Author Huma Khan, MD Resident Physician, Department of Emergency Medicine, Northshore-Long Island Jewish Medical Center

Disclosure: Nothing to disclose. Coauthor(s) Natalie B Meirowitz, MD Assistant Professor of Obstetrics and Gynecology, Albert Einstein College of Medicine; Chief of Maternal-Fetal Medicine, Director, Center for Maternal-Fetal Health, Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Northshore-Long Island Jewish Medical Center

Natalie B Meirowitz, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists and Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose. http://emedicine.medscape.com/article/1394126-overview#showallUpdated: Jul 22, 2013

BackgroundHELLP syndrome, named for 3 features of the disease (hemolysis, elevated liver enzyme levels, and low platelet levels), is a life-threatening condition that can potentially complicate pregnancy. HELLP was once known as edema-proteinuria-hypertension gestosis type B in the early 20th century and was later renamed in 1982 by Louis Weinstein. Although the idea is controversial, some propose that HELLP is a severe form of preeclampsia, which, in turn, is defined as gestational hypertension accompanied by proteinuria after the 20th week of gestation. Others believe that HELLP syndrome is an entity of its own. Although the cause of HELLP syndrome is unknown, certain risk factors, including a maternal age of older than 34 years, multiparity, and European descent, have been described.[1, 2, 3] There is no known preventive management.PathophysiologyHELLP is a syndrome characterized by thrombocytopenia, hemolytic anemia, and liver dysfunction believed to result from microvascular endothelial activation and cell injury.The pathophysiology of HELLP syndrome is ill-defined. Some theorize that, because HELLP is a variant of preeclampsia, the pathophysiology stems from a common source. In preeclampsia, defective placental vascular remodeling during weeks 16-22 of pregnancy with the second wave of trophoblastic invasion into the decidua results in inadequate placental perfusion. The hypoxic placenta then releases various placental factors such as soluble vascular endothelial growth factor receptor-1 (sVEGFR-1), which then binds vascular endothelial growth factor (VEGF) and placental growth factor (PGF), causing endothelial cell and placental dysfunction by preventing them from binding endothelial cell receptors. The result is hypertension, proteinuria, and increased platelet activation and aggregation. Furthermore, activation of the coagulation cascade causes consumption of platelets due to adhesion onto a damaged and activated endothelium, in addition to microangiopathic hemolysis caused by shearing of erythrocytes as they traverse through capillaries laden with platelet-fibrin deposits. Multiorgan microvascular injury and hepatic necrosis causing liver dysfunction contribute to the development of HELLP.[4, 1, 5, 5, 6, 7, 8, 9] Another hypothesis proposes acute maternal immune rejection due to immunocompetent maternal cells coming into contact with a genetically distinct fetus, altering the maternal-fetal immune balance and causing endothelial dysfunction, platelet activation and aggregation, and arterial hypertension.[10] Other theories include inborn errors of fatty acid oxidative metabolism secondary to long- and medium-chain fatty acid mutations, which cause liver damage secondary to insufficient mitochondrial oxidation of fatty acids required for ketogenesis.[11, 12] Yet another theory suggests a placental-instigated acute inflammatory condition targeting the liver.[13] In addition, dysfunction in the complement system via excessive activation or defective regulation for a given amount of endothelial injury has been proposed to cause damage to hepatic vessels in HELLP.[14] Many hypotheses attempt to define the pathogenesis of HELLP syndrome, but the true pathology remains a mystery.EtiologyThe cause of HELLP syndrome is currently unknown, although theories as described in Pathophysiology have been proposed.Risk factors for HELLP syndrome include the following: Maternal age older than 34 years Multiparity White race or European descent History of poor pregnancy outcome [1, 15] EpidemiologyHELLP syndrome occurs in 0.1%-0.6% of all pregnancies and in 4%-12% of patients with preeclampsia. HELLP syndrome typically occurs between week 27 of gestation and delivery, or immediately postpartum in 15%-30% of cases.[16, 17, 18, 19] The incidence of HELLP syndrome is significantly higher in whites and women of European descent.[18] HELLP has been shown to occur in older maternal age groups, with a mean age of 25 years. In contrast, preeclampsia is most common in younger patients (mean age, 19 years).[18] PrognosisMost patients with HELLP syndrome stabilize within 24-48 hours, with the most protracted postpartum recovery time in patients with class 1 disease.[2] The recurrence rate is 2%-27% in subsequent pregnancies.[20, 21] Patients are at increased risk of preeclampsia or pregnancy-induced hypertension, in addition to preterm delivery, fetal growth restriction, and placental abruption in future pregnancies.[20, 2] Women with HELLP syndrome are also at increased risk of developing hypertension and cardiovascular disease.[7] MaternalMaternal mortality ranges from 1%-3%, with a perinatal mortality rate of 35%.[22] Class 1 or complete HELLP (see Stages) is associated with the highest incidence of perinatal morbidity and mortality. Sixty percent of deaths occur in patients with class 1 disease; cerebral hemorrhage is the most common autopsy finding.[23, 24] Morbidity includes the following: Disseminated intravascular coagulation (DIC) (20%) Placental abruption (16%) Acute renal failure (7%) Pulmonary edema (6%) [22] NeonatalFetal morbidity and mortality rates range from 9%-24%[25] and usually result from placental abruption, intrauterine asphyxia, or prematurity.[26] See Complications for a complete list of adverse maternal outcomes.Patient EducationPatients with HELLP syndrome should be educated on the risk of maternal and fetal morbidity and mortality in future pregnancies.HistoryHELLP syndrome typically occurs between 27 weeks gestation and delivery in women with a mean age of 25 years.A complete review of systems may reveal malaise, nausea, vomiting, weight gain, and various other nonspecific symptoms.A wide range of symptoms, none of which are diagnostic, may be present in persons with HELLP syndrome. For instance, nausea, vomiting, and epigastric and right upper quadrant pain has been reported in 30%-90%[18, 27, 28] of patients, headache in 33%-68%,[29, 27] visual changes in 10%-20%,[30] and jaundice in 5%.[22] In a series by Sibai et al, most patients with HELLP syndrome presented preterm with complaints of epigastric or right upper quadrant pain and nonspecific viral syndrome types of symptoms.[18] In earlier studies by Weinstein, nausea, vomiting, and epigastric pain were found to be the most common symptoms in patients with HELLP symptoms.[31, 3] Common history findings are as follows: Malaise Nausea and vomiting Edema with secondary weight gain Epigastric or right upper quadrant pain Dyspnea (if pulmonary edema present) Physical ExaminationA complete physical examination may reveal signs of dehydration, including dry mucous membranes, sunken eyes, weakness, and imbalance secondary to dizziness from excessive vomiting. Vital signs may reveal tachycardia, tachypnea, and hypertension. Patients with HELLP syndrome show various signs and symptoms, many of which are synonymous with preeclampsia. Proteinuria is shown to be present in 86%-100% of patients and hypertension in 80%.[22] However, it should be noted that 15% of patients do not present with either.[18, 16, 30] In addition, 55%-67% of patients present with nondependent edema, which can be periorbital or in the upper and lower extremities.[2] Right upper quadrant tenderness is found in 65%-90% of patients, while jaundice is evident in 5% of patients.[22] A lung examination may reveal crackles if pulmonary edema is present. Vital signs may include the following: Hypertension Tachycardia Tachypnea Generalized findings may include the following: Fatigue or weakness Distress due to pain Jaundice Head, ears, eyes, nose and throat (HEENT) findings may include the following: Signs of dehydration including sunken eyes Edema leading to puffy eyes Dry mucous membranes Pulmonary findings may include crackles secondary to noncardiogenic pulmonary edema.Abdominal findings may include right upper quadrant to epigastric tenderness.Extremities findings may include edema.ComplicationsMaternal complications of HELLP syndrome may include the following: Hematologic: DIC, bleeding, hematoma Cardiac: Cardiac arrest, myocardial ischemia Pulmonary: Pulmonary edema, respiratory failure, pulmonary embolism, adult respiratory distress syndrome (ARDS) CNS: Hemorrhage/stroke, cerebral edema, central venous thrombosis, seizures, retinal detachment Renal: Acute renal failure, chronic renal failure requiring dialysis Hepatic: Hepatic (usually subcapsular) hematoma with possible rupture, ascites, nephrogenic diabetes insipidus Infection [16, 2] Neonatal complications of HELLP syndrome may include the following: Prematurity Intrauterine growth retardation (39%) [2] Thrombocytopenia (one third of neonates born to patients with HELLP; 4% of these infants will have intraventricular hemorrhage [26] ) Imaging StudiesAlthough rare, large-vessel vasculopathy could result in hepatic infarction or subcapsular hematomas. If suspected (usually correlated with worsening hepatic function test results), CT scanning or MRI should be obtained. Hepatic ultrasonography may reveal increased echogenicity in irregular, well-demarcated areas of the liver.[20, 2] Histologic FindingsHepatic endothelial disruption and subsequent platelet activation, aggregation, and consumption lead to distal ischemia and hepatocyte death, which can be segmental or apparent diffusely throughout the liver.[32] Since HELLP tends to involve smaller terminal arterioles, characteristic histiologic features are periportal or focal parenchymal necrosis with hyaline deposits of fibrinlike material in the sinusoids.[33, 34, 35] If larger-vessel vasculopathy occurs, hepatic infarction or subcapsular hematomas may result, both of which would require imaging studies such as MRI or CT scanning.[36] StagingTwo common classifications used to predict maternal morbidity and mortality were described in and are known as the Mississippi and the Tennessee classifications. The two methods of classification, while useful, should not be regarded as a hard and fast rule. Partial HELLP syndrome, as per the Tennessee classification, can progress to the complete form. In addition, increased eclampsia and higher perinatal morbidity and mortality have been demonstrated in patients with HELLP syndrome,[37] while those with Mississippi class 3 disease have been shown to exhibit hepatic rupture.[38, 39] Mississippi classificationThe Mississippi classification divides HELLP syndrome into 3 classes based on platelet count, AST or ALT levels, and LDH levels.Class 1, which has an approximately 13% incidence of bleeding, is associated with the highest maternal morbidity and mortality rates and longest recovery time. Patients in this class have a platelet count less than 50,000/L, liver dysfunction with AST or ALT levels greater than 70 IU/L, and hemolysis as evidenced by an LDH level greater than 600 IU/L. Class 2 includes platelet counts from 50,000-100,000/L with AST, ALT, and LDH levels similar to those in class 1. Class 2 has an 8% incidence of bleeding. The mild form of HELLP, class 3, has a platelet count from 100,000-150,000/L, AST and ALT greater than 40 IU/L, and LDH greater than 600 IU/L, with no increased risk of bleeding. The more severe the class, the longer the recovery time postpartum.[23, 38] Table 2: Mississippi Classification of HELLP Syndrome (Open Table in a new window)Class 1 (Severe)Class 2 (Moderate)Class 3 (Mild)

Platelets50,000/L50,000-100,000/L100,000-150,000/L

AST or ALT70 IU/L70 IU/L40 IU/L

LDH600 IU/L600 IU/L600 IU/L

Incidence of bleeding13%8%No increased risk

Tennessee classificationThe Tennessee classification describes HELLP as either complete or partial.Complete HELLP is defined as hemolysis with an abnormal peripheral smear finding and an LDH level greater than 600 IU/L or bilirubin level greater than 1.2 mg/dL. Patients with complete HELLP have platelet counts less than 100,000/L and AST levels over 70 IU/L. Partial HELLP describes severe preeclampsia plus some features of HELLP. These features are further defined as LP, or low platelet syndrome (slightly thrombocytopenic but no hemolysis or liver dysfunction); EL, or elevated liver enzyme syndrome (mildly elevated liver enzymes but no hemolysis or thrombocytopenia); HEL syndrome (hemolysis, elevated liver enzyme levels, but no thrombocytopenia); and ELLP syndrome (elevated liver enzyme levels and low platelet counts, no hemolysis).[38] Complete HELLP syndrome is characterized by the following: Platelet count of 100,000/L or less AST or ALT levels of 70 IU/L or more LDH (or bilirubin) (with hemolysis as evidenced on abnormal peripheral smear) levels of 600 IU/L (0.2 mg/dL) or more Partial HELLP syndrome is characterized by severe preeclampsia plus one of the following: ELLP: Elevated liver enzyme levels, thrombocytopenia, no hemolysis EL: Mildly elevated liver enzyme levels, no thrombocytopenia, no hemolysis LP: Thrombocytopenia, no hemolysis, normal liver enzyme levels HEL: Hemolysis, liver dysfunction, no thrombocytopenia Laboratory StudiesLaboratory evaluation should include the following: Type and screen Complete blood cell (CBC) count: Thrombocytopenia, anemia with possible reticulocytosis Coagulation studies: Normal prothrombin time, 50% may have prolonged activated partial thromboplastin time Peripheral smear: Schistocytes, helmet cells, and burr cells secondary to microangiopathic hemolytic anemia Serum aspartate aminotransferase/alanine aminotransferase (AST/ALT) levels: Elevated secondary to liver dysfunction Lactate dehydrogenase (LDH) level: Elevated secondary to liver dysfunction or hemolysis Complete metabolic panel (CMP): Elevated blood urea nitrogen (BUN)/creatinine with acute renal failure Bilirubin level: Increased secondary to hemolysis Haptoglobin level: Decreased secondary to hemolysis Fibrinogen levels: Low secondary to increased coagulation D-dimer: Increased due to fibrinolysis/DIC Laboratory abnormalities apparent in HELLP syndrome and the recovery time postpartum required for normalization of these findings are summarized in Table 1.[22, 2] Table 1: Laboratory Findings in HELLP Syndrome (Open Table in a new window)Laboratory TestPossible ResultCauseRecovery to Baseline (in Number of Hours or Days Postpartum)

HaptoglobinHemolysis24-30 hours

LDHHemolysis or liver dysfunction3-5 days

AST or ALTLiver dysfunction3-5 days

BilirubinHemolysis-

Platelets (CBC)Consumption6-11 days

Hemoglobin/Hematocrit (CBC)Hemolysis-

PTNormal

PTTLiver dysfunction-

D-dimerIncreased coagulation and secondary fibrinolysis-

Fibrinogen-

StabilizationManagement of HELLP syndrome begins with early and immediate recognition of the diagnosis. Stabilization of the patient begins in the prehospital setting. If seizures and hypertension are present, both should be controlled en route to the hospital. Emergency Department CareEarly recognition of HELLP syndrome begins with a close look at the history, vital signs, and physical examination findings. Emergency department management includes seizure prophylaxis, hypertension control, repletion of blood products, as indicated, and general stabilization of patient condition. See section Medications for a more complete pharmacologic guide. Intravenous fluids should be given cautiously. Patients with HELLP syndrome may be volume overloaded and present with edema but are in fact intravascularly depleted. Seizure ProphylaxisIntravenous magnesium sulfate is given until 24 hours after delivery, at which point it can be stopped if the maternal condition is improved. The dosage for magnesium sulfate IV is a 6-g loading dose over 20 minutes with a 2-g per hour maintenance dose.[16, 9] Treatment of HypertensionHypertension is managed similarly to hypertension in preeclampsia. The blood pressure (BP) goal is to keep the systolic at 160 or less and the diastolic at 105 or less. Labetalol and hydralazine are the recommended drugs to treat a hypertensive crisis.[16, 9] Corticosteroid TherapyAlthough controversial, corticosteroids can be given as a treatment regimen for antepartum and postpartum management in patients with HELLP. Steroids are theorized to alter the degree of intravascular endothelial injury and prevent further hepatocyte death and platelet activation.[20] While evidence of maternal improvement is limited, studies have demonstrated improved laboratory findings, including improved platelet counts, liver function, blood pressure, and urine output with the use of high-dose dexamethasone.[27] Intravenous glucocorticoids appear superior to intramuscular steroids[40] and are dose-dependent.[41] Therefore, aggressive therapy with high-dose dexamethasone has been recommended over the standard regimens used for enhancing fetal lung maturity. Steroids are also believed to improve fetal morbidity by reducing the incidence of respiratory distress syndrome and intraventricular hemorrhage, as well as maternal morbidity.[20] Dosing for high-risk patients with severe disease (platelet count < 20,000 or CNS dysfunction): 20 mg IV dexamethasone every 6 hours for up to 4 doses Dosing for all other patients with HELLP syndrome: 10 mg IV dexamethasone every 6 hours for 2 doses then 6 mg IV dexamethasone every 6 hours for 2 doses If no clinical or laboratory improvement, expedite delivery.[20] Steroids can be initiated in the emergency department and continued on an inpatient basis.Once stabilized, the patient should be transferred to a labor and delivery unit under an obstetricians care, where she can be closely monitored. DeliveryDelivery: Delivery (either vaginal delivery or cesarean section) is indicated if HELLP syndrome occurs close to 34 weeks gestation, in the setting of fetal lung maturity, or upon evidence of significant maternal or fetal distress before 34 weeks gestation. Steroids administered antenatally may increase the platelet count so that regional anesthesia can be given. General guidelines are as follows: If at 34 weeks gestation or later and unstable, deliver immediately If at 34 weeks gestation or later and stable, consider administration of steroids; evaluate over 24-48 hours and deliver If at 24-34 weeks gestation and stable, consider administration of steroids; wait 24-48 hours and evaluate for delivery based on the maternal-fetal condition If 34 weeks gestation or earlier with evidence of maternal or fetal distress, deliver immediately[22] Cesarean versus vaginal delivery should be determined based on the following: Cervical ripening Fetal nonstress test or biophysical profile results Umbilical artery Doppler studyIf there is a preterm gestation with intrauterine growth restriction or significantly abnormal Doppler test results, cesarean section should be performed.[20] Cesarean sectionPrior to performing a cesarean section, severe thrombocytopenia should be corrected. While platelets should be transfused, caution is advised owing to increased consumption of these platelets. A recommended dose is 6-10 U of platelets with thrombocytopenia of less than 40,000/L. General anesthesia should be performed for thrombocytopenia of less than 75,000/L.[22] The type of skin incision and primary versus secondary skin closure do not affect wound complications.[42] Hematoma formation at the operative site occurs in 20% of cases. To reduce this risk, an open bladder flap is recommended, and a subfascial drain can be used for 24-48 hours.[22, 20] Postpartum CurettagePostpartum curettage lowers the mean arterial pressure and improves oliguria and thrombocytopenia.[2] Treatment of Hepatic HematomaHepatic hematoma usually manifests as a subcapsular hematoma.If it is not ruptured, it may be managed conservatively with close hemodynamic and coagulation status monitoring. Avoid any increase in intraabdominal pressure, including emesis, palpation, or trauma in transport. Serial CT scans or careful ultrasonography should guide management. Rupture most often involves the anterior superior portion of the right upper lobe. Patients may present with shoulder pain, ascites, respiratory distress, shock, or fetal demise. Immediate intervention is mandated for a ruptured hematoma. Treatment considerations are as follows: Resuscitate: Transfuse and correct any coagulopathy with fresh frozen plasma or platelets Emergent laparotomy by general or vascular surgeon: Based on extent of bleeding, may pack and drain or surgically ligate hepatic segments that are bleeding, embolize the hepatic artery to the affected hepatic segment, and loosely suture the omentum or surgical mesh to the liver; hepatic lobectomy or liver transplantation in patients with total hepatic necrosis may also be indicated in some cases[22, 2] Owing to exsanguination and coagulopathy, maternal and fetal mortality rates are close to 50%, even with treatment[22, 20] ActivityPatients should be on bedrest. If hepatic hematoma is present, the risk of trauma should be minimized.ConsultationsConsider consultations with the following: Surgeon Hematologist Renal specialistTransferMaternal and fetal conditions can deteriorate rapidly, necessitating a closely monitored unit under an experienced obstetricians care. Patients, once stabilized and if remote from term, should be transferred to a tertiary care facility, where such monitoring can be provided. Long-Term MonitoringRepeat laboratory tests every 6-12 hours until improvement noted. Monitor for remission.[20] Consider steroids for up to 2 days postpartum to prevent rebound of liver dysfunction, thrombocytopenia, and oliguria.[2] Antihypertensive AgentClass SummaryThese agents reduce blood pressure through various mechanisms.View full drug information Labetalol (Trandate) Combined alpha- and beta-adrenergic blocking agent widely used in treating hypertension during pregnancy. Not associated with mild fetal growth restriction (unlike some other beta-blockers).Intravenous and oral forms are used as an alternative to hydralazine in severe preeclampsia/eclampsia. View full drug information Hydralazine Decreases systemic resistance through direct vasodilation of arterioles.View full drug information Nifedipine (Adalat, Afeditab, Nifediac, Procardia, Procardia XL) Relaxes coronary smooth muscle and produces coronary vasodilation, which in turn improves myocardial oxygen delivery. Sublingual administration is generally safe, despite theoretical concerns. AnticonvulsantsClass SummaryMagnesium can terminate ongoing eclamptic seizures and prevent further seizures.View full drug information Magnesium sulfate Several studies have revealed that it is the drug of choice for treating eclamptic seizures. Successful in controlling seizures in >95% of cases. Agent gives physiologic advantages to fetus by increasing uterine blood flow.Inhibits the release of acetylcholine at motor endplate. In addition, magnesium has direct effect on skeletal muscle by virtue of competitive antagonistic effects with calcium.Exclusively by kidneys and has little antihypertensive effect. Effective anticonvulsant and helps prevent recurrent seizures and maintain uterine and fetal blood flow.Can be administered both IV and IM. Intravenous route is preferred over IM route because administration is controlled more easily and time to therapeutic levels is shorter. Intramuscular administration of magnesium sulfate tends to be more painful and less convenient. If IV access or close patient monitoring is unavailable, this is an effective therapy.Goals of magnesium therapy are to terminate ongoing seizures and prevent further seizures. Patient should be evaluated qh to assure that deep tendon reflexes are present, respirations are at least 12 breaths per min, and urine output is at least 100 mL during the preceding 4 h.When using magnesium sulfate IV, close monitoring of patient and fetus is necessary.Magnesium therapy usually is continued for 12-24 h following delivery and may be stopped when hypertension resolves and patient has shown adequate diuresis.Renally compromised patients should be monitored with magnesium levels, with aggressive adjustments made to facilitate levels at 6-8 mg/dL. Patients with increased urine output may need maintenance dose increased to 3 g/h to maintain therapeutic levels. Monitor patient for signs of worsening condition and magnesium toxicity.The Parkland IM protocol is as follows:Magnesium sulfate 4 g IV over 5 min, plus magnesium sulfate 10 g deep IM (3-in needle) divided in both buttocks and mixed with 1 mL 2% lidocaine. If a seizure persists more than 15 min after above dose, administer an additional 2 g of magnesium sulfate IV over 3-5 min.Magnesium sulfate 5 g IM q4h, starting 4 h later unless patellar reflexes are absent, respiratory depression occurs, or urine output is < 100 mL in the prior 4 h. Therapeutic levels are 4.8-8.4 mg/dL. With the above protocol, serum magnesium levels usually are 4-7 mg/dL in a patient with an average volume of distribution and normal renal function.Actual serum magnesium levels are monitored only in patients with symptomatic magnesium toxicity or renal compromise.Patients may have seizures while receiving magnesium sulfate. If seizure occurs in first 20 min after loading dose, the convulsion usually is short, and no additional treatment is indicated. If seizure occurs >20 min after the loading dose, an additional 2-4 g of magnesium may be administered.In adults, 60-180 mEq of potassium, 10-30 mEq of magnesium, and 10-40 mM of phosphate per day may be necessary for optimum metabolic response.Give IV/IM for seizure prophylaxis in preeclampsia. Use IV route for quicker onset of action in true eclampsia.CorticosteroidsClass SummaryThese agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli. View full drug information Dexamethasone (Baycadron, Maxidex, Ozurdex, Dexamethasone Intensol) Has many pharmacologic benefits but significant adverse effects. Stabilizes cell and lysosomal membranes, increases surfactant synthesis, increases serum vitamin A concentration, inhibits prostaglandin and proinflammatory cytokines (eg, TNF-alpha, IL-6, IL-2, and IFN-gamma). The inhibition of chemotactic factors and factors that increase capillary permeability inhibits recruitment of inflammatory cells into affected areas. Suppresses lymphocyte proliferation through direct cytolysis and inhibits mitosis. Breaks down granulocyte aggregates, and improves pulmonary microcirculation. Adverse effects are hyperglycemia, hypertension, weight loss, GI bleeding or perforation synthesis, cerebral palsy, adrenal suppression, and death. Most of the adverse effects of corticosteroids are dose-dependent or duration-dependent.Readily absorbed via the GI tract and metabolized in the liver. Inactive metabolites are excreted via the kidneys. Lacks salt-retaining property of hydrocortisone.Patients can be switched from an IV to PO regimen in a 1:1 ratio.

REFERENCES1. Rahman TM, Wendon J. Severe hepatic dysfunction in pregnancy. Q J Med. 2002;95:343:[Medline].2. Lichtman, M, Kipps T, Seligsohn U, Kaushansky K, Prchal J. Hemolytic Anemia resulting from physical Injury to Red Cells. In: Williams Hematology, Eighth Edition. 8. McGraw-Hill Companies; 2010:Chapter 50. 3. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: A severe consequence of hypertension in pregnancy. Am J Obstet Gynecol. 1982;142:159. [Medline]. 4. Lichtman, M, Kipps T, Seligsohn U, Kaushansky K, Prchal J. Thrombocytopenia. In: Williams Hematology, Eighth Edition. 8. McGraw-Hill Companies; 2010:Chapter 119. 5. Knerr I, Beinder E, Rascher W. Syncytin, a novel human endogenous retroviral gene in human placenta: Evidence for its dysregulation in preeclampsia and HELLP syndrome. Am J Obstet Gynecol. 2002;186:210. [Medline]. 6. Levine RJ, Maynard SE, Qian C, et al. Circulating angiogenic factors and the risk of preeclampsia. N Engl J Med. 2004;350:672. [Medline]. 7. Mutter WP, Karumanchi SA. Molecular mechanisms of preeclampsia. Microvasc Res. 2008;75:1. [Medline]. 8. Widmer M, Villar J, Beniani A, et al. Mapping the theories of preeclampsia and the role of angiogenic factors: A systematic review. Obstet Gynecol 109:168, 2007. Obstet Gynecol. 2007;109:168. [Medline]. 9. Semenovskaya Z, Erogul M. Pregnancy, Preeclampsia. Medscape Reference [serial online]. May 2010;Available from: emedicine.com. Accessed May 2010. Available at http://emedicine.medscape.com/article/796690-overview. 10. Zhou Y, McMaster M, Woo K, et al. Vascular endothelial growth factor ligands and receptors that regulate human cytotrophoblast survival are dysregulated in severe preeclampsia and hemolysis, elevated liver enzymes, and low platelets syndrome. Am J Pathol. 2002;160:1405-23. [Medline]. 11. Nelson J, Lewis B, Walters B. The HELLP syndrome associated with fetal medium chain acyl-CoA dehydrogenase deficiency. Journal of Inherited Metabolic Diseases. 2000;23:518-519. 12. Ibdah JA, Bennet MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. New England Journal of Medicine. 1999;340:1723-1731. 13. Strand S, Strand D et al. Placenta-derived CD59 ligand causes liver damage in hemolysis, elevated liver enzymes and low platelet count syndrome. Gastroenterology. 2004;126:849-858. 14. Fang C, Richards A et al. Advances in understanding of pathogenesis of aHUS and HELLP. BJH British Journal of Haematology. 2008;143:336-348. 15. Ohara Padden, M. HELLP Syndrome: Recognition and Perinatal Management. The Academy of Family Physicians. Sept 1, 1999;[Full Text]. 16. Martin JN Jr, Magann EF, Blake PG. Analysis of 454 pregnancies with severe preeclampsia/eclampsia/HELLP syndrome using the 3-class system of classification. Am J Obstret Gynecol 1993. 1993;168:386. 17. Martin JN Jr, Magann EF. HELLP syndrome current principles and recommended practice. Curr Obstet Med. 1996;4:129-75.18. Sibai BM, Ramamdan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal morbidity and mortality in 442 pregnancies with HELLP syndrome. Am J Obstet Gynecol. 1993;169:1000-6. 19. Ukomadu C, Greenberger N, Blumberg R, Burakoff R. Hepatic Complications of Pregnancy. In: Current Diagnosis and Treatment: Gastroenterology, Hepatology and Endoscopy. McGraw Hills and Company; 2009:Chapter 8. 20. O'Brien JM, Barton JR. Controversies with the diagnosis and management of HELLP syndrome. Clinical Obstetrics and Gynecology. June 2005;48:2:460-477. 21. Sullivan CA, Magann EF, Perry KG Jr, et al. The recurrence risk of the syndrome of hemolysis, elevated liver enzymes, and low platelets: Subsequent pregnancy outcome and long term prognosis. Am J Obstet Gynecol. 1995;172:125. 22. Barton JR, Sibai BM. Diagnosis and management of hemolysis, elevated liver enzymes, and low platelets syndrome. Clin Perinatol. 2004;31:807-33. [Medline]. 23. Magann EF, Martin JN Jr. Twelve steps to optimal management of HELLP syndrome. Clin Obstet Gynecol. 1999;42:532. [Medline]. 24. Isler CM, Rinehart CK, Terrone DA, Martin RW, Magann EF, Martin JN Jr. Maternal mortality associated with HELLP syndrome. Am J Obstet Gynecol. 1999;181:924-8. 25. Rath W, Faridi A, Dudenhausen JW. HELLP syndrome. J Perinat Med. 2000;28:249. [Medline]. 26. Harms K, Rath W, Herting E, Kuhn W. Maternal hemolysis, elevated liver enzymes, low platelet count, and neonatal outcome. Am J Perinatol. 1995;12:1:[Medline]. 27. Sibai BM. Diagnosis, controversies and management of the syndrome of hemolysis, elevated liver enzymes and low platelet count. Obstet and Gynecol. 2004;103:981-91. 28. Vigil-De Gracia P. Pregnancy complicated by pre-eclampsia-eclampsia with HELLP syndrome. Int J Gynecol Obstet. 2001;72:17-23. 29. Martin JN Jr. Rinehart K, May WL, Magann EF, Terrone DA, Blake PG. The spectrum of severe preeclampsia: comparative analysis by HELLP syndrome classification. Am J Obstet Gynecol. 1999;180:1373-84. 30. Sibai B. HELLP Syndrome. UptoDate [serial online]. 2010;Available from: uptodate.com. Available at http://www.uptodate.com/patients/content/topic.do?topicKey=~SSpiieD1u9r0YDp. 31. Weinstein L. Preeclampsia/eclampsia with hemolysis, elevated liver enzymes and thrombocytopenia. Obstet Gynecol. 1985;66:657- 60. 32. Roberts JM. Cooper DW. Pathogenesis and genetics of pre-eclampsia. Lancet. 2001;357:53-56.33. Aarnoudse JG, Houthhoff HF et al. A syndrome of liver damage and intravascular coagulation in the last trimester of normotensive pregnancy. A clinical and histopathological study. BR J Obstet Gynaecol. 1986;93:145-155. 34. Arias F, Mancilla-Jimenez R. Hepatic fibrinogen deposits in pre-eclampsia. Immunofluorescent evidence. N Engl J Med. 1976;295:578-582. 35. Barton JR, Riely CA, Adamel TA, et al. Hepatic histopathologic condition does not correlate with laboratory abnormalities in HELLP syndrome (hemolysis, elevated liver enzymes and low platelet count). Am J Obstet Gynecol. 1992;167:1538-1543. 36. Barton JR, Sibai BM. Hepatic imaging in HELLP syndrome. Am J Obstet Gynecol. 1996;174:1820-1827.37. Martin JN Jr., Magann EF, Isler CM. HELLP Syndrome: the scope of disease and treatment. In: HELLP Syndrome: the scope of disease and treatment. Belfort MA. Thornton S, Saade GR. Hypertension in Pregnancy. Oxford:Marcel Dekker; 2003:Chapter 7 p 141-88. 38. Martin JN Jr. Rose C, Briery C. Understanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child. Am J of Obst & Gyn. 2006;195:914-34. 39. Van Runnard Heimel PH, Juisjes AJM, Franx A, Koopman C, Bots ML, Bruinse HW. A randomized placebo-controlled trial of prolonged administration to patients with HELLP syndrome remote from term: maternal and neonatal complications. Am J Obstet Gynecol. 2004;191:S41. 40. Isler CM, Barrilleaux PS, Magann EF et al. A prospective randomized trial comparing the efficacy of dexamethasone and betamethasone for the treatment of antepartum HELLP syndrome. Am J Obstet Gynecol. 2001;184:1332-39. 41. O'Brien JM, Milligan DA, Barton JR. Impact of high-dose corticosteroid therapy for patients with HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome. Am J Obstet Gynecol. 2000;183:921-4. 42. Briggs R, Chari RS, Mercer B, Sibai BM. Postoperative incision complications after cesareansection in patients with antepartum syndrome of hemolysis, elevated liver enzymes, and lowplatelets (HELLP): does delayed primary closure make a difference?. Am J Obstet Gynecol. 1996;175:183-6.