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| ICDO = | | | ICDO = | |
| OMIM = 235400 | | | OMIM = 235400 | |
| MedlinePlus = | | | MedlinePlus = 000510 | |
| MeshID = D006463 | | | MeshID = D006463 | |
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| {{SI}}
| | __NOTOC__ |
| {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}
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| {{Editor Help}} | | '''For patient information click [[Hemolytic-uremic syndrome (patient information)|here]]''' |
| | {{HUS}} |
| | {{CMG}}; {{AE}}{{S.G.}}, {{AHS}} |
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| ==Overview==
| | {{SK}}HUS |
| In [[medicine]], '''hemolytic-uremic syndrome''' (or '''haemolytic-uraemic syndrome''', abbreviated HUS) is a disease characterized by [[microangiopathic hemolytic anemia]], acute [[renal failure]] and a low [[platelet]] count ([[thrombocytopenia]]).
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| ==History== | | ==[[Hemolytic-uremic syndrome overview|Overview]]== |
| :*Described by Moschowitz in 1925 - disease with hyaline thrombi in many organs
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| :*Pentad from 1964 case series of 271 published cases:
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| ::*[[Thrombocytopenia]]
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| ::*Microangiopathic [[hemolytic anemia]]
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| ::*Neurologic symptoms and signs
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| ::*Renal function abnormalities
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| ::*[[Fever]]
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| :*When first described, >90% fatal
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| HUS first described in 1955 in 5 children with [[ARF]] who died with renal cortical necrosis
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| ==Epidemiology== | | ==[[Hemolytic-uremic syndrome historical perspective|Historical Perspective]]== |
| HUS has a peak incidence between 6 months and 4 years of age.<ref>{{cite journal | author=Corrigan JJ Jr, Boineau FG | title=Hemolytic-uremic syndrome | journal=Pediatr Rev | year=2001 | pages=365-9 | volume=22 | issue=11 | pmid= 11691946}} </ref>
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| HUS and the ''[[E. coli]]'' infections which caused it have been the source of much negative publicity for the [[Food and Drug Administration]] ([[FDA]]), meat industries, and fast-food restaurants since the 1990's, especially in the Jack in the Box contaminations. It was also featured in the Robin Cook novel ''Toxin''. In 2006, an epidemic of harmful ''[[E. coli]]'' emerged in the United States due to contaminated spinach. 183 known cases have been reported, including 29 cases of HUS.
| | ==[[Hemolytic-uremic syndrome classification|Classification]]== |
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| ==Molecular Biology== | | ==[[Hemolytic-uremic syndrome pathophysiology |Pathophysiology]]== |
| :*Platelet-rich thrombi in affected organs (unclear etiology of tissue specificity CD36)
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| :*vWF (endothelially synthesized) -> ULvWf multimers -> shear stress unfolds and causes massive platelet aggregation
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| ::*Normally, UlvWf digested by metalloprotease to “normal” size vWf multimers
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| ::*Familial forms of TTP lack metalloprotease activity
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| ::*Acquired forms of TTP have IgG antibody, which reduce metalloprotease activity during flares
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| Metalloprotease activity appears normal in HUS
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| :*In HUS, and in cases of TTP without decreased metalloprotease activity, other etiologies of platelet activation have been proposed:
| | ==[[Hemolytic-uremic syndrome causes|Causes]]== |
| ::*Endothelial injury (esp. drug induced)
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| ::*Toxins (i.e. Shiga toxin)
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| ::*PAI – 1
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| ::*Other genetic factors (Factor H, Factor I deficiencies, complement derangements)
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| ==Pathophysiology and Etiology== | | ==[[Hemolytic-uremic syndrome differential diagnosis|Differentiating Hemolytic-uremic syndrome from other Diseases]]== |
| :*Childhood HUS
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| ::*After enterohemorrhagic [[E. Coli]] (usually O157:H7)
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| ::*Idiopathic
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| :*Adult TTP-HUS
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| ::*Idiopathic
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| ::*Drug toxicity
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| :::*[[Mitomycin C]], [[cyclosporine]]
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| :*Immune mediated:
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| ::*[[Quinine]]
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| ::*[[Ticlopidine]]
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| ::*[[Clopidogrel]]
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| ::*[[Bleomycin]]
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| ::*[[Cisplatin]]
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| ::*OCPs
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| ::*[[Tacrolimus]]
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| ::*[[Valacyclovir]]
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| ::*[[Rifampin]]
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| ::[[Simvastatin]]
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| ::*[[Penicillin]]
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| ::*[[Quinolone]]s
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| ::*[[Risperidone]]
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| :*MDS
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| :*Conditioning for BMT
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| :*[[Pregnancy]] or postpartum
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| :*Autoimmune disease (APL syndrome, [[SLE]], [[scleroderma]])
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| :*[[HIV]]/[[AIDS]]
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| :*[[CMV]]
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| :*[[Tuberculosis]]
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| :*After enterohemorrhagic [[E. Coli]]
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| ==Diagnosis== | | ==[[Hemolytic-uremic syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
| Clinically, HUS can be very hard to distinguish from [[thrombotic thrombocytopenic purpura]] (TTP). The [[laboratory]] features are almost identical, and not every case of HUS is preceded by diarrhea. The only distinguishing feature is that in TTP, [[neurology|neurological]] symptoms occur more often, but this is not always the case.
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| ==Signs and symptoms== | | ==[[Hemolytic-uremic syndrome risk factors|Risk Factors]]== |
| The classic childhood case of HUS occurs after bloody [[diarrhea]] caused by [[Escherichia coli O157:H7|''E. coli'' O157:H7]], a strain of ''[[E. coli]]'' that expresses [[verotoxin]] (also called [[Shiga toxin]]). The toxin enters the bloodstream, attaches to [[kidney|renal]] [[endothelium]] and initiates an [[inflammation|inflammatory]] reaction leading to [[acute renal failure]] (ARF) and [[disseminated intravascular coagulation]] (DIC). The [[fibrin]] mesh destroys [[red blood cell]]s and captures [[thrombocytes]], leading to a decrease of both on [[complete blood count]]. The usual age of onset is between 2 and adolescence.
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| HUS occurs after 2-7% of all ''E. coli'' O157:H7 infections.
| | ==[[Hemolytic-uremic syndrome screening|Screening]]== |
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| Adult HUS has similar symptoms and pathology but is an uncommon outcome of the following: [[HIV]]; antiphospholipid syndrome (associated with [[Lupus erythematosus]] and generalized hypercoagulability); post partum renal failure; [[malignant hypertension]]; [[scleroderma]]; and cancer [[chemotherapy]] (mitomycin, cyclosporine, cisplatin and bleomycin).
| | ==[[Hemolytic-uremic syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| A third category is referred to as familial HUS. It represents 5-10% of HUS cases and is largely due to mutations in the complement proteins [[factor H]], [[membrane cofactor protein]] and [[factor I]] leading to uncontrolled [[complement system]] activation. Recurrent thromboses result in a high mortality rate.
| | ==Diagnosis== |
| | [[Hemolytic-uremic syndrome diagnostic study of choice|Diagnostic Study of Choice]] |[[Hemolytic-uremic syndrome history and symptoms|History and Symptoms]] | [[Hemolytic-uremic syndrome physical examination|Physical Examination]] | [[Hemolytic-uremic syndrome laboratory findings|Laboratory Findings]] | [[Hemolytic-uremic syndrome electrocardiogram|Electrocardiogram]] | [[Hemolytic-uremic syndrome x ray|X-ray]] | [[Hemolytic-uremic syndrome echocardiography and ultrasound|Echocardiography and Ultrasound]] | |
| | [[Hemolytic-uremic syndrome CT scan|CT scan]] | [[Hemolytic-uremic syndrome MRI|MRI]] | [[Hemolytic-uremic syndrome imaging findings|Other Imaging Findings]] | [[Hemolytic-uremic syndrome other diagnostic studies|Other Diagnostic Studies]] |
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| ==Treatment== | | ==Treatment== |
| Treatment is generally supportive with [[dialysis]] as needed. [[Platelet]] [[blood transfusion|transfusion]] may actually worsen outcome.
| | [[Hemolytic-uremic syndrome medical therapy|Medical Therapy]] | [[Hemolytic-uremic syndrome surgery|Surgery]] | [[Hemolytic-uremic syndrome primary prevention|Primary Prevention]] | [[Hemolytic-uremic syndrome secondary prevention|Secondary Prevention]] |[[Hemolytic-uremic syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Hemolytic-uremic syndrome future or investigational therapies|Future or Investigational Therapies]] |
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| In severe cases or when there is diagnostic uncertainty between HUS and [[Thrombotic thrombocytopenic purpura|TTP]], [[plasmapheresis]] is the treatment of choice.
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| Antibiotic treatment of O157:H7 colitis may stimulate further verotoxin production and thereby increase the risk of HUS.<ref>http://www.emedicine.com/EMERG/topic238.htm</ref>
| | ==Case Studies== |
| | [[Hemolytic-uremic syndrome case study one|Case #1]] |
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| :*Plasma exchange daily until LDH normal and platelets stable
| | ==Related chapters== |
| :*Renal pathology may not entirely resolve (no data on continued plasma exchange after plts and markers of hemolysis have resolved)
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| :*Average 7-16 exchanges required to induce remission
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| :*Caution plasmapheresis-associated thrombocytopenia (more with certain instruments)
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| :*Cryopoor plasma exchange not better than regular FFP
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| == Future or Investigational Therapies == | |
| :*[[ASA]] and [[dipyridamole]] not effective alone, ? benefit when added to plasma exchange
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| :*In poorly responsive or resistant disease, INCREASE PLASMA EXCHANGE
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| :*Then consider:
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| ::* [[prednisone]] (1 mg/kg per day)
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| ::* [[methylprednisolone]] (125 mg IV bid)
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| ::*[[Vincristine]]
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| ::*IVIG
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| ==Prognosis==
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| With aggressive treatment > 90% survive acute phase. About 9% may develop end stage renal disease.
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| About one-third of persons with ''hemolytic-uremic syndrome'' have abnormal kidney function many years later, and a few require long-term [[dialysis]]. Another 8% of persons with hemolytic uremic syndrome have other lifelong complications, such as [[high blood pressure]], [[seizure]]s, [[blindness]], [[paralysis]], and the effects of having part of their [[Colon (anatomy)|colon]] removed. The overall mortality rate from HUS is 5-15%. Older children and adults have a worse prognosis.<ref>{{cite book | author = Chu P, Hemphill RR | year = 2004 | title = Emergency Medicine: A Comprehensive Study Guide | chapter = 222: Acuired hemolytic anemia | editor = Tintinalli JE, Kelen GD, Stapczynski JS | edition = 6th Edition | publisher = McGraw-Hill | location = New York, NY}} ISBN 0-07-138875-3</ref>
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| ==References==
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| {{Reflist|2}}
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| ==See also==
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| * [[Escherichia coli O157:H7|''E. coli'' O157:H7]] | | * [[Escherichia coli O157:H7|''E. coli'' O157:H7]] |
| * [[Shigellosis]] | | * [[Shigellosis]] |
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| {{Hematology}} | | {{Hematology}} |
| {{SIB}}
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| [[Category:Disease state]]
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| [[Category:Nephrology]]
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| [[Category:Hematology]]
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| [[Category:Mature chapter]]
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| [[Category:Syndromes]]
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| [[de:Hämolytisch-urämisches Syndrom]] | | [[de:Hämolytisch-urämisches Syndrom]] |
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