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| '''For patient information, click [[HELLP syndrome (patient information)|here]]''' | | '''For patient information, click [[HELLP syndrome (patient information)|here]]''' |
| | | {{HELLP syndrome}} |
| {{DiseaseDisorder infobox | | |
| Name = HELLP syndrome |
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| ICD10 = {{ICD10|O|14|1|o|10}} |
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| ICD9 = ''Not assigned'' |
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| ICDO = |
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| Image = |
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| Caption = |
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| OMIM = |
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| MedlinePlus = 000890 |
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| eMedicineSubj = |
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| DiseasesDB = |
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| MeshID = D017359 |
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| }}
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| {{SI}}
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| {{CMG}} | | {{CMG}} |
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| ==Overview== | | ==[[HELLP syndrome overview|Overview]]== |
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| '''HELLP syndrome''' is a life-threatening [[obstetric]] complication considered by many to be a variant of [[pre-eclampsia]]. Both conditions occur during the later stages of [[pregnancy]], or sometimes after [[childbirth]].
| | ==[[HELLP syndrome historical perspective|Historical Perspective]]== |
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| HELLP is an abbreviation of the main findings:<ref name="Weinstein">{{cite journal |author=Weinstein L |title=Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy |journal=Am. J. Obstet. Gynecol. |volume=142 |issue=2 |pages=159-67 |year=1982 |pmid=7055180 |doi=}}</ref>
| | ==[[HELLP syndrome classification|Classification]]== |
| * [[Hemolysis|'''H'''emolytic anemia]]
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| * '''E'''levated [[Liver enzyme|'''L'''iver enzymes]] and
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| * [[Thrombocytopenia|'''L'''ow '''P'''latelet count]]
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| ==Historical Perspective== | | ==[[HELLP syndrome pathophysiology|Pathophysiology]]== |
| HELLP syndrome was identified as a distinct clinical entity (as opposed to severe preeclampsia) by Dr Louis Weinstein in 1982.<ref name="Weinstein"> </ref> | |
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| ==Pathophysiology== | | ==[[HELLP syndrome causes|Causes]]== |
| The exact cause of HELLP is unknown, but general activation of the coagulation cascade is considered the main underlying problem. Fibrin forms crosslinked networks in the small [[blood vessel]]s. This leads to a [[microangiopathic hemolytic anemia]]: the mesh causes destruction of [[red blood cell]]s as if they were being forced through a strainer. Additionally, [[platelet]]s are consumed. As the [[liver]] appears to be the main site of this process, downstream liver cells suffer [[ischemia]], leading to periportal necrosis. Other organs can be similarly affected. HELLP syndrome leads to a variant form of [[disseminated intravascular coagulation]] (DIC), leading to paradoxical [[hemorrhage|bleeding]], which can make emergency surgery a serious challenge.
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| ==Classification== | | ==[[HELLP syndrome differential diagnosis|Differentiating HELLP syndrome from other Diseases]]== |
| The [[platelet]] count has been found to be moderately predictive of the severity of HELLP syndrome. This system is termed the Mississippi classification.<ref>{{cite journal |author=Martin JN, Blake PG, Lowry SL, Perry KG, Files JC, Morrison JC |title=Pregnancy complicated by preeclampsia-eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet count: how rapid is postpartum recovery? |journal=Obstetrics and gynecology |volume=76 |issue=5 Pt 1 |pages=737-41 |year=1990 |pmid=2216215 |doi=}}</ref>
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| ===Class 1===
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| Severe: < 50 K
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| ===Class 2===
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| Moderately severe: Between 50 and 100 K
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| ===Class 3===
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| Mild: > 100 K
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| ==Risk Factors== | | ==[[HELLP syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
| Often, a patient who develops HELLP syndrome has already been followed up for [[pregnancy-induced hypertension]] (''gestational hypertension''), or is suspected to develop [[pre-eclampsia]] (high blood pressure and [[proteinuria]]). Up to 8% of all cases present ''after'' delivery.
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| ==Differentiating HELLP from other Disorders== | | ==[[HELLP syndrome risk factors|Risk Factors]]== |
| Rarely, post caesarean patient with HELLP may present in shock mimicking either pulmonary embolism or hemorrhage.
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| ==Epidemiology== | | ==[[HELLP syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| The incidence of HELPP is reported to be 0.2-0.6% of all pregnancies. Of women with (pre)eclampsia, 4-12% also develop signs of a "superimposed" HELLP syndrome. HELLP usually begins during the third trimester, and usually in Caucasian women over the age of 25. Rarely, cases have been reported as early as 23 weeks gestation.
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| ==Natural History, Complications, Prognosis==
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| Mortality is 7-35% and perinatal mortality of the child may be up to 40%.
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| ==Diagnosis== | | ==Diagnosis== |
| Patients who present symptoms of HELLP can be misdiagnosed in the early stages, increasing the risk of liver failure and morbidity.<ref>{{cite journal |author=Padden MO |title=HELLP syndrome: recognition and perinatal management |journal=American family physician |volume=60 |issue=3 |pages=829-36, 839 |year=1999 |pmid=10498110 |doi=}} </ref>
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| ===Symptoms===
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| There is gradual but marked onset of [[headache]]s (30%), blurred vision, [[malaise]] (90%), [[nausea]]/vomiting (30%), "band pain" around the upper [[abdomen]] (65%) and tingling in the extremities. [[Edema]] may occur but its absence does not exclude HELLP syndrome. If the patient develops a [[seizure]] or [[coma]], the condition has progressed into full-blown [[eclampsia]].
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| ===Physical Examination===
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| ====Vital Signs====
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| [[Arterial hypertension]] is a diagnostic requirement, but may be mild.
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| ====Abdomen====
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| Rupture of the liver capsule and a resultant [[hematoma]] may occur.
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| ===Laboratory Studies===
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| *[[Complete blood count]]
| | [[HELLP syndrome history and symptoms|History and Symptoms]] | [[HELLP syndrome physical examination|Physical Examination]] | [[HELLP syndrome laboratory findings|Laboratory Findings]] | [[HELLP syndrome other diagnostic studies|Other Diagnostic Studies]] |
| *[[Liver enzyme]]s
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| *[[Renal function]] and [[electrolyte]]s
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| *[[Coagulation]] studies.
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| *Often, ''[[fibrin]] degradation products'' (FDPs) are determined, which can be elevated.
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| *[[Lactate dehydrogenase]] is a marker of hemolysis and is elevated (>600 U/liter).
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| *[[Proteinuria]] is present but can be mild.
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| *A positive [[D-dimer]] test in the presence of preeclampsia has recently been reported to be predictive of patients who will develop HELLP syndrome.<ref name="pmid10498110">{{cite journal |author=Padden MO |title=HELLP syndrome: recognition and perinatal management |journal=American family physician |volume=60 |issue=3 |pages=829–36, 839 |year=1999 |pmid=10498110 |doi=}}</ref> D-dimer is a more sensitive indicator of subclinical coagulpathy and may be a positive before coagulation studies are abnormal.
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| ==Treatment== | | ==Treatment== |
| The only effective treatment is delivery of the baby. Several medications have been investigated for the treatment of HELLP syndrome, but evidence is conflicting as to whether [[magnesium sulfate]] decreases the risk of seizures and progress to eclampsia. The DIC is treated with [[fresh frozen plasma]] to replenish the coagulation proteins, and the [[anemia]] may require [[blood transfusion]]. In mild cases, [[corticosteroid]]s and [[antihypertensive]]s ([[labetalol]], [[hydralazine]], [[nifedipine]]) may be sufficient. Intravenous fluids are generally required.
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| ==See also==
| | [[HELLP syndrome medical therapy|Medical Therapy]] | [[HELLP syndrome surgery|Surgery]] | [[HELLP syndrome secondary prevention|Secondary Prevention]] | [[HELLP syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[HELLP syndrome future or investigational therapies|Future or Investigational Therapies]] |
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| *[[Acute fatty liver of pregnancy]]
| | ==Case Studies== |
| | | [[HELLP syndrome case study one|Case #1]] |
| ==References== | |
| {{reflist|2}}
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| | ==Related Chapters== |
| | *[[Acute fatty liver of pregnancy|Acute Fatty Liver of Pregnancy]] |
| | *[[Fatty liver]] |
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| | [[de:HELLP-Syndrom]] |
| | [[fr:HELLP syndrome]] |
| | [[nl:HELLP-syndroom]] |
| | [[ja:HELLP症候群]] |
| | [[no:HELLP-syndrom]] |
| | [[pl:Zespół HELLP]] |
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| [[Category:Obstetrics]] | | [[Category:Obstetrics]] |
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| [[Category:Syndromes]] | | [[Category:Syndromes]] |
| [[Category:Intensive care medicine]] | | [[Category:Intensive care medicine]] |
| | | [[Category:Disease]] |
| [[de:HELLP-Syndrom]] | |
| [[fr:HELLP syndrome]]
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| [[nl:HELLP-syndroom]]
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| [[ja:HELLP症候群]]
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| [[no:HELLP-syndrom]]
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| [[pl:Zespół HELLP]]
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