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HELLP Syndrome– A Therapeutic Challenge

HELLP Syndrome– A Therapeutic Challenge. Layali Jodeh Razan Malhees 5 th year mdical students. Pre-eclampsia multisystemic, idiopathic disorder specific to the pregnancy and puerperium of the human species. It is characterized by the presence of ; Hypertension Proteinuria.

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HELLP Syndrome– A Therapeutic Challenge

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  1. HELLP Syndrome– A Therapeutic Challenge Layali JodehRazan Malhees5th year mdical students

  2. Pre-eclampsia multisystemic, idiopathic disorder specific to the pregnancy and puerperium of the human species. It is characterized by the presence of ; • Hypertension • Proteinuria

  3. Literature dating from the XIXth century report: • Very unusual varieties of severe pre-eclampsia with complicated progress. • These unusual descriptions of pre-eclampsia are recognised today as the HELLP Syndrome. Today: • HELLP Syndrome is considered to be an association of characteristic hepatic and hematologic disorders.

  4. HELLP WEINSTEIN(1982) HHEMOLYSIS ELELEVATED LIVER ENZYMES LP LOW PLATELETS

  5. The reported incidence 0.2-0.6% • Approximately 4 to 12 percent of patients with preeclampsia develop superimposed HELLP syndrome. • Elevated perinatal morbidity and mortality. • Maternal Mortality 35%.

  6. Factors to consider : • ERITHROCYTIC MORPHOLOGY • PLATELETDISORDERS • RENALCOMPROMISE • HEPATICDISORDERS • IMMUNOLOGIC DISORDERS • GENETICDISORDERS

  7. HELLP SYNDROME : POSIBLE PATHOPHYSIOLOGY  CAUSAL AGENTES : Increase in volume., Fetal presence / decidual cell?, Vasospasm?, Deficiente vascular repair?, Idiopathic? Vasculo-endothelial Disorder Platelet Agregation/Consumption Fibrin Activation/Consumption Selective organic Isquemia/nsuficiency Variable Manifestations

  8. The Causal Factors induce: • Thrombocytopenia • Microangiopathic Hemolytic Anemia • Periportal necrosis and distension of the liver´s Glisson´s capsule.

  9. DIAGNOSIS • Third TRIMESTRE • FIRST DAYS POSTPARTUM 31% • Antepartum diagnosis is made in 70% between 27 and 37 weeks of gestation.

  10. Criteria for establishing the diagnosis of the HELLP Syndrome • HemolysisAbnormalperipherical blood smear (reveal spherocytes, schistocytes, triangular cells and burr cells )Elevated Bilirubin >1.2 mg/dl • Elevated liver enzymesSGOT >72 UI / LLDH >600 UI / L • Low PlateletsPlatelet Count < 100 × 103 /mm3

  11. We can also observe • Excessive body weight increase . • Ophthalmic disorders -Minor alterations -Cortical blindness (amaurosis) -Retinal detachment -Vitreous hemorrhage.

  12. We can also observe Alternation in biomarkers Increase in ; -Maternal alfa-fetal protein -LDH Decrease in ; -Serum Haptoglobin -Hematocrit

  13. Clinical Presentation • Approximately 90 percent of patients present with generalized malaise • 65 percent with epigastric pain • 30 percent with nausea and vomiting • 31 percent with headache.

  14. Clasification of the HELLP Syndrome based on the platelet count (MISSISSIPPI)1. • Class 1 – Platelet count <50 000/mm3. • Class 2- Platelet count between 50 000 y 100 000/mm3. • Class 3 - Platelet count <between 100 000 y 150 000/mm3.

  15. Another classification based on the partial or complete expression of the HELLP Syndrome(MEMPHIS)1.

  16. Complete HELLP – *Microangiopathic hemolytic anemia in women with severe pre-eclampsia   *LDH ≥ 600 UI / L *SGOT ≥ 70 UI/l * Thrombocytopenia < 100 000/mm3 • PARTIAL HELLP– One or two of the above.

  17. MANAGEMENT OF THE HELLP SYNDROME

  18. THROMBOTICMICROANGIOPATHIES -Thrombotic thrombocytopenic purpura- Microangiopathic hemolytic anemia induced by sepsis or drugs- Hemolytic UremicSyndrome FIBRINOGEN CONSUMPTION DISORDERS– CID-Acute fatty liver-Sepsis- Severa Hypovolemia / Hemorrhage (Abruptio/Amniotic fluid embolism)CONNECTIVO TISSUE DISORDERS-Systemic Lupus Erithematosus Differential Diagnosis of the HELLP Syndrome

  19. *PRIMARY RENAL DISEASEGlomerulonefritis *OTHERSHepatic encephalopathiesViral hepatitisHyperemesis Gravidarum Idiopathic Thrombocytopenia Renalcalculi Peptic ulcerPielonephritisApendicitisDiabetes Mellitus Differential Diagnosis of the HELLP Syndrome

  20. The Maternal Condition can be evaluated by: • Complete hemogram . If platelets <150.000/mm3 requieres more study. • Liver Enzymes. The elevation of the transaminases and LDH is a sign of hepatic disfunction. • Renal function. Deficencies in renal function are observed in late stages of the illness. Creatinine and Uric acid levels are variable.

  21. Bilirubin . Unconjugated bilirubin is increased due to the hemolysis but rarely above 1-2 mg%. • Differential diagnosis with othere pathologies.

  22. Evaluating the FetalCondition • Determine the gestational age. • Evaluate fetal well-being: Non-stress test, Tolerance to contracction test and/or biophysical profile. • Use corticosteroids between 24 and 34 weeks to improve fetal pulmonary maturity/neonatal pulmonary function as well as maternal and perinatal results.

  23. Controlling the hypertension • 80-85% of patients with HELLP need control of their BP to avoid significant maternal and perinatal morbidity and mortality. • Treat systolic BP when>150mmHg and avoid placental hypoperfusion maintaining the diastolic BP not less than 80-90 mmHg.

  24. Choice of hypotensive medication • Hydralazine: Bolus of 5-10 mg IV every 20-40 min. If uneffective or unavailable, use labetalol, nifedipine o sodium nitroprussiate. • Labetalol: Initial bolus of 20 mg IV, with increases in dosage until a satisfactory BP is obtained or up to maximum dose of 300 mg. • Nifedipina oral(not sublingual) at usual dosage.

  25. Sodium Nitroprussiate is a fast acting hypotensive agent(venous and arterial) which can be used in an hypertinsive crisis when all other hypotensive drugs have failed Loading dose: 0,25 μg/kg/min, increasing upto 10 μg/kg/min. Above this dose there is a greater risk of cyanide intoxication of the fetus. When using, remember it’s photosensitivty and sever rebound effect.

  26. Preventing Convulsions • MgSO4: Initial bolus of 4-6g IV, followed by a continous infusion at 1,5-4g/h, individualized according to the patient. Continue 48 horas o more postpartum until clinical and laboratory signs of improvement are obtained. • If contraindications of MgSO4 exist, use Phenytoin.

  27. Hemotherapy The base of hemotherapy in patients with HELLP is the transfusion of platelets. • The usual dose is one unit per every 10 kg of corporal weight. • Spontaneous bleeding occurs in most cases with a platelet count of <50.000/mm3.

  28. Hemotherapy • The aggresive use of Dexamethasone in patients with HELLP and severe thrombocytopenia has eliminated virtually all need for platelet transfusion. • Other therapeutic alternatives: -Plasmaphersis -Immunoglobulins

  29. Management of labor and delivery When considering termination of gestation in a patient with HELLP, determine: • Gestational age. • Maternal and fetal conditions. • Fetal presentation. • Cervical maturity

  30. Management of labor and delivery • timing of delivery • if > 34 weeks gestation, deliver • if < 34 weeks gestation, administer corticosteroids, then deliver in 48 hours

  31. Optimizing perinatal care. • The main risk for the fetus in pregnancies with HELLP is it´s prematurity. • The use of corticosteroids decreases the morbidity associated with pulmonary immaturity in preterm babies. • Delivery should be in a center with capability of treating these children with a major risk of cardiopulmonary instability.

  32. Postpartum Intensive Care. • Admision in an obstetrical intensive care unit until: • Sustained increase in the platelet count and a maintained decrease in LDH. • Diuresis >100ml/h for 2 consecutive hours without duiretics.

  33. (3)Well controled BP with systolic pressure 150 mmHg and diastolic pressure < 100 mmHg. (4) Obvious clinical improvement and bsence of complications. The absence of improvement of the thrombocytopenia within 72-96 hours postpartum indicates severe compromise of compensatory mechanisms and possibel MULTIPLE ORGAN FAILURE.

  34. Be on the lookout for: • Signs of multiple organ failure. • Complications: • Subcapsular Hematoma • Subcapsular hepatica hemorrhage • Hepatic Rupture.

  35. Hepatic Rupture • The incidence of hepatic rupture varies from one in 40,000 to one in 250,000 pregnancies . • Hepatic infarction is even more rare and commonly involves the right lobe. • It is believed to be a continuum of preeclampsia, in which areas of coalescing hemorrhage result in thinning of the capsule and intraperitoneal hemorrhage.

  36. Advising on future pregnancies. • The risk of recurrence of preeclampsia-eclampsia is 42-43% and for the HELLP syndrome: 19-27%. • The risk of recurrence of preterm delivery is high, about 61%.1

  37. Conclusions • HELLP Syndrome and its management still poses a problem in modern obstetrics • Precise diagnosis and early treatment with non-mineral corticosteroides such as Dexamethasone may help achieve favorable maternal and perinatal results.

  38. THANK YOU!

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