|
|
| (31 intermediate revisions by 13 users not shown) |
| Line 8: |
Line 8: |
| ICDO = | | | ICDO = | |
| OMIM = 235400 | | | OMIM = 235400 | |
| MedlinePlus = | | | MedlinePlus = 000510 | |
| eMedicineSubj = ped |
| |
| eMedicineTopic = 960 |
| |
| MeshID = D006463 | | | MeshID = D006463 | |
| }} | | }} |
| {{SI}}
| | __NOTOC__ |
| {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}
| |
|
| |
|
| {{Editor Help}} | | '''For patient information click [[Hemolytic-uremic syndrome (patient information)|here]]''' |
| | {{HUS}} |
| | {{CMG}}; {{AE}}{{S.G.}}, {{AHS}} |
|
| |
|
| ==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]]).
| |
|
| |
|
| ==History== | | ==[[Hemolytic-uremic syndrome overview|Overview]]== |
| :*Described by Moschowitz in 1925 - disease with hyaline thrombi in many organs
| |
| :*Pentad from 1964 case series of 271 published cases:
| |
| ::*[[Thrombocytopenia]]
| |
| ::*Microangiopathic [[hemolytic anemia]]
| |
| ::*Neurologic symptoms and signs
| |
| ::*Renal function abnormalities
| |
| ::*[[Fever]]
| |
| :*When first described, >90% fatal
| |
| HUS first described in 1955 in 5 children with [[ARF]] who died with renal cortical necrosis
| |
|
| |
|
| ==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>
| |
|
| |
|
| 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]]== |
|
| |
|
| ==Molecular Biology== | | ==[[Hemolytic-uremic syndrome pathophysiology |Pathophysiology]]== |
| :*Platelet-rich thrombi in affected organs (unclear etiology of tissue specificity CD36)
| |
| :*vWF (endothelially synthesized) -> ULvWf multimers -> shear stress unfolds and causes massive platelet aggregation
| |
| ::*Normally, UlvWf digested by metalloprotease to “normal” size vWf multimers
| |
| ::*Familial forms of TTP lack metalloprotease activity
| |
| ::*Acquired forms of TTP have IgG antibody, which reduce metalloprotease activity during flares
| |
| Metalloprotease activity appears normal in HUS
| |
|
| |
|
| :*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)
| |
| ::*Toxins (i.e. Shiga toxin)
| |
| ::*PAI – 1
| |
| ::*Other genetic factors (Factor H, Factor I deficiencies, complement derangements)
| |
|
| |
|
| ==Pathophysiology and Etiology== | | ==[[Hemolytic-uremic syndrome differential diagnosis|Differentiating Hemolytic-uremic syndrome from other Diseases]]== |
| :*Childhood HUS
| |
| ::*After enterohemorrhagic [[E. Coli]] (usually O157:H7)
| |
| ::*Idiopathic
| |
| :*Adult TTP-HUS
| |
| ::*Idiopathic
| |
| ::*Drug toxicity
| |
| :::*[[Mitomycin C]], [[cyclosporine]]
| |
| :*Immune mediated:
| |
| ::*[[Quinine]]
| |
| ::*[[Ticlopidine]]
| |
| ::*[[Clopidogrel]]
| |
| ::*[[Bleomycin]]
| |
| ::*[[Cisplatin]]
| |
| ::*OCPs
| |
| ::*[[Tacrolimus]]
| |
| ::*[[Valacyclovir]]
| |
| ::*[[Rifampin]]
| |
| ::[[Simvastatin]]
| |
| ::*[[Penicillin]]
| |
| ::*[[Quinolone]]s
| |
| ::*[[Risperidone]]
| |
| :*MDS
| |
| :*Conditioning for BMT
| |
| :*[[Pregnancy]] or postpartum
| |
| :*Autoimmune disease (APL syndrome, [[SLE]], [[scleroderma]])
| |
| :*[[HIV]]/[[AIDS]]
| |
| :*[[CMV]]
| |
| :*[[Tuberculosis]]
| |
| :*After enterohemorrhagic [[E. Coli]]
| |
|
| |
|
| ==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.
| |
|
| |
|
| ==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.
| |
|
| |
|
| HUS occurs after 2-7% of all ''E. coli'' O157:H7 infections.
| | ==[[Hemolytic-uremic syndrome screening|Screening]]== |
|
| |
|
| 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]]== |
|
| |
|
| 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]] |
|
| |
|
| ==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]] |
| | |
| In severe cases or when there is diagnostic uncertainty between HUS and [[Thrombotic thrombocytopenic purpura|TTP]], [[plasmapheresis]] is the treatment of choice.
| |
|
| |
|
| 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]] |
|
| |
|
| :*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)
| |
| :*Average 7-16 exchanges required to induce remission
| |
| :*Caution plasmapheresis-associated thrombocytopenia (more with certain instruments)
| |
| :*Cryopoor plasma exchange not better than regular FFP
| |
| | |
| == Future or Investigational Therapies == | |
| :*[[ASA]] and [[dipyridamole]] not effective alone, ? benefit when added to plasma exchange
| |
| :*In poorly responsive or resistant disease, INCREASE PLASMA EXCHANGE
| |
| :*Then consider:
| |
| ::* [[prednisone]] (1 mg/kg per day)
| |
| ::* [[methylprednisolone]] (125 mg IV bid)
| |
| ::*[[Vincristine]]
| |
| ::*IVIG
| |
| | |
| ==Prognosis==
| |
| With aggressive treatment > 90% survive acute phase. About 9% may develop end stage renal disease.
| |
| 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>
| |
| | |
| ==References==
| |
| {{Reflist|2}}
| |
| | |
| ==See also==
| |
| * [[Escherichia coli O157:H7|''E. coli'' O157:H7]] | | * [[Escherichia coli O157:H7|''E. coli'' O157:H7]] |
| * [[Shigellosis]] | | * [[Shigellosis]] |
| Line 129: |
Line 55: |
|
| |
|
| {{Hematology}} | | {{Hematology}} |
| {{SIB}}
| |
|
| |
| [[Category:Disease state]]
| |
| [[Category:Nephrology]]
| |
| [[Category:Hematology]]
| |
| [[Category:Mature chapter]]
| |
| [[Category:Syndromes]]
| |
|
| |
|
| [[de:Hämolytisch-urämisches Syndrom]] | | [[de:Hämolytisch-urämisches Syndrom]] |
| Line 147: |
Line 66: |
| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |
| | |
| | [[Category:Nephrology]] |
| | [[Category:Hematology]] |