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Wednesday, February 16, 2022

HELLP SYNDROME: Symptoms, Causes, Diagnosis, Complications and Treatment by Nurses Note



Description: Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome is considered to be a variant of pregnancy-induced hypertension (PIH) and preeclampsia, which are dominated by hepatic and hematologic changes.

Prevalence: 0.1%–0.8% of pregnancies; up to 20% of patients with severe preeclampsia.

Predominant Age: Reproductive age. Eighty percent of cases are diagnosed prior to 37 weeks of gestation.

Genetics: No genetic pattern.


Causes: Unknown. Genetic, endocrine/metabolic (including altered prostaglandin production), uteroplacental ischemia, immunologic. A connection to fetal long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency has been proposed.

Clinical symptoms

 Nausea, vomiting

 Right upper quadrant pain


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• Preeclampsia or eclampsia with hemolysis, thrombocytopenia (the degree of thrombocytopenia is predictive of the severity of the disease and the likelihood of poor outcome), elevated hepatic transaminase levels (any or all; blood pressure may be normal in up to 20% of patients)

• Right upper quadrant or epigastric pain

• Nausea, vomiting, and malaise

Risk Factors: Older than 40 years, African-American race, family history of pregnancy-induced hypertension, renal disease, antiphospholipid syndrome, diabetes mellitus, multiple gestation, past history of preeclampsia or HELLP syndrome. Chronic hypertension increases the risk for pregnancy-induced hypertension.

Laboratory findings OF HELLP SYNDROME 

• Hemolysis (with schistocytes seen on peripheral smear)

• Elevated liver function tests

• Low platelet count.

Complications OF HELLP SYNDROME 

• Placental abruption

• Hepatic subcapsular hematoma

• Retinal detachment

• Acute kidney injury

• Pulmonary edema

• Disseminated intravascular coagulation (DIC).


Differential Diagnosis

• Preeclampsia or eclampsia

• Secondary hypertension

• Improper blood pressure measurement (wrong cuff size, position, technique), resulting in false elevation of readings

• Multiple pregnancy

• Molar pregnancy

• Primary hepatic disease and acute fatty liver of pregnancy.

 Associated Conditions: Intrauterine growth restriction, prematurity.

Workup and Evaluation

Laboratory: Liver and renal function studies (eg, enzymes, renal clearance, 24-hour urinary protein), platelet counts, clotting studies (platelet counts of >50,000/mm3 are generally not associated with spontaneous bleeding).

Imaging: Ultrasonography to monitor fetal growth (frequently restricted).

Special Tests: Assessment of fetal lung maturation may be performed, but if maternal disease is severe, management is based on maternal factors and not fetal maturation.

Diagnostic Procedures: Measurement of blood pressure, laboratory confirmation. 

Pathologic Findings

HELLP syndrome is a multiorgan process, including the renal, hepatic, hematologic, and nervous systems.



General Measures: Evaluation, support, and preparation for delivery.

Specific Measures: Patients with HELLP syndrome often represent the sickest patients with preeclampsia or eclampsia. The only true treatment is delivery. The presence of HELLP syndrome generally militates against conservative treatment for any but the briefest stabilization period.

Diet: No specific dietary changes indicated.

Activity: No restriction.

Patient Education: American College of Obstetricians and Gynecologists Patient Education Pamphlet AP034 (Preeclampsia and High Blood Pressure During Pregnancy).

Drug(s) of Choice
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For mild to moderate chronic hypertension, α-methyldopa is considered to be the first-line therapy.

During labor or labor induction, magnesium sulfate is often used to reduce the chance of seizures or to provide fetal neuroprotection for fetuses below 32 weeks (4 g IV for 20 minutes, then 2–3 g/h IV continuous infusion; therapeutic range 4–8 mg/dL).

If blood pressure is >180 torr systolic or 110 torr diastolic—hydralazine HCl 5–10 mg IV bolus every 20 minutes as needed or labetalol 20 mg IV bolus every 10 minutes as needed to a maximum of 300 mg in 24 hours. Sodium nitroprusside may be used for extreme disease.

Steroids have been advocated, but their use has not been supported by large, well-designed randomized, double-blind, placebo-controlled trials.

ContraindicationsAngiotensin-converting enzyme (ACE) inhibitors are teratogenic and are contraindicated in pregnancy. Diuretics should be avoided in pregnancy because of the possibility of adverse fetal effects caused by reduced plasma volume. Despite the common occurrence of edema, these patients have constricted circulatory volume.

Precautions: Central hemodynamic monitoring should be considered if blood pressure is high or potent agents are used.

Alternative Drugs

Verapamil or nifedipine may also be used to acutely reduce blood pressure.

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Patient Monitoring: Increased maternal and fetal surveillance, antenatal testing.

Prevention/Avoidance: The value of low-dose aspirin therapy or Calcium supplementation remains unproved.

Possible Complications: Maternal—cardiac decompensation, stroke, pulmonary edema (10%) and respiratory failure, renal failure (5%), disseminated intravascular coagulation (DIC), subcapsular or intraparenchymal liver hematoma, seizures and seizure-related injuries (6%), retinal detachment, intracranial hemorrhage, coma, and death (0.5%–5% mortality). Fetal risk (growth restriction and death) is directly proportional to both the degree of proteinuria and the level of maternal diastolic blood pressure. Placental abruption may occur in up to 10% of cases.

Expected Outcome: HELLP syndrome generally resolves after delivery, but the risk for recurrence with future pregnancies or elevated blood pressure in later life is increased.

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