal=J Am Soc Nephrol | year= 2006 | volume= 17 | issue= 7 | pages= 2017-25 | pmid=16762990 | doi=10.1681/ASN.2005101051 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16762990  }} </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>
** Diacylglycerol Kinase Epsilon gene mutations  
** Diacylglycerol Kinase Epsilon gene mutations<ref name="pmid26887830">{{cite journal| author=Zhu J, Chaki M, Lu D, Ren C, Wang SS, Rauhauser A et al.| title=Loss of diacylglycerol kinase epsilon in mice causes endothelial distress and impairs glomerular Cox-2 and PGE2 production. | journal=Am J Physiol Renal Physiol | year= 2016 | volume= 310 | issue= 9 | pages= F895-908 | pmid=26887830 | doi=10.1152/ajprenal.00431.2015 | pmc=4867310 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26887830  }} </ref>
==== Secondary Causes ====
* Infection
** Pneumococcal infection (commonly pneumonia, empyema, meningitis, and less commonly pericarditis, peritonitis, otitis media and bacteremia)<ref>Copelovitch, L. & Kaplan, B.S. Pediatr Nephrol (2008) 23: 1951. https://doi.org/10.1007/s00467-007-0518-y</ref>
** HIV infection
** Other Viral or Bacterial Infections
* Drug associated
** Antineoplastic, immunosuppressive and anti platelet
* Malignancy
* Pregnancy<ref name="pmid20203157">{{cite journal| author=Fakhouri F, Roumenina L, Provot F, Sallée M, Caillard S, Couzi L et al.| title=Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. | journal=J Am Soc Nephrol | year= 2010 | volume= 21 | issue= 5 | pages= 859-67 | pmid=20203157 | doi=10.1681/ASN.2009070706 | pmc=2865741 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20203157  }} </ref>
* Organ Transplantation
* Other medical conditions( Antiphospholipid syndrome, Scleroderma, Lupus)


==References==
==References==

Revision as of 18:45, 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)[1]
    • Complement Factor I(CFI) Defeciency (Acquired antibody mediated)[2][3]
    • Membrane co-factor protein Defeciency (MCP; CD46)[4][5]
    • Factor B Overactivity (Complement Factor B mutation)[6]
    • Diacylglycerol Kinase Epsilon gene mutations[7]

Secondary Causes

  • Infection
    • Pneumococcal infection (commonly pneumonia, empyema, meningitis, and less commonly pericarditis, peritonitis, otitis media and bacteremia)[8]
    • HIV infection
    • Other Viral or Bacterial Infections
  • Drug associated
    • Antineoplastic, immunosuppressive and anti platelet
  • Malignancy
  • Pregnancy[9]
  • Organ Transplantation
  • Other medical conditions( Antiphospholipid syndrome, Scleroderma, Lupus)

References

  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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