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HELLPSyndrome - HELLP Syndrome as a separate entity Dr...

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HELLP Syndrome as a separate entity Dr. Mohammed Abdalla EGYPT, Domiat G. Hospital
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HELLP Syndrome may it be a separate entity? yes
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HELLP , a syndrome characterized by h h emolysis, emolysis, e e levated levated l l iver enzyme iver enzyme levels and a levels and a l l ow ow p p latelet count latelet count , is an obstetric complication that is frequently misdiagnosed at initial presentation. Many investigators consider the syndrome to be a variant of preeclampsia, but it may be a separate entity.
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In some cases , HELLP symptoms are the first warning of preeclampsia and the condition is misdiagnosed as hepatitis, idiopathic thrombocytopenic purpura, gallbladder disease, or thrombotic thrombocytopenic purpura.
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Epidemiology and Risk Factors HELLP syndrome 0.2 to 0.6 % of all pregnancies. Preeclampsia 5 to 7 % of all pregnancies. Superimposed HELLP syndrome develops in 4 to 12 percent of women with preeclampsia or eclampsia. Wolf JL. Liver disease in pregnancy. Med Clin North Am 1996 . Maternal mortality has been estimated to be as high as 2- 24% Perinatal mortality is equally high, ranging from 9 –39 %.
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The hemolysis in HELLP syndrome is a microangiopathic hemolytic anemia . 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. increase in Bilirubin and lactic dehydrogenase levels. Etiology and Pathogenesis
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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. Etiology and Pathogenesis
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The thrombocytopenia has been attributed to increased consumption and/or destruction of platelets . With platelet activation, thromboxane A and serotonin are released, causing vasospasm, platelet agglutination and aggregation, and further endothelial damage. Etiology and Pathogenesis
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Clinical Presentation 90%of patients present with generalized malaise, 65 % with epigastric pain, 30 % with nausea and vomiting, 31 percent with headache. All are nonspecific symptoms
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Because of the variable nature of the clinical presentation, the diagnosis of HELLP syndrome is generally delayed for an average of eight days. Usually presented by complications Usually presented by complications
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In one retrospective chart review of patients with HELLP syndrome, only two of 14 patients entered the hospital with the correct diagnosis.
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Because early diagnosis of this syndrome is critical, any pregnant woman who presents with malaise or a viral-type illness in the third trimester should be evaluated with a complete blood cell count and liver function tests.
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Clinical Presentation The physical examination may be normal in patients with HELLP syndrome.
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