Pregnancy-induced hypertension, preeclampsia and HELLP syndrome are related and overlap in their presentations. Because of the serious associated morbidity and mortality, family physicians who provide maternity care need to be aware of HELLP syndrome so that they can identify it early.
The findings of this multisystem disease are attributed to abnormal vascular tone, vasospasm and coagulation defects. The syndrome seems to be the final manifestation of some insult that leads to microvascular endothelial damage and intravascular platelet activation. With platelet activation, thromboxane A and serotonin are released, causing vasospasm, platelet agglutination and aggregation, and further endothelial damage.
Red blood cells become fragmented as they pass through small blood vessels with endothelial damage and fibrin deposits. The peripheral smear may reveal spherocytes, schistocytes, triangular cells and burr cells. The elevated liver enzyme levels in the syndrome are thought to be secondary to obstruction of hepatic blood flow by fibrin deposits in the sinusoids. This obstruction leads to periportal necrosis and, in severe cases, intrahepatic hemorrhage, subcapsular hematoma formation or hepatic rupture.
Although some investigators speculate that disseminated intravascular coagulopathy DIC is the primary process in HELLP syndrome, most patients show no abnormalities on coagulation studies. HELLP syndrome occurs in approximately 0. The syndrome generally presents in the third trimester of pregnancy, although it occurs at less than 27 weeks of gestation in an estimated 11 percent of patients. Diabetes mellitus.
Chronic hypertension. Multiple gestation. The vague nature of the presenting complaints can make the diagnosis of HELLP syndrome frustrating to physicians. Approximately 90 percent of patients present with generalized malaise, 65 percent with epigastric pain, 30 percent with nausea and vomiting, and 31 percent with headache.
However, right upper quadrant tenderness is present in as many as 90 percent of affected women. The differential diagnosis of HELLP syndrome includes acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Because of the variable nature of the clinical presentation, the diagnosis of HELLP syndrome is generally delayed for an average of eight days. The three chief abnormalities found in HELLP syndrome are hemolysis, elevated liver enzyme levels and a low platelet count.
The hematocrit may be decreased or normal and is typically the last of the three abnormalities to appear. The finding of a decreased serum haptoglobin level may confirm ongoing hemolysis when the hematocrit is normal. In a patient with a plasma fibrinogen level of less than mg per dL 3 g per L , DIC should be suspected, especially if other laboratory abnormalities are also present.
Proteinuria and an increased uric acid concentration are useful in diagnosing preeclampsia but not HELLP syndrome. Therefore, HELLP syndrome should be suspected in any patient who shows a significant drop in the platelet count during the antenatal period.
The first is based on the number of abnormalities that are present. Consequently, patients with the full syndrome should be considered for delivery within 48 hours, whereas those with partial HELLP syndrome may be candidates for more conservative management. Once the diagnosis of HELLP syndrome has been established, the best markers to follow are the maternal lactate dehydrogenase level and the maternal platelet count.
Prompt recognition of HELLP syndrome and timely initiation of therapy are vital to ensure the best outcome for mother and fetus. When the syndrome was first described, prompt delivery was recommended.
Management of severe preeclampsia. Curr Opin Obstet Gynecol ;—3. One study 20 found that pregnancy was prolonged by an average of 15 days when conservative management i. Maternal morbidity was not increased. For infants, the prolongation of pregnancy translated into less time in the neonatal intensive care unit, a decreased incidence of necrotizing enterocolitis and a decreased incidence of respiratory distress syndrome. In the past, delivery in patients with HELLP syndrome was routinely accomplished by cesarean section.
A trial of labor is appropriate in patients with mild to moderate HELLP syndrome who are stable, have a favorable cervix and are at 32 weeks of gestation or greater. The antenatal administration of dexamethasone Decadron in a high dosage of 10 mg intravenously every 12 hours has been shown to markedly improve the laboratory abnormalities associated with HELLP syndrome. Steroids given antenatally do not prevent the typical worsening of laboratory abnormalities after delivery.
However, laboratory abnormalities resolve more quickly in patients who continue to receive steroids postpartum. Patients with HELLP syndrome should be treated prophylactically with magnesium sulfate to prevent seizures, whether hypertension is present or not. A bolus of 4 to 6 g of magnesium sulfate as a 20 percent solution is given initially. This dose is followed by a maintenance infusion of 2 g per hour.
The infusion should be titrated to urine output and magnesium level. Patients should be observed for signs and symptoms of magnesium toxicity. If toxicity occurs, 10 to 20 mL of 10 percent calcium gluconate should be given intravenously. This reduces the risk of maternal cerebral hemorrhage, placental abruption and seizure. The goal is to maintain diastolic blood pressure between 90 and mm Hg.
The most commonly used antihypertensive agent has been hydralazine Apresoline , which is given intravenously in small incremental doses of 2.
Labetolol Normodyne and nifedipine Procardia have also been used with success. Once your baby is born, the illness usually goes away. If you have it once, you are more likely to have it again in future pregnancies. This article was contributed by: familydoctor. This information provides a general overview and may not apply to everyone. Talk to your family doctor to find out if this information applies to you and to get more information on this subject. Pregnancy screenings can provide valuable information before your baby is born about the risks for common birth defects.
If your pregnancy is unexpected, you may be feeling scared or confused about what to do. It is important…. Visit The Symptom Checker. Read More. Recovering from Delivery Postpartum Recovery. Table of Contents. These carry oxygen from the lungs to the rest of the body. E levated L iver enzyme levels — High levels can mean there are liver problems. L ow P latelet counts — Platelets help the blood clot. Pain in the upper right part of your belly.
Bad headaches. Nausea or vomiting. Swelling, especially in your face and hands. Blurry vision. Fluid retention and weight gain. HELLP syndrome treatment.
Other treatments you may receive in the hospital include: A blood transfusion if you have severe bleeding. High blood pressure medicines.
If HELLP syndrome is undiagnosed or untreated, it can result in life-threatening complications for both mother and baby. The most serious complications and risks include:. The maternal mortality rate is about 1. Because there is not a known cause for HELLP syndrome, there is also no identified way to prevent it. Since it is believed to be related to preeclampsia, staying vigilant about diet, exercise and healthy blood pressure can only help.
Scott, James R. Cunningham, F. Gary, et al, Ch. Sibai BM. Obstetrics — Normal and Problem Pregnancies. Philadelphia, Pa: Elsevier Churchill Livingstone; chap American Journal of Obstetrics and Gynecology.
What are the Symptoms? The most common symptoms of HELLP syndrome include: Headaches Nausea and vomiting that continues to get worse— This may also feel like a serious case of the flu. Upper right abdominal pain or tenderness Fatigue or malaise A woman with HELLP may experience other symptoms that often can be attributed to other things such as normal pregnancy concerns or other pregnancy conditions. The most serious complications and risks include: Placental Abruption Pulmonary Edema fluid buildup in the lungs Disseminated intravascular coagulation DIC—blood clotting problems that result in hemorrhage Adult Respiratory distress syndrome lung failure Ruptured liver hematoma Acute renal failure Intrauterine Growth restriction IUGR Infant respiratory distress syndrome lung failure Blood transfusion The maternal mortality rate is about 1.
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