Hemolytic-uremic syndrome classification: Difference between revisions

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{{HUS}}
{{HUS}}


{{CMG}}; {{AE}} {{AHS}}
{{CMG}}; {{AE}} {{S.G.}}, {{AHS}}
==Overview==
==Overview==
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).
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).
==Classification==
==Classification==


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=== '''Typical Or Diarrhea Related''' ===
=== '''Typical Or Diarrhea Related''' ===
* Shiga-Toxin producing E.Coli/ Shigella infection
* Shiga-toxin producing [[E. Coli]]/ [[Shigella infection]]


=== Atypical or Non Diarrheal ===
=== Atypical or Non Diarrheal ===


==== Primary Causes ====
==== Primary Causes ====
* Complement Factor abnormalities
* Complement factor abnormalities
** Complement factor H (CFH) mutation/ Factor H Defeciency (Autosomal Dominant)<ref name="pmid14978182">{{cite journal| author=Dragon-Durey MA, Frémeaux-Bacchi V, Loirat C, Blouin J, Niaudet P, Deschenes G et al.| title=Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. | journal=J Am Soc Nephrol | year= 2004 | volume= 15 | issue= 3 | pages= 787-95 | pmid=14978182 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14978182  }} </ref>
** Complement factor H (CFH) mutation/ factor H deficiency ([[autosomal dominant]])<ref name="pmid14978182">{{cite journal| author=Dragon-Durey MA, Frémeaux-Bacchi V, Loirat C, Blouin J, Niaudet P, Deschenes G et al.| title=Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. | journal=J Am Soc Nephrol | year= 2004 | volume= 15 | issue= 3 | pages= 787-95 | pmid=14978182 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14978182  }} </ref>
** Complement Factor I(CFI) Defeciency (Acquired antibody mediated)<ref name="pmid15173250">{{cite journal| author=Fremeaux-Bacchi V, Dragon-Durey MA, Blouin J, Vigneau C, Kuypers D, Boudailliez B et al.| title=Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome. | journal=J Med Genet | year= 2004 | volume= 41 | issue= 6 | pages= e84 | pmid=15173250 | doi= | pmc=1735822 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15173250  }} </ref><ref name="pmid15917334">{{cite journal| author=Kavanagh D, Kemp EJ, Mayland E, Winney RJ, Duffield JS, Warwick G et al.| title=Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome. | journal=J Am Soc Nephrol | year= 2005 | volume= 16 | issue= 7 | pages= 2150-5 | pmid=15917334 | doi=10.1681/ASN.2005010103 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15917334  }} </ref>
** Complement factor I (CFI) deficiency (acquired antibody mediated)<ref name="pmid15173250">{{cite journal| author=Fremeaux-Bacchi V, Dragon-Durey MA, Blouin J, Vigneau C, Kuypers D, Boudailliez B et al.| title=Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome. | journal=J Med Genet | year= 2004 | volume= 41 | issue= 6 | pages= e84 | pmid=15173250 | doi= | pmc=1735822 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15173250  }} </ref><ref name="pmid15917334">{{cite journal| author=Kavanagh D, Kemp EJ, Mayland E, Winney RJ, Duffield JS, Warwick G et al.| title=Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome. | journal=J Am Soc Nephrol | year= 2005 | volume= 16 | issue= 7 | pages= 2150-5 | pmid=15917334 | doi=10.1681/ASN.2005010103 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15917334  }} </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>
** Membrane co-factor protein deficiency (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<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>
** 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 ====
==== Secondary Causes ====
* Infection
* 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>
** [[Pneumococcal infections|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
** [[HIV]]
** Other Viral or Bacterial Infections
** Other [[viral]] or [[bacterial infections]]
* Drug associated
* Drug associated
** Antineoplastic, immunosuppressive and anti platelet
** [[Antineoplastic]]
* Malignancy
** [[Immunosuppressive]]
* 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>
** [[Anti-platelet]]
* Organ Transplantation
* [[Malignancy]]
* Other medical conditions( Antiphospholipid syndrome, Scleroderma, Lupus)
* [[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 18:30, 20 August 2018

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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]

Overview

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).

Classification

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

Typical Or Diarrhea Related

Atypical or Non Diarrheal

Primary Causes

  • Complement factor abnormalities
    • Complement factor H (CFH) mutation/ factor H deficiency (autosomal dominant)[1]
    • Complement factor I (CFI) deficiency (acquired antibody mediated)[2][3]
    • Membrane co-factor protein deficiency (MCP, CD46)[4][5]
    • Factor B overactivity (complement factor B mutation)[6]
    • Diacylglycerol kinase epsilon gene mutations[7]

Secondary Causes

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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