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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. - PowerPoint PPT Presentation
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HELLP HELLP Syndrome– Syndrome–
A Therapeutic A Therapeutic ChallengeChallenge
Layali JodehLayali JodehRazan MalheesRazan Malhees55thth year mdical year mdical
studentsstudents
Pre-eclampsia
multisystemic, idiopathic disorder specific to the pregnancy and puerperium of the human species. It is characterized by the presence of ;
Hypertension
Proteinuria
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.
•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%.
ERITHROCYTIC MORPHOLOGY
PLATELET DISORDERS
RENAL COMPROMISE
HEPATIC DISORDERS
IMMUNOLOGIC DISORDERS
GENETIC DISORDERS
Factors to considerFactors to consider :
HELLP SHELLP SYYNDROME NDROME : POSIBLE : POSIBLE PATHOPHYSIOLOGYPATHOPHYSIOLOGY
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
The Causal Factors induce:
Thrombocytopenia
Microangiopathic Hemolytic Anemia
Periportal necrosis and distension of the liver´s Glisson´s capsule.
DIAGNOSISDIAGNOSIS• Third TRIMESTRE
• FIRST DAYS POSTPARTUM 31%
• Antepartum diagnosis is made Antepartum diagnosis is made in 70% between 27 and 37 in 70% between 27 and 37
weeks of gestation.weeks of gestation.
Criteria for establishing the
diagnosis of the HELLP Syndrome
HemolysisAbnormal peripherical 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
We can also observe
Excessive body weight increase .
Ophthalmic disorders -Minor alterations -Cortical blindness (amaurosis)-Retinal detachment-Vitreous hemorrhage.
We can also observe
Alternation in biomarkersAlternation in biomarkers
Increase in ;-Maternal alfa-fetal protein -LDH
Decrease in ;-Serum Haptoglobin-Hematocrit
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.
HELLP SYNDROME: Risk Factors for maternal morbidity.
LABORATORY
CLÍNICAL Platelets< 50.000 Epigastric pain
LDH >1400 UI/L Nauseas
CPK > 200 UI/L Vomitng
ALT > 100 UI/L Eclampsia
AST > 150 UI/L Severe hypertension
Creatinine > 1.0 Abruptio Placentae
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.
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 MICROANGIOPATHIES -Thrombotic thrombocytopenic purpura- Microangiopathic hemolytic anemia induced by sepsis or drugs- Hemolytic Uremic Syndrome 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
Differential Diagnosis of the HELLP Syndrome
*PRIMARY RENAL DISEASE Glomerulonefritis
*OTHERSHepatic encephalopathiesViral hepatitisHyperemesis Gravidarum Idiopathic Thrombocytopenia Renal calculi Peptic ulcerPielonephritisApendicitisDiabetes Mellitus
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.
Bilirubin . Unconjugated bilirubin is
increased due to the hemolysis but rarely above 1-2 mg%.
Differential diagnosis with othere pathologies.
Evaluating the Fetal Condition
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
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.
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.
Choice of hypotensive medication
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.
Preventing Convulsions MgSOMgSO44: 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 PhenytoinPhenytoin.
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.
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
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
Management of labor and delivery
timing of delivery– if > 34 weeks gestation, deliver
– if < 34 weeks gestation, administer corticosteroids, then deliver in 48 hours
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.
Postpartum Intensive Care.
Admision in an obstetrical intensive care unit until:
(1)Sustained increase in the platelet count and a maintained decrease in LDH.
(2)Diuresis >100ml/h for 2 consecutive hours without duiretics.
(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.
Signs of multiple organ failure.
Complications:- Subcapsular Hematoma- Subcapsular hepatica hemorrhage- Hepatic Rupture.
Be on the lookout for:
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.
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
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.