The HELLP Syndrome - PowerPoint PPT Presentation

1 / 41
About This Presentation
Title:

The HELLP Syndrome

Description:

The HELLP Syndrome A Therapeutic Challenge JOHN ESSIEN M.D. JESSICA BARDALES MITAC M.D. J.M RODR GUEZ FERN NDEZ M.D. EMILIO ORTEGA CALLAVA M.D. – PowerPoint PPT presentation

Number of Views:700
Avg rating:3.0/5.0
Slides: 42
Provided by: JOHNE164
Category:

less

Transcript and Presenter's Notes

Title: The HELLP Syndrome


1
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.

2
  • 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

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)
H
HEMOLYSIS
EL ELEVATED LIVER
ENZYMES
LP LOW
PLATELETS
5
  • The reported incidence 2 a 12 .
  • Elevated perinatal morbidity and mortality.
  • Maternal Mortality 35.

6
HELLP SYNDROME POSIBLE PATHOPHYSIOLOGY  CAUSAL
AGENTES Increase in volume., Fetal presence /
decidual cell?, Vasospasm?, Deficiente vascular
repair?, Idiopathic?   Vasculo-endothelial
Disorder   Platelet Agregation/Consumption   Fibri
n Activation/Consumption   Selective organic
Isquemia/nsuficiency   Variable Manifestations
7
Other Factors to consider
  • ERITHROCYTIC MORPHOLOGY
  • PLATELET DISORDERS
  • RENAL COMPROMISE
  • HEPATIC DISORDERS
  • IMMUNOLOGIC DISORDERS
  • GENETIC DISORDERS

8
The Causal Factors induce
  • Thrombocytopenia
  • Microangiopathic Hemolytic Anemia
  • Periportal necrosis and distension of the livers
    Glissons capsule.

9
DIAGNOSIS
  • MID-II TRIMESTRE
  • FIRST DAYS POSTPARTUM
  • Antepartum diagnosis is made in 70 between 27
    and 37 weeks of gestation.

10
Criteria for establishing the diagnosis of the
HELLP Syndrome
  • HemolysisAbnormal peripherical blood smear
    Elevated Bilirubin gt1.2 mg/dl
  • Elevated liver enzymesSGOT gt72 UI / LLDH gt600
    UI / L
  • Low PlateletsPlatelet Count lt 100 103 /mm3

11
We can also observe
  • Excessive body weight increase .
  • Pulse pressure amplification.
  • Systole pressure gt 140 mmHg, but diastole
    pressure lt 90 mmHg.
  • Ophthalmic disorders
  • -Minor alterations
  • -Cortical blindness (amaurosis)
  • -Retinal detachment
  • -Vitreous hemorrhage.

12
We can also observe
  • Elevation of Biomarkers
  • -HCG
  • -Maternal alfa-fetal protein
  • -LDH
  • -Serum Haptoglobin

13
  • The presence of these disorders in an
    hypertensive woman with epigastric and/or right
    hypochondrial pain, nausea, vomiting as well as
    hemolysis, will help in making the right
    diagnosis.

14
Clasification of the HELLP Syndrome based on the
platelet count (MISSISSIPPI)1.
  • Class 1 Platelet count lt50 000/mm3.
  • Class 2 - Platelet count between 50 000 y 100
    000/mm3.
  • Class 3 - Platelet count ltbetween 100 000 y 150
    000/mm3.

15
  • Hemolysis Liver disfunction
  • LDH 600 UI/l
  • ASAT (SGOT) and/or ALAT (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.

16
Another classification 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 lt 100 000/mm3
  • PARTIAL HELLP One or two of the above.

17
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
18
PRIMARY RENAL DISEASE Glomerulonefritis
OTHERSHepatic encephalopathiesViral
hepatitisHyperemesis Gravidarum Idiopathic
Thrombocytopenia Renal calculi Peptic
ulcerPielonephritisApendicitisDiabetes
Mellitus
Differential Diagnosis of the HELLP Syndrome
19
(No Transcript)
20
MANAGEMENT OF THE HELLP SYNDROME
21
1. ANTICIPATE THE DIAGNOSIS2. EVALUATE THE
MATERNAL CONDITION3. EVALUATE THE FETAL
CONDITION 4. CONTROL THE HYPERTENSION5. PROFILAX
IS 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
22
The Maternal Condition can be evaluated by
  • Complete Hemogram. If plateletslt150.000/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.

23
  • Bilirubin. 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.

24
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.

25
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 whengt150mmHg and avoid
    placental hypoperfusion maintaining the diastolic
    BP not less than 80-90 mmHg.

26
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.

27
  • 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
    its photosensitivty and sever rebound effect.

28
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. 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.

29
Water and Electrolytic Management
  • Objectives
  • -diuresis of 30-40ml/h.
  • -Limit intake of liquids to 150ml/h.
  • -Balance of electrolytes.
  • REMEMBER
  • NEGATIVE BALANCEvasoconstriction.
  • EXCESIVE POSITIVE BALANCE pulmonary damage
  • Monitorization of volume through pulmonary
    capilar wedge pressure

30
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 lt50.000/mm3.

31
Hemotherapy
  • To avoid postpartum hemorrhage in a transvaginal
    delivery the indication for platelet transfusion
    is a count lt40.000/mm3.
  • In the immediate postpartum periodo Maintain
    the count gt50.000/mm3 abdominal deliveries and
    gt20.000/ mm3 in transvaginal deliveries.

32
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

33
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

34
Management of labor and delivery
  • 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

35
Optimizing perinatal care.
  • The main risk for the fetus in pregnancies with
    HELLP is its 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.

36
Postpartum Intensive Care.
  • Admision in an obstetrical intensive care unit
    until
  • Sustained increase in the platelet count and a
    maintained decrease in LDH.
  • Diuresis gt100ml/h for 2 consecutive hours
    without duiretics.
  • Well controled BP with systolic pressure ? 150
    mmHg and diastolic pressure lt 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.

37
Postpartum Intensive Care - The use of
Dexamethasone
  • ANTEPARTUM (0,15mg/kg)10mg IV bid
  • - when Platelets lt100.000/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 gt100.000mm3, decreased
    LDH, diuresis gt100 ml/h)

38
Be on the lookout for
  • Signs of multiple organ failure.
  • Complications
  • Subcapsular Hematoma
  • Subcapsular hepatica hemorrhage
  • Hepatic Rupture.
  • Therapeutic solutions
  • Conservative Procedures
  • Surgery.

39
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

40
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.

41
THANK YOU!
Write a Comment
User Comments (0)
About PowerShow.com