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The HELLP Syndrome– A Therapeutic Challenge. JOHN ESSIEN M.D. JESSICA BARDALES MITAC M.D. J.M RODRÍGUEZ FERNÁNDEZ M.D. EMILIO ORTEGA CALLAVA M.D. HOSPITAL GINECOBSTÉTRICO PROVINCIAL CAMAGÜEY.

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The hellp syndrome a therapeutic challenge

The HELLP Syndrome– A Therapeutic Challenge

JOHN ESSIEN M.D.

JESSICA BARDALES MITAC M.D.

J.M RODRÍGUEZ FERNÁNDEZ M.D.

EMILIO ORTEGA CALLAVA M.D.

HOSPITAL GINECOBSTÉTRICO PROVINCIAL

CAMAGÜEY.


  • Pre-eclampsia - Is a multisystemic, idiopathic disorder specific to the pregnancy and puerperium of the human species. It is characterized by the clinical triad of:

  • Hypertension

  • Proteinuria

  • Edema


  • 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.


HELLP

WEINSTEIN(1982)

HHEMOLYSIS

ELELEVATED LIVER ENZYMES

LP LOW PLATELETS



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


Other Factors to consider :

  • ERITHROCYTIC MORPHOLOGY

  • PLATELETDISORDERS

  • RENALCOMPROMISE

  • HEPATICDISORDERS

  • IMMUNOLOGIC DISORDERS

  • GENETICDISORDERS


The causal factors induce
The Causal Factors induce:

  • Thrombocytopenia

  • Microangiopathic Hemolytic Anemia

  • Periportal necrosis and distension of the liver´s Glisson´s capsule.


Diagnosis
DIAGNOSIS

  • MID-II TRIMESTRE

  • FIRST DAYS POSTPARTUM

  • Antepartum diagnosis is made in 70% between 27 and 37 weeks of gestation.


Criteria for establishing the diagnosis of the hellp syndrome
Criteria for establishing the diagnosis of the HELLP Syndrome

  • HemolysisAbnormalperipherical blood smear Elevated Bilirubin >1.2 mg/dl

  • Elevated liver enzymesSGOT >72 UI / LLDH >600 UI / L

  • Low PlateletsPlatelet Count < 100 × 103 /mm3


We can also observe
We can also observe Syndrome:

  • Excessive body weight increase .

  • Pulse pressure amplification.

  • Systole pressure > 140 mmHg, but diastole pressure < 90 mmHg.

  • Ophthalmic disorders

    -Minor alterations

    -Cortical blindness (amaurosis)

    -Retinal detachment

    -Vitreous hemorrhage.


We can also observe1
We can also observe Syndrome:

  • Elevation of Biomarkers:

    -HCG

    -Maternal alfa-fetal protein

    -LDH

    -Serum Haptoglobin



Clasification of the hellp syndrome based on the platelet count mississippi 1
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.


  • Hemolysis + Liver disfunction count (MISSISSIPPI

    *LDH ≥ 600 UI/l

    *ASAT (SGOT) and/orALAT (SGPT)≥ 40 UI/l

    *ALL HAVE TO PRESENT

    1Magann E.F., Martín J.N. – Twelve Steps to Optimal Management of HELLP Syndrome. Clinical Obstetrics and Gynecology. Lippincott Williams & Wilkins, Philadelphia, 1999. Vol. 42 No. 3: 532-50.


Another c lassification based on the partial or complete expression of the hellp syndrome memphis 1
Another c count (MISSISSIPPIlassification based on the partial or complete expression of the HELLP Syndrome(MEMPHIS)1.

  • 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.


THROMBOTIC count (MISSISSIPPIMICROANGIOPATHIES -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


* count (MISSISSIPPIPRIMARY RENAL DISEASE Glomerulonefritis*OTHERSHepatic encephalopathiesViral hepatitisHyperemesis Gravidarum Idiopathic Thrombocytopenia Renalcalculi Peptic ulcerPielonephritisApendicitisDiabetes Mellitus

Differential Diagnosis of the HELLP Syndrome



1. count (MISSISSIPPI ANTICIPATE THE DIAGNOSIS2.EVALUATE THE MATERNAL CONDITION3.EVALUATE THE FETAL CONDITION 4.CONTROL THE HYPERTENSION5. PROFILAXIS OF CONVULSIONES WITH MgSO46.WATER AND ELECTROLITIC BALANCE 7.HEMOTHERAPY8.MANAGEMENT OF LABOR AND DELIVERY9.OPTIMIZE PERINATAL CARE10.INTENSIVE POSTPARTUM TREATMENT OF THE PATIENT 11.BE ALERT FOR MULTIPLE ORGAN FAILURE12.ADVISE ON FUTURE PREGNANCY


The maternal condition can be evaluated by
The Maternal Condition can be evaluated by: count (MISSISSIPPI

  • 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.


  • Bilirubin. count (MISSISSIPPI Unconjugated bilirubin is increased due to the hemolysis but rarely above 1-2 mg%.

  • Serial evaluation laboratory parameters every 12 to 24 hours or more if necessary.

  • Differential diagnosis with othere pathologies.


Evaluating the fetal condition
Evaluating the Fetal count (MISSISSIPPICondition

  • 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.


Controlling the hypertension
Controlling the hypertension count (MISSISSIPPI

  • 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.


Choice of hypotensive medication
Choice of hypotensive medication count (MISSISSIPPI

  • 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.


  • Sodium Nitroprussiate count (MISSISSIPPIis 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.


Preventing convulsions
Preventing Convulsions count (MISSISSIPPI

  • 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. Loading dose: 15 mg/kg at 40 mg/min with continous monitorization of the cardiac function and BP every 5 minutes. The therapeutic range is 10-20 μg/ml.


Water and electrolytic management
Water and Electrolytic Management count (MISSISSIPPI

  • Objectives:

    -diuresis of 30-40ml/h.

    -Limit intake of liquids to 150ml/h.

    -Balance of electrolytes.

    REMEMBER

    NEGATIVE BALANCE=vasoconstriction.

    EXCESIVE POSITIVE BALANCE= pulmonary damage

  • Monitorization of volume through pulmonary capilar wedge pressure


Hemotherapy
Hemotherapy count (MISSISSIPPI

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.


Hemotherapy1
Hemotherapy count (MISSISSIPPI

  • To avoid postpartum hemorrhage in a transvaginal delivery the indication for platelet transfusion is a count <40.000/mm3.

  • In the immediate postpartum periodo : Maintain the count >50.000/mm3 abdominal deliveries and >20.000/ mm3 in transvaginal deliveries.


Hemotherapy2
Hemotherapy count (MISSISSIPPI

  • 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


Management of labor and delivery
Management of labor and delivery count (MISSISSIPPI

When considering termination of gestation in a patient with HELLP, determine:

  • Gestational age.

  • Maternal and fetal conditions.

  • Fetal presentation.

  • Cervical maturity


Management of labor and delivery1
Management of labor and delivery count (MISSISSIPPI

If transabdominal delivery is requiered, perform:

  • Vertical skin incision.

  • Corporeal incision of the uterus (due to scarse development of the inferior segment and abnormal presentationes).

  • Spontaneous delivery of the placenta to avoid hemorrhage


Optimizing perinatal care
Optimizing perinatal care. count (MISSISSIPPI

  • 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.


Postpartum intensive care
Postpartum Intensive Care. count (MISSISSIPPI

  • 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.

  • Well controled BP with systolic pressure  150 mmHg and diastolic pressure < 100 mmHg.

  • Obvious clinical improvement and absence 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.


Postpartum intensive care the use of dexamethasone
Postpartum Intensive Care - The use of Dexamethasone count (MISSISSIPPI

  • ANTEPARTUM: (0,15mg/kg)10mg IV bid

    - when Platelets <100.000/mm3

    - if Platelets 100.000-50.000/mm3 AND

    Eclampsia, Severe Hypertension, Epigastric Pain

  • POSTPARTUM: 10mg IV bid for 2 dosis, then 5mg bid for 2 additional doses:

    - when steroids were used in antepartum

    - suspend when there is clinical and laboratory improvement (platelets >100.000mm3, decreased LDH, diuresis >100 ml/h)


Be on the lookout for
Be on the lookout for: count (MISSISSIPPI

  • Signs of multiple organ failure.

  • Complications:

  • Subcapsular Hematoma

  • Subcapsular hepatica hemorrhage

  • Hepatic Rupture.

  • Therapeutic solutions:

  • Conservative Procedures

  • Surgery.


Advising on future pregnancies
Advising on future pregnancies. count (MISSISSIPPI

  • 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


Conclusions
Conclusions count (MISSISSIPPI

  • 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.


Thank you

THANK YOU! count (MISSISSIPPI


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