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    HELLP Syndrome

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    Jan.23.2024

    HELLP Syndrome

    Synopsis

    Key Points

    • HELLP syndrome is a rare, potentially life-threatening obstetric complication characterized by hemolysis, elevated liver enzyme concentrations, and low platelet count
    • Widely considered to represent a severe form of severe preeclampsia; however, relationship between disorders is controversial, and some consider HELLP syndrome a separate disorder
    • Typically develops in the third trimester of pregnancy or early postpartum period and, in most cases, is preceded by hypertension and proteinuria
    • Presentation can be variable and symptoms are often nonspecific; right upper quadrant or epigastric pain, nausea, vomiting, and headache are common
    • Diagnosis is based on presence of microangiopathic hemolytic anemia on peripheral blood smear; elevated liver enzyme concentrations, specifically AST more than 2 times upper reference range for local laboratory (usually 70 units/L or higherr2); and thrombocytopenia (typical cutoff is less than 100,000/mm³); usually in conjunction with hypertension and proteinuria r1
    • Delivery is the only effective method of treatment; timing depends on gestational age and patient and fetal condition
    • Immediate delivery after maternal stabilization is indicated in pregnancies at 34 weeks of gestation or longer, less than 23 to 24 weeks gestation, and between 23 and 34 weeks of gestation if there is disseminated intravascular coagulation, liver infarction or hemorrhage, renal failure, pulmonary edema, suspected abruptio placentae, or nonreassuring fetal status r1
    • Delivery can be delayed for 24 to 48 hours to allow administration of corticosteroid to assist fetal lung maturation in pregnancies between 23 and 34 weeks of gestation, providing patient and fetal conditions remain stable
    • Route of delivery is selected on basis of gestational age, fetal presentation, cervical status, obstetric history, and patient and fetal condition
    • Prognosis for both patient and fetus is generally good, although serious complications are common; perinatal morbidity and mortality is primarily caused by early gestational age at delivery

    Urgent Action

    • HELLP syndrome requires immediate obstetric consultation, patient stabilization, and delivery of fetus within 48 hours r2

    Pitfalls

    • Not all patients with HELLP syndrome have hypertension and proteinuria
    • Nonspecific symptoms, such as abdominal pain, malaise, headache, and vomiting, can be mistaken for manifestations of a viral gastrointestinal syndrome

    Terminology

    Clinical Clarification

    • HELLP syndrome is a rare, potentially life-threatening obstetric complication; the acronym reflects that the syndrome is characterized by hemolysis, elevated liver enzyme concentrations, and a low platelet count r3
      • Occurs in 0.5% to 0.9% of all pregnancies and in 10% to 20% of patients with severe preeclampsia r2
      • Widely considered to represent an especially severe form of severe preeclampsia; however, relationship between disorders is controversial, and some consider HELLP syndrome a separate disorder r4

    Diagnosis

    Clinical Presentation

    History

    • Typically develops in the third trimester of pregnancy or early postpartum period
      • About 70% of cases develop before delivery r2c1c2c3
        • Of those, about 70% occur between 27 and 36 weeks of gestation
        • Almost 20% of cases develop beyond 37 weeks of gestation r2c4
      • About 30% of cases develop postpartum r2r5c5
        • Most postpartum cases develop within the first 48 hours but may occur as late as 7 days after delivery
        • In 80% of cases, evidence of preeclampsia is present before delivery r6
    • Onset is usually rapid and, in most cases, preceded by hypertension and proteinuria c6c7
    • Presentation can be variable, and symptoms are often nonspecific, leading to missed or delayed diagnosis r7
    • Typical symptoms are similar to those of preeclampsia and include: r2
      • Abdominal pain c8
        • Right upper quadrant or epigastric pain c9c10
        • May be fluctuating and colicky c11c12
      • Nausea and vomiting c13c14
      • Malaise c15
      • Headache (30%-60% of cases) r2c16
      • Visual changes (20% of cases) r2c17
      • Nonspecific viral syndrome–like symptoms, without fever c18

    Physical examination

    • Physical findings are similar to those of preeclampsia and include:
      • Hypertension c19
        • Systolic 140 mm Hg or higher or diastolic 90 mm Hg or higher r1
          • Diagnosis of hypertension is based on 2 blood pressure measurements made at least 4 hours apart r1
        • Severe hypertension: Systolic 160 mm Hg or higher or diastolic 110 mm Hg or higher r1c20
          • Diagnosis can be based on 2 blood pressure measurements within a short interval (to initiate prompt treatment) r1
        • Hypertension may be absent in 10% to 20% of patients r2c21
      • Excessive weight gain r2c22
      • Generalized edema r2c23
      • Jaundice (rarely) c24

    Causes and Risk Factors

    Causes

    • Pathogenesis is unclear; may represent a severe form of preeclampsia associated with abnormal placental development and function c25
      • Thought to occur due to a systemic inflammatory disorder with endothelial injury, mediated by a complement cascade r8
      • Develops in 10% to 20% of patients with severe preeclampsia r5c26
      • Patients with preeclampsia are at higher risk of developing HELLP syndrome than those without preeclampsia

    Risk factors and/or associations

    Age
    • Mean age of patients with HELLP syndrome is usually higher than mean age of those with preeclampsia r2
    • Persons aged 25 years or older are at higher risk for HELLP syndrome than those who are younger than 25 years r9c27c28
    Genetics
    • HELLP syndrome demonstrates a familial tendency like preeclampsia; however, may have different genetic basis than preeclampsia r10c29
    • No single genetic cause has been identified; multiple gene variants along with environmental factors may contribute r6c30
    Ethnicity/race
    • No clear evidence indicates persons of a particular race are at increased risk for HELLP syndrome r11c31c32c33c34
      • However, higher rates of preeclampsia are seen in Black, Native American, and Alaskan Native patients
        • Among those with preeclampsia, higher rates of severe morbidity and mortality for both the patient and the fetus are seen among non-Hispanic Black patients than any other race or ethnicity
      • Differences in prevalence and outcomes appear to be linked to racial inequities, with mixed results in research on biological differences r11
    Other risk factors/associations
    • History of preeclampsia or HELLP syndrome r9c35c36
    • Unlike in preeclampsia, nulliparity is not a risk factor because at least 50% of patients with HELLP syndrome are multiparous r5r12c37

    Diagnostic Procedures

    Primary diagnostic tools

    • Initial evaluation includes a thorough history, physical examination, blood pressure measurements, and blood and urine testing to detect presence of proteinuria and/or end-organ involvement c38
    • Obtain CBC, peripheral blood smear, and hepatic function panel (including AST, ALT, and bilirubin levels) and lactate dehydrogenase in everyone with suspected preeclampsia or HELLP syndrome based on clinical presentation (typical symptoms, usually in association with hypertension and proteinuria) r2c39c40c41
      • Two methods of diagnosis are commonly used c42
        • Tennessee classification r2
          • Hemolysis, indicated by 2 or more of the following: evidence of microangiopathic hemolysis on peripheral smear (schistocytes, burr cells); serum bilirubin 1.2 mg/dL or greater; serum haptoglobin 25 mg/dL or less or lactate dehydrogenase 2 or more times the upper level of normal; severe anemia not due to blood loss (hemoglobin 8-10 g/dL, depending on gestation)
          • Elevated liver enzymes, indicated by AST or ALT 2 or more times the upper level of normal
          • Low platelets, indicated by platelet count less than 100,000/mm³
          • HELLP is classified as complete when all 3 above findings are present and as partial or incomplete when just 1 or 2 are present
        • American College of Obstetricians and Gynecologists criteria r1
          • Lactate dehydrogenase 600 IU/L or greater
          • AST and ALT 2 or more times the upper level of normal
          • Platelet count less than 100,000/mm³
    • Obtain comprehensive metabolic panel, including creatinine concentration, disseminated intravascular coagulation panel, and urine protein concentration (24-hour urine collection or protein-creatinine ratio) c43c44c45c46
    • Assess fetal well-being with nonstress test and ultrasonographic evaluation to estimate fetal weight and amniotic fluid index; biophysical profile is indicated if nonstress test is nonreactive r1c47c48c49c50c51
      • In potentially unstable situations, fetal monitoring is always performed first using bedside ultrasonography
    • Determine if patient is in labor and evaluate cervix with Bishop score r2

    Laboratory c52c53c54c55c56c57

    • CBC r2c58
      • Platelet count less than 100,000/mm³ is necessary to diagnose HELLP syndrome r1
        • Platelet counts can decrease to as low as 6000/mm³ (6 × 10⁹/L), but any platelet count less than 150,000/mm³ (150 × 10⁹/L) warrants attention r9c59
        • Mississippi subclassification assigns severity by nadir of thrombocytopenia but is not widely used clinically r2
          • Class I: platelet count less than 50,000/mm³, AST or ALT 70 IU/L or higher, and lactate dehydrogenase 600 IU/L or higher
          • Class II: platelet count between 50,000/mm³ and 100,000/mm³, AST or ALT 70 IU/L or higher, and lactate dehydrogenase 600 IU/L or higher
          • Class III: platelet count between 100,000/mm³ and 150,000/mm³, AST or ALT 40 IU/L or higher, and lactate dehydrogenase 600 IU/L or higher
            • Note that Class III levels do not meet commonly used diagnostic criteria
      • Reticulocyte count can be increased c60
      • Hematocrit may be decreased or within reference range and is typically the last of the abnormalities to appear; finding of decreased serum haptoglobin concentration may confirm ongoing hemolysis when hematocrit is within reference range r2
    • Peripheral blood smear r2c61
      • Microangiopathic hemolysis is suggested by presence of fragmented RBCs (schistocytes) or contracted, spiculated RBCs (Burr cells) in peripheral blood smear
    • Hepatic function panel c62c63c64c65
      • AST or ALT concentration of 70 units/L or higher is typical in HELLP syndrome r2
        • Laboratory thresholds vary; diagnosis is based on AST and/or ALT more than 2 times the upper reference range for local laboratory r1
      • Intravascular hemolysis is supported by elevated serum bilirubin concentration (at least 20.5 μmol/L or at least 1.2 mg/100 mL) and elevated lactate dehydrogenase concentration greater than 600 units/L (or at least 2 times the upper level of normal for laboratory range r2
    • Serum haptoglobin level c66
      • Decrease is useful to corroborate presence of intravascular hemolysis in conjunction with minimal microangiopathic changes in RBC morphology r13
    • Urine protein to creatinine ratio r1c67
      • Proteinuria is defined by ratio 0.3 or greater (or protein of 300 mg or more in a 24-hour urine collection)

    Differential Diagnosis

    Most common

    • Pregnancy-related conditions
      • Preeclampsia c68d1
        • Complication of pregnancy characterized by hypertension and proteinuria or, in the absence of proteinuria, by new onset of organ dysfunction
        • HELLP syndrome may represent a severe form of preeclampsia, and there is significant overlap in clinical and biochemical features
        • Like HELLP syndrome, preeclampsia may manifest with hypertension, proteinuria, epigastric pain, headache, and visual symptoms
        • Unlike HELLP syndrome, preeclampsia is more common in nulliparous females, and hypertension and proteinuria are always present
        • May be differentiated on basis of the following laboratory findings:
          • No evidence of microangiopathic hemolysis on peripheral blood smear
          • Platelet count may be within reference range (may be decreased in severe cases)
      • Acute fatty liver of pregnancy r14c69
        • Rare complication of pregnancy caused by disordered maternal metabolism of fatty acids occurring in third trimester
        • Clinical signs are variable, and in severe cases there is significant overlap in clinical and biochemical features with HELLP syndrome r2
        • Like HELLP syndrome, acute fatty liver of pregnancy typically occurs in third trimester of pregnancy and manifests as malaise, anorexia, nausea, vomiting, mid-epigastric or right upper abdominal pain, and headache
        • Unlike most cases of HELLP syndrome, may be associated with jaundice and low-grade fever, and hypertension and proteinuria are usually absent
        • May be differentiated on basis of the following laboratory findings: r15
          • Platelet count within reference range at presentation
          • Elevated WBC count, creatinine concentration, and bilirubin concentration
          • Prolonged prothrombin time or activated partial thromboplastin time
          • Antithrombin III activity reduced below 65%
          • Hypoglycemia
    • Non–pregnancy-related conditions
      • Conditions such as gastroenteritis, acute cholecystitis, appendicitis, and acute hepatitis may have similar clinical presentation with abdominal pain, malaise, nausea, and vomiting c70c71c72c73
        • Unlike HELLP, presenting symptoms are not associated with hypertension or proteinuria
        • Differentiated on basis of history, clinical features, and laboratory findings
          • No evidence of microangiopathic hemolysis on peripheral blood smear; platelet count within reference range
      • Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome also have hemolysis, thrombocytopenia, and may have similar clinical presentation (eg, nausea, vomiting, abdominal pain, headache) r2
        • Unlike HELLP, elevation of AST and ALT is usually mild and anemia is more prominent

    Treatment

    Goals

    • Stabilize patient and assess fetal condition
    • Promptly deliver infant

    Disposition

    Admission criteria

    All patients require admission for immediate obstetric consultation and delivery r2

    Criteria for ICU admission
    • Most patients with HELLP syndrome require admission to obstetric ICU; neonatal ICU is required for infant in most cases r16

    Recommendations for specialist referral

    • All patients are managed by obstetrician in consultation with maternal-fetal medicine specialist, hematologist, and anesthesiologist as necessary

    Treatment Options

    Promptly initiate antihypertensive therapy in patients who have persistent (15 minutes or more) hypertension with systolic blood pressure higher than 160 mm Hg or diastolic blood pressure higher than 110 mm Hg, or both r1

    • Optimal target has not been determined, but protocols recommend systolic blood pressure lower than 160 mm Hg and diastolic blood pressure lower than 100 mm Hg r17
    • Lowering blood pressure too much may be harmful; avoid systolic blood pressure lower than 120 mm Hg or diastolic blood pressure lower than 80 mm Hg r17
    • If patient is already established on daily antihypertensive therapy, choose an agent from a different drug class of current regimen r18
    • For treatment during pregnancy, use fetal surveillance during antihypertensive treatment if fetus is viable r17

    Administer magnesium sulfate for seizure prophylaxis r1

    Delivery is definitive treatment for cases during pregnancy (although postpartum cases occur)

    • Timing of delivery depends on gestational age and conditions of patient and fetus
      • Deliver immediately after patient stabilization in the following: r1
        • Pregnancies at 34 weeks of gestation or longer
        • Pregnancies shorter than 23 weeks of gestation or if fetus is nonviable
        • Pregnancies between 23 and 34 weeks of gestation if there is disseminated intravascular coagulation, liver infarction or hemorrhage, renal failure, pulmonary edema, suspected abruptio placentae, or nonreassuring fetal status
          • Administering corticosteroids for fetal lung maturation is recommended, but do not delay delivery to complete course r1
      • Deliver within 24 to 48 hours after patient stabilization in pregnancies between 23 and 34 weeks of gestation, provided patient and fetus remain stable r1
        • Administering corticosteroids for fetal lung maturation is recommended, but do not complete course if condition of pregnant patient or fetus deteriorates r2
    • Route of delivery is selected on basis of gestational age, fetal presentation, cervical status, obstetric history, and patient and fetal condition r1
      • Cesarean delivery is recommended for standard obstetric indications (eg, unfavorable cervix, oligohydramniosr2)
      • Platelet transfusion before vaginal delivery is recommended by some experts if platelet count is less than 20,000/mm³ and before cesarean delivery if less than 50,000/mm³; precise threshold is determined in consultation with hematologist r19r20r21
      • Spinal or epidural anesthesia is recommended if analgesia or anesthesia is required for delivery r1
        • No definitive lower limit platelet count is known for neuraxial anesthesia; however, risk of epidural hematoma is exceptionally low in patients with stable platelet counts 70,000/mm³ without anticoagulants or coagulopathy r1
    • Expectant management is not typically recommended r2
      • Expectant management before completing 34 weeks of gestation may be an acceptable option in carefully selected patients if it is performed in tertiary care units under close surveillance; however, there is limited evidence that this is beneficial r2r22r23
      • Not recommended if neonate is not expected to survive r1
      • Deliver immediately if fetal or maternal condition deteriorates during expectant management r1

    Corticosteroids do not appear to reduce mortality and morbidity r1r24r25

    • May improve maternal platelet counts, but no overall evidence of benefit exists r24r25
      • May be considered in settings in which improved platelet count is clinically important

    Drug therapy

    • Antihypertensive drugs r2r17c74
      • Beta-blocker
        • Labetalol c75
          • Bolus dose
            • Labetalol Hydrochloride Solution for injection; Adolescents†: 10 to 20 mg IV, then 20 to 80 mg IV every 10 to 30 minutes until goal blood pressure is attained. Max cumulative dose: 300 mg.
            • Labetalol Hydrochloride Solution for injection; Adults: 10 to 20 mg IV, then 20 to 80 mg IV every 10 to 30 minutes until goal blood pressure is attained. Max cumulative dose: 300 mg.
          • Continuous infusion
            • Labetalol Hydrochloride Solution for injection; Adolescents†: 0.5 to 2 mg/minute continuous IV infusion, initially. Titrate dose every 15 minutes until goal blood pressure is attained. Max: 10 mg/minute. Max cumulative dose: 300 mg.
            • Labetalol Hydrochloride Solution for injection; Adults: 0.5 to 2 mg/minute continuous IV infusion, initially. Titrate dose every 15 minutes until goal blood pressure is attained. Max: 10 mg/minute. Max cumulative dose: 300 mg.
      • Calcium channel blocker
        • Nifedipine (immediate release) c76
          • Nifedipine Oral capsule; Adolescents: 10 to 20 mg PO every 15 to 30 minutes, then 10 to 20 mg PO every 2 to 6 hours until blood pressure control is attained. Max: 180 mg/day.
          • Nifedipine Oral capsule; Adults: 10 to 20 mg PO every 15 to 30 minutes, then 10 to 20 mg PO every 2 to 6 hours until blood pressure control is attained. Max: 180 mg/day.
      • Vasodilator
        • Hydralazine c77
          • Bolus dose
            • Hydralazine Hydrochloride Solution for injection; Adolescents: 5 mg IV, then 5 to 10 mg IV every 20 to 40 minutes until goal blood pressure is attained. Max cumulative dose: 20 mg.
            • Hydralazine Hydrochloride Solution for injection; Adults: 5 mg IV, then 5 to 10 mg IV every 20 to 40 minutes until goal blood pressure is attained. Max cumulative dose: 20 mg.
          • Continuous infusion
            • Hydralazine Hydrochloride Solution for injection; Adolescents: 0.5 to 5 mg/hour continuous IV infusion, initially. Titrate by 1 to 2 mg/hour every 15 to 20 minutes until goal blood pressure is attained. Max: 10 mg/hour.
            • Hydralazine Hydrochloride Solution for injection; Adults: 0.5 to 5 mg/hour continuous IV infusion, initially. Titrate by 1 to 2 mg/hour every 15 to 20 minutes until goal blood pressure is attained. Max: 10 mg/hour.
    • Antenatal corticosteroids c78
      • Betamethasone r2c79
        • Betamethasone Acetate, Betamethasone Sodium Phosphate Suspension for injection; Adolescents: 12 mg IM every 24 hours for 2 doses. Consider a repeat or rescue course when at risk of preterm delivery within the next 7 days and prior course administered more than 14 days previously. Rescue course could be provided as early as 7 days from the prior dose if indicated by clinical situation.
        • Betamethasone Acetate, Betamethasone Sodium Phosphate Suspension for injection; Adults: 12 mg IM every 24 hours for 2 doses. Consider a repeat or rescue course when at risk of preterm delivery within the next 7 days and prior course administered more than 14 days previously. Rescue course could be provided as early as 7 days from the prior dose if indicated by clinical situation.
    • Seizure prophylaxis
      • Magnesium sulfate r1r26c80
        • Intravenous
          • Magnesium Sulfate Solution for injection; Adolescents: 4 to 6 g IV loading dose, followed by 1 to 2 g/hour continuous IV infusion for at least 24 hours. Max: 30 to 40 g/24 hours.
          • Magnesium Sulfate Solution for injection; Adults: 4 to 6 g IV loading dose, followed by 1 to 2 g/hour continuous IV infusion for at least 24 hours. Max: 30 to 40 g/24 hours.
        • Intramuscular
          • Magnesium Sulfate Solution for injection; Adolescents: 10 g IM loading dose, followed by 5 g IM every 4 hours for at least 24 hours. Max: 30 to 40 g/24 hours.
          • Magnesium Sulfate Solution for injection; Adults: 10 g IM loading dose, followed by 5 g IM every 4 hours for at least 24 hours. Max: 30 to 40 g/24 hours.

    Nondrug and supportive care

    Administer IV fluids r2c81

    Closely monitor vital signs and fluid balance

    Consider platelet transfusion in actively bleeding patients with platelet count less than 50,000/mm³, before vaginal delivery in those with platelet count less than 20,000/mm³, and before cesarean delivery in those with platelet count less than 40,000 to 50,000/mm³ r13r19r20r21c82

    • Because evidence is limited regarding indications for platelet transfusion, consult hematology and anesthesiology specialists to determine thresholds
    Procedures
    Cesarean delivery c83
    General explanation
    • Surgical delivery of the fetus through incisions in abdominal wall (laparotomy) and uterine wall (hysterotomy)
    Indication
    • Indications for immediate delivery (requiring cesarean delivery if vaginal delivery is not imminent) include: r2
      • Blood pressure higher than 160/110 mm Hg despite treatment with antihypertensive drugs
      • Persisting or worsening clinical symptoms
      • Deteriorating renal function
      • Severe ascites
      • Abruptio placentae
      • Oliguria
      • Pulmonary edema
      • Eclampsia
      • Ruptured subcapsular liver hematoma r2
    • If delivery is required at less than 30 to 32 weeks gestation and cervix is unfavorable for induction, some experts recommend cesarean delivery to avoid risks to fetus associated with long labor r13
    Contraindications
    • No absolute contraindications; however, vaginal delivery is preferred due to lower complications
    • Relative contraindications
      • Severe thrombocytopenia; platelet transfusions are required before procedure
    Complications
    • Same as those with cesarean delivery for any cause
      • Uterine lacerations
      • Bladder lacerations
      • Ureter injury
      • Bowel injury
      • Uterine atony
      • Hemorrhage
      • Postoperative infection
    Induction of labor r27c84
    General explanation
    • Procedure to stimulate uterine contractions before development of spontaneous labor; may be preceded by measures to facilitate cervical ripening
    • Oxytocin is the most common medication used to induce labor c85
    • Other methods to induce labor include prostaglandin E analogues, mechanical cervix dilation, membrane stripping, and amniotomy
    Indication
    • Indicated to initiate delivery before onset of spontaneous labor in patients with HELLP syndrome
    Contraindications
    • General contraindications for induction of labor
      • Active genital HSV infection
      • Vasa previa or complete placenta previa
      • Transverse fetal lie
      • Umbilical cord prolapse
      • Previous classic cesarean delivery
      • Previous myomectomy
    • Specific contraindications in HELLP syndrome
      • Ruptured subcapsular liver hematoma r2
      • If the cervix is unfavorable for induction, some experts recommend against induction of labor at less than 30 to 32 weeks to avoid risks to fetus associated with long labor r13
    Complications
    • Same as those associated with induction of labor for any cause
      • Uterine hyperstimulation
      • Uterine rupture
      • Fetal distress and fetal acidosis
      • Failed labor induction requiring cesarean delivery

    Comorbidities c86

    Special populations

    • HELLP that develops after delivery r2
      • Most within 48 hours but can occur up to 7 days after delivery
      • Risk of renal failure and pulmonary edema is significantly higher than with pre-partum HELLP
      • For persistent hemolysis, thrombocytopenia, and hypoproteinemia, RBC and platelet transfusion and protein supplementation are standard therapy
      • Use of steroids is not indicated

    Monitoring

    • Maternal r1
      • Closely monitor vital signs, fluid intake, and urine output c87c88c89
      • Monitor for contractions, rupture of membranes, abdominal pain, or bleeding
      • Obtain CBC, platelet count, liver enzyme, and creatinine concentrations at least every 12 hours r1c90c91c92c93
    • Fetal r1
      • Continuous fetal monitoring in a labor and delivery unit is recommended c94
    • Pharmacotherapy
      • Antihypertensive use: if either blood pressure threshold is still exceeded after reaching max doses, obtain emergency consultation from maternal–fetal medicine, internal medicine, anesthesia, or critical care subspecialists r28
        • Hydralazine: reflex tachycardia, prolonged hypotension, headache, palpitations, tremors, vomiting, and fluid retention; may cause abnormal fetal heart rate tracings r1r17
        • Labetalol: neonatal bradycardia; avoid in patients with history of asthma, heart failure, or preexisting myocardial disease r18r28
        • Nifedipine: reflex tachycardia and headache r1r28
      • Monitor for magnesium toxicity; assess deep tendon reflexes periodically r29
        • Measure serum magnesium concentration at 4 to 6 hours and adjust infusion to maintain concentrations between 4 and 7 mEq/L (5-9 mg/dL) r1

    Complications and Prognosis

    Complications

    • Maternal complications r2
      • Eclampsia c95
      • Abruptio placentae c96
      • Disseminated intravascular coagulation c97
      • Acute renal failure c98
      • Severe ascites c99
      • Cerebral edema c100
      • Pulmonary edema c101
      • Acute respiratory distress syndrome c102
      • Wound hematoma or infection after cesarean delivery c103c104
      • Subcapsular liver hematoma c105
      • Liver rupture c106
      • Hepatic infarction c107
      • Recurrent thrombosis c108
      • Cerebral hemorrhage c109
      • Retinal detachment c110
      • Cerebral infarction c111
    • Fetal complications r2
      • Intrauterine growth restriction c112
      • Preterm delivery and associated complications c113
      • Neonatal thrombocytopenia c114
      • Respiratory distress syndrome c115

    Prognosis

    • Prompt diagnosis and delivery result in best outcome for patient and fetus
    • Maternal complications are relatively common
      • Mortality reported in 1% to 25% of patients r2
      • AST levels greater than 2000 units/L and lactate dehydrogenase levels greater than 3000 units/L are associated with increased risk of mortality r1
    • Patients with HELLP syndrome might have an increased risk of future cardiovascular and metabolic disease (eg, hypertension, elevated BMI, elevated glucose, metabolic syndrome, unfavorable lipid parameters) based on studies of patients with preeclampsia and eclampsia, some of which included those with HELLP syndrome r30
    • Fetal prognosis is strongly influenced by gestational age at delivery; prognosis is poorer with delivery at earlier gestational ages
      • Perinatal death reported in 7.4% to 34% of patients r2

    Screening and Prevention

    Screening

    At-risk populations

    • Patients with HELLP syndrome in previous pregnancies are at risk in subsequent pregnancies r2

    Screening tests

    • No specific screening tests; early antenatal care and more frequent antenatal monitoring are required c116

    Prevention

    • No specific preventive therapy for HELLP syndrome; however, daily low-dose aspirin after 12 weeks of gestation is recommended to reduce risk of preeclampsia in those at high risk r20c117c118
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