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Hemolytic–Uremic Syndrome

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Hemolytic-uremic syndrome (or haemolytic-uraemic syndrome), abbreviated HUS, is a disease characterized by hemolytic anemia, acute renal failure (uremia) and a low platelet count (thrombocytopenia). It predominantly but not exclusively affects children. Most cases are preceded by an episode of diarrhea caused by E. coli O157:H7, which is acquired as a foodborne illness. It is a medical emergency and carries a 5–10% mortality; of the remainder, the majority recover without major consequences but a small proportion develop chronic kidney disease and become reliant on renal replacement therapy.[1] HUS was first defined as a syndrome in 1955.[2][3]

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<h2>Signs and symptoms</h2>

<h3>In children</h3>

In the classical form (90% of cases), the STEC toxin enters the bloodstream and causes damage to the body's vascular endothelium. This is especially damaging to the kidney, where the toxin attaches to the glomerular endothelium and initiates a noninflammatory reaction leading to acute renal failure. Moreover, the generalized endothelial damage leads to platelet activation that causes thrombocytopenia (low platelet count). The renal glomerular endothelial cells express a receptor for the toxin. [6]

Shiga-like toxin 2 (Stx2) from Escherichia coli O157:H7. From PDB 1R4P.</div>

</div>

<li id="cite_note-pmid11691946-0">^ <a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid11691946_0-0">a</a> <a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid11691946_0-1">b</a> Corrigan JJ, Boineau FG (November 2001). "Hemolytic-uremic syndrome". Pediatr Rev 22 (11): 365–9. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/11691946~~" rel="nofollow~~">11691946</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Hemolytic-uremic+syndrome&rft.jtitle=Pediatr+Rev&rft.aulast=Corrigan+JJ%2C+Boineau+FG&rft.au=Corrigan+JJ%2C+Boineau+FG&rft.date=November+2001&rft.volume=22&rft.issue=11&rft.pages=365%E2%80%939&rft_id=info:pmid/11691946&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li> <li id="cite_note-pmid1816053-1"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid1816053_1-0">^</a> Anagnou NP, Papanicolaou N, Fessas P (1991). "Recurrent attacks of hemolytic uremic syndrome". Haematologia (Budap) 24 (2): 101–5. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/1816053~~" rel="nofollow~~">1816053</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Recurrent+attacks+of+hemolytic+uremic+syndrome&rft.jtitle=Haematologia+%28Budap%29&rft.aulast=Anagnou+NP%2C+Papanicolaou+N%2C+Fessas+P&rft.au=Anagnou+NP%2C+Papanicolaou+N%2C+Fessas+P&rft.date=1991&rft.volume=24&rft.issue=2&rft.pages=101%E2%80%935&rft_id=info:pmid/1816053&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li> <li id="cite_note-pmid13274004-2"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid13274004_2-0">^</a> GAsser C, Gautier E, Steck A, Siebenmann RE, Oechslin R (September 1955). "Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia" (in German). Schweiz Med Wochenschr 85 (38-39): 905–9. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/13274004~~" rel="nofollow~~">13274004</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Hemolytic-uremic+syndrome%3A++bilateral+necrosis+of+the+renal+cortex+in+acute+acquired+hemolytic+anemia&rft.jtitle=Schweiz+Med+Wochenschr&rft.aulast=GAsser+C%2C+Gautier+E%2C+Steck+A%2C+Siebenmann+RE%2C+Oechslin+R&rft.au=GAsser+C%2C+Gautier+E%2C+Steck+A%2C+Siebenmann+RE%2C+Oechslin+R&rft.date=September+1955&rft.volume=85&rft.issue=38-39&rft.pages=905%E2%80%939&rft_id=info:pmid/13274004&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li>

<li id="cite_note-ReferenceA-3">^ <a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-ReferenceA_3-0">a</a> <a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-ReferenceA_3-1">b</a> Robbins Basic Pathology, 7th edition</li>

<li id="cite_note-4"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-4">^</a> Kumar et al., Robbins Basic Pathology, 8th Edition; <a class="internal mw-magiclink-isbn" href="http://en.wikipedia.org/wiki/Special:BookSources/9781416029731">ISBN 978-1-4160-2973-1</a></li>

<li id="cite_note-5"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-5">^</a> Kumar et al., Robbins Basic Pathology, 8th Edition; <a class="internal mw-magiclink-isbn" href="http://en.wikipedia.org/wiki/Special:BookSources/9781416029731">ISBN 978-1-4160-2973-1</a></li>

<li id="cite_note-pmid15661753-6"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid15661753_6-0">^</a> Esparza-Gordillo J, Goicoechea de Jorge E, Buil A, et al. (March 2005). <a class="external text" rel="nofollow" href="http://hmg.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=15661753~~" rel="nofollow~~">"Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32"</a>. Hum. Mol. Genet. 14 (5): 703–12. <a title="Digital object identifier" href="http://en.wikipedia.org/wiki/Digital_object_identifier">doi</a>:<a class="external text" rel="nofollow" href="http://dx.doi.org/10.1093%2Fhmg%2Fddi066~~" rel="nofollow~~">10.1093/hmg/ddi066</a>. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/15661753~~" rel="nofollow~~">15661753</a>. <a class="external free" rel="nofollow" href="http://hmg.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=15661753~~" rel="nofollow~~">http://hmg.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=15661753</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Predisposition+to+atypical+hemolytic+uremic+syndrome+involves+the+concurrence+of+different+susceptibility+alleles+in+the+regulators+of+complement+activation+gene+cluster+in+1q32&rft.jtitle=Hum.+Mol.+Genet.&rft.aulast=Esparza-Gordillo+J%2C+Goicoechea+de+Jorge+E%2C+Buil+A%2C+%27%27et+al.%27%27&rft.au=Esparza-Gordillo+J%2C+Goicoechea+de+Jorge+E%2C+Buil+A%2C+%27%27et+al.%27%27&rft.date=March+2005&rft.volume=14&rft.issue=5&rft.pages=703%E2%80%9312&rft_id=info:doi/10.1093%2Fhmg%2Fddi066&rft_id=info:pmid/15661753&rft_id=http%3A%2F%2Fhmg.oxfordjournals.org%2Fcgi%2Fpmidlookup%3Fview%3Dlong%26pmid%3D15661753&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li> <li id="cite_note-pmid12544708-7"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid12544708_7-0">^</a> Coppo P, Bussel A, Charrier S, et al. (January 2003). <a class="external text" rel="nofollow" href="http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0025-7974&volume=82&issue=1&spage=27~~" rel="nofollow~~">"High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome"</a>. Medicine (Baltimore) 82 (1): 27–38. <a title="Digital object identifier" href="http://en.wikipedia.org/wiki/Digital_object_identifier">doi</a>:<a class="external text" rel="nofollow" href="http://dx.doi.org/10.1097%2F00005792-200301000-00003~~" rel="nofollow~~">10.1097/00005792-200301000-00003</a>. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/12544708~~" rel="nofollow~~">12544708</a>. <a class="external free" rel="nofollow" href="http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0025-7974&volume=82&issue=1&spage=27~~" rel="nofollow~~">http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0025-7974&volume=82&issue=1&spage=27</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=High-dose+plasma+infusion+versus+plasma+exchange+as+early+treatment+of+thrombotic+thrombocytopenic+purpura%2Fhemolytic-uremic+syndrome&rft.jtitle=Medicine+%28Baltimore%29&rft.aulast=Coppo+P%2C+Bussel+A%2C+Charrier+S%2C+%27%27et+al.%27%27&rft.au=Coppo+P%2C+Bussel+A%2C+Charrier+S%2C+%27%27et+al.%27%27&rft.date=January+2003&rft.volume=82&rft.issue=1&rft.pages=27%E2%80%9338&rft_id=info:doi/10.1097%2F00005792-200301000-00003&rft_id=info:pmid/12544708&rft_id=http%3A%2F%2Fmeta.wkhealth.com%2Fpt%2Fpt-core%2Ftemplate-journal%2Flwwgateway%2Fmedia%2Flandingpage.htm%3Fissn%3D0025-7974%26volume%3D82%26issue%3D1%26spage%3D27&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li> <li id="cite_note-pmid11604563-8"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid11604563_8-0">^</a> Medina PJ, Sipols JM, George JN (September 2001). <a class="external text" ~~href~~rel="nofollow" href="http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=1065-6251&volume=8&issue=5&spage=286~~" rel="nofollow~~">"Drug-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome"</a>. Curr. Opin. Hematol. 8 (5): 286–93. <a title="Digital object identifier" href="http://en.wikipedia.org/wiki/Digital_object_identifier">doi</a>:<a class="external text" rel="nofollow" href="http://dx.doi.org/10.1097%2F00062752-200109000-00004~~" rel="nofollow~~">10.1097/00062752-200109000-00004</a>. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/11604563~~" rel="nofollow~~">11604563</a>. <a class="external free" rel="nofollow" href="http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=1065-6251&volume=8&issue=5&spage=286~~" rel="nofollow~~">http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=1065-6251&volume=8&issue=5&spage=286</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Drug-associated+thrombotic+thrombocytopenic+purpura-hemolytic+uremic+syndrome&rft.jtitle=Curr.+Opin.+Hematol.&rft.aulast=Medina+PJ%2C+Sipols+JM%2C+George+JN&rft.au=Medina+PJ%2C+Sipols+JM%2C+George+JN&rft.date=September+2001&rft.volume=8&rft.issue=5&rft.pages=286%E2%80%9393&rft_id=info:doi/10.1097%2F00062752-200109000-00004&rft_id=info:pmid/11604563&rft_id=http%3A%2F%2Fmeta.wkhealth.com%2Fpt%2Fpt-core%2Ftemplate-journal%2Flwwgateway%2Fmedia%2Flandingpage.htm%3Fissn%3D1065-6251%26volume%3D8%26issue%3D5%26spage%3D286&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li> <li id="cite_note-pmid12823037-9"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid12823037_9-0">^</a> Hosler GA, Cusumano AM, Hutchins GM (July 2003). <a class="external text" rel="nofollow" href="http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=127&page=834~~" rel="nofollow~~">"Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases"</a>. Arch. Pathol. Lab. Med. 127 (7): 834–9. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/12823037~~" rel="nofollow~~">12823037</a>. <a class="external free" rel="nofollow" href="http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=127&page=834~~" rel="nofollow~~">http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=127&page=834</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Thrombotic+thrombocytopenic+purpura+and+hemolytic+uremic+syndrome+are+distinct+pathologic+entities.+A+review+of+56+autopsy+cases&rft.jtitle=Arch.+Pathol.+Lab.+Med.&rft.aulast=Hosler+GA%2C+Cusumano+AM%2C+Hutchins+GM&rft.au=Hosler+GA%2C+Cusumano+AM%2C+Hutchins+GM&rft.date=July+2003&rft.volume=127&rft.issue=7&rft.pages=834%E2%80%939&rft_id=info:pmid/12823037&rft_id=http%3A%2F%2Fjournals.allenpress.com%2Fjrnlserv%2F%3Frequest%3Dget-abstract%26issn%3D0003-9985%26volume%3D127%26page%3D834&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li> <li id="cite_note-10"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-10">^</a> <a class="external text" rel="nofollow" href="http://www.emedicine.com/EMERG/topic238.htm~~" rel="nofollow~~">eMedicine - Hemolytic Uremic Syndrome : Article by William Shapiro</a></li> <li id="cite_note-pmid16918877-11"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-pmid16918877_11-0">^</a> Panos GZ, Betsi GI, Falagas ME (September 2006). <a class="external text" rel="nofollow" href="http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0269-2813&date=2006&volume=24&issue=5&spage=731~~" rel="nofollow~~">"Systematic review: are antibiotics detrimental or beneficial for the treatment of patients with Escherichia coli O157:H7 infection?"</a>. Aliment. Pharmacol. Ther. 24 (5): 731–42. <a title="Digital object identifier" href="http://en.wikipedia.org/wiki/Digital_object_identifier">doi</a>:<a class="external text" rel="nofollow" href="http://dx.doi.org/10.1111%2Fj.1365-2036.2006.03036.x~~" rel="nofollow~~">10.1111/j.1365-2036.2006.03036.x</a>. <a class="mw-redirect" title="PubMed Identifier" href="http://en.wikipedia.org/wiki/PubMed_Identifier">PMID</a> <a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/pubmed/16918877~~" rel="nofollow~~">16918877</a>. <a class="external free" rel="nofollow" href="http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0269-2813&date=2006&volume=24&issue=5&spage=731~~" rel="nofollow~~">http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0269-2813&date=2006&volume=24&issue=5&spage=731</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Systematic+review%3A+are+antibiotics+detrimental+or+beneficial+for+the+treatment+of+patients+with+Escherichia+coli+O157%3AH7+infection%3F&rft.jtitle=Aliment.+Pharmacol.+Ther.&rft.aulast=Panos+GZ%2C+Betsi+GI%2C+Falagas+ME&rft.au=Panos+GZ%2C+Betsi+GI%2C+Falagas+ME&rft.date=September+2006&rft.volume=24&rft.issue=5&rft.pages=731%E2%80%9342&rft_id=info:doi/10.1111%2Fj.1365-2036.2006.03036.x&rft_id=info:pmid/16918877&rft_id=http%3A%2F%2Fwww.blackwell-synergy.com%2Fopenurl%3Fgenre%3Darticle%26sid%3Dnlm%3Apubmed%26issn%3D0269-2813%26date%3D2006%26volume%3D24%26issue%3D5%26spage%3D731&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li>

<li id="cite_note-12"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-12">^</a> Chu P, Hemphill RR (2004). "222: Acuired hemolytic anemia". In Tintinalli JE, Kelen GD, Stapczynski JS. Emergency Medicine: A Comprehensive Study Guide (6th ed.). New York, NY: McGraw-Hill. <a title="International Standard Book Number" href="http://en.wikipedia.org/wiki/International_Standard_Book_Number">ISBN</a> <a title="Special:BookSources/0-07-138875-3" href="http://en.wikipedia.org/wiki/Special:BookSources/0-07-138875-3">0-07-138875-3</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=bookitem&rft.btitle=222%3A+Acuired+hemolytic+anemia&rft.atitle=Emergency+Medicine%3A+A+Comprehensive+Study+Guide&rft.aulast=Chu+P%2C+Hemphill+RR&rft.au=Chu+P%2C+Hemphill+RR&rft.date=2004&rft.edition=6th&rft.place=New+York%2C+NY&rft.pub=McGraw-Hill&rft.isbn=0-07-138875-3&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li>

<li id="cite_note-13"><a href="http://en.wikipedia.org/wiki/Hemolytic-uremic_syndrome#cite_ref-13">^</a> <a class="external text" rel="nofollow" href="http://www.guardian.co.uk/world/2011/jun/02/e-coli-outbreak-uk-cases~~" rel="nofollow~~">"E coli outbreak: three UK cases have rare strain"</a>. The Guardian. 2 June 2011. <a class="external free" rel="nofollow" href="http://www.guardian.co.uk/world/2011/jun/02/e-coli-outbreak-uk-cases~~" rel="nofollow~~">http://www.guardian.co.uk/world/2011/jun/02/e-coli-outbreak-uk-cases</a>.<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=E+coli+outbreak%3A+three+UK+cases+have+rare+strain&rft.jtitle=The+Guardian&rft.date=2+June+2011&rft_id=http%3A%2F%2Fwww.guardian.co.uk%2Fworld%2F2011%2Fjun%2F02%2Fe-coli-outbreak-uk-cases&rfr_id=info:sid/en.wikipedia.org:Hemolytic-uremic_syndrome"> </li>

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<h2>External links</h2>

<ul>

<li><a class="external text" rel="nofollow" href="http://www.ncbi.nlm.nih.gov/books/NBK1367/~~" rel="nofollow~~">GeneReviews/NCBI/NIH/UW entry on Atypical hemolytic-uremic syndrome</a></li>

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Hemolytic–Uremic Syndrome

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