Hellp syndrome Done by:

Islam abdalsalam

Iman abdulghafour

Supervised by:

Dr.daher jameel

The history of the patient

:Nameغزالة حمد احمد •

Age: 35 years old

•Sex:female

•Marital status:married

•Occupation: housewife

:Residenceالمحلبية..موصل•

•Date of admishion: 4th of January 2013

•Date of examination: 13 of January 2013

Chief complain: abnormal body movement

Duration: 5 min.

History of present illness:

A36weeks pregnant lady, suddenly developed abnormal movement while she was at home , the movement was tonic clonic of her upper and lower limbs lasting 5 min with drooling of saliva from her mouth and upward eye movement with no cyanosis, no incontinence of sphincters and no tongue biting . She is known case of pregnancy induced hypertension at first trimester ,she complained from nausea, vomiting, urinary tract infection, with poor appetite, with headache and leg edema, no anaemia.

The history of the patient

At second trimester also she developed urinary tract

infection with leg edema.

At third trimester she developed hypertension with

frontal headache radiate to both eyes with blurred vision.

During the last 2 weeks before admission she noticed her

body swell more and more and she said (my clothes

became tight on me).

She was admitted to Albatool hospital and stayed under

observation and treated conservatively until the time of

operation

The history of the patient

she delivered by caesarian section ,the operation lasted

for one hour .

The patient regain her conscious after 30 minutes .

At the end of the operation day she had dry cough but

at next day she developed fever ,convulsion ,disturbance

level of consciousness ,dyspnea, jaundice and she was

hypertensive

The patient was admitted to the respiratory care unite at Ibn sinna hospital

The history of the patient

Review to other systems: Urinary system: she has dysuria, frequency, yellow color urine and loin pain. Respiratory system: no chest pain, no cough, no dyspnea, no hemoptysis . Gastrointestinal system: good appetite but refuse eating because of hypertension, no abdominal pain, normal bowel motion, she had weight gain, no dysphagia, no heart burn. Cardiovascular system: no chest pain, no palpation, no syncope

Locomotor system: backache, bilateral leg pain, no joint pain, no joint swelling

The history of the patient

Past medical history She had pregnancy induced hypertension from first pregnancy at 2010 when she was pregnant by twin, both died after few hours of delivery . The second pregnancy delivered by caesarian section and this new pregnancy is the 3rd one. Past surgical history:cs at 2011 Drug history: during pregnancy she received antihypertensive drug and folic acid

The history of the patient

Social and personal history: She live in moderate socioeconomic state, she is non smoker but her husband is smoker 40 packs/year

Family history: Her sister also has pregnancy induced hypertension

Her father died from carcinoma of colon

The history of the patient

General examination

Young age female patient ,in sitting position ,she look ill.

Conscious,alert,cooperative with good built with presence of CV line in the neck and folly's catheter(tea color urine in the bag)

General examination

• Face: pale , yellowish discoloration of skin and sclera and pallor of the conjectiva ,No cyanosis .

General examination

• The lips are dry ,clotted blood on the lips

General examination

• Tongue:Furred tounge , yellowish discoloration of frenllum

General examination

•Gum : look unhealthy

General examination

•Neck: central venous line on the left side , no lymph nodes enlagement , no visible pulsation , normal thyroid gland

General examination

• Hands and arms:

• skin smooth ,pallor of the palm, bilateral edema of the hands, no palmar erythema, multiple ecchymosis in both arms and hands with tattoo in left arm

General examination

• Nails: no clubbing , no leuconychia ,no koilonychias ,no half and half ,no splinter hemorrhage ,no Dupuytren contracture

bilateral leg pitting edema

Sacral odema

Vital signs • 1-pulse :95

beat/min,regular,large volume,collapsing,just palpable vessles wall

• 2-respiratory rate:22 breath/min.

• 3-blood pressure:

• 120/80mmhg

• 4-temprature:38 c

Systemic examination •Abdominalexamination:

–Inspection: • Huge symmetrical distended

abdomen

• Umbilicus inverted

• Decrease movement with respiration

• Multiple stria whitish in color

• Dilated veins .Plaster on the supra pubic region socked with with blood

Palpation:

• Superficial palpation:

• No tenderness

• No palpable mass

No rigidity

Special test:

• Shifting dullness positive

• Transmitting thrill positive

Systemic examination

•Shifting dullness: positive

•Dipping method :no

•organomegally

Systemic examination

Cardiovascular system:

Inspection:

No visible apex beat, No visible pulsation in the pericardium

No prominent veins on chest, No skeletal abnormality in the chest

Palpation :

no palpable apex beat , no thrill , no left parasternal heave

Auscultation:

Normal heart sound

No any added sound

Systemic examination

Respiratory system: Inspection: Normal shape, Symmetrical, No visible pulsation or scar or dilated veins

Palpation: Normal position of trachea,

Non palpable apex beat,

Symmetrical chest expansion,

Vocal fremitus decrease in the left side

No tenderness

Percussion:dulness in the left side

Auscultation: vesicular breathing , inspiration more prolonge than

expiration with diminish respiration on left side, decrease vocal resonance on left side

Systemic examination

Neurological examination: General Neurological examination: 1-gait: normal

2-level of consciousness:normal

3-mental state:normal

4-speech:normal

Cranial nerve:normal

Sign of meningeal irritation:-ve

Motor system and coordination:normal

Sensory system:normal

Investigation

Investigation

Investigation

Investigation

Investigation

GUE

Appearance of urine

Clotting screen

Test for hepatitis&HIV

US

Chest x-ray

Treatment

Treatment

Treatment

HELLP Syndrome:

What is HELLP Syndrome?

The name HELLP stands for: •H- hemolysis ( breakdown of red blood cells) •EL- elevated liver enzymes (liver function) •LP- low platelets counts (platelets help the blood clot)

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 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.

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.

The thrombocytopenia has been attributed to increased consumption

and/or destruction of platelets.

Although some investigators speculate that disseminated intravascular coagulopathy

(DIC) is the primary process in HELLP syndrome, most patients show no abnormalities

on coagulation studies. Patients who develop DIC generally do so in the setting of well-

developed HELLP syndrome. All patients with HELLP syndrome may have an

underlying coagulopathy that is usually undetectable.

The following is a list of factors that are believed to increase the risk of a woman developing HELLP syndrome: •Previous pregnancy with HELLP Syndrome (19-27% chance of recurrence in each pregnancy)

•Preeclampsia or pregnancy induced hypertension

•Women over the age of 25

•Caucasian

•Multiparous (given birth two or more times)

What are the Symptoms of HELLP Syndrome? The vague nature of the presenting complaints can make the diagnosis of HELLP syndrome frustrating to physicians. Approximately

90% of patients present with generalized malaise

65 %with epigastric pain

30% with nausea and vomiting

31 %with headache 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.

A woman with HELLP may experience other symptoms that often can be attributed to other things such as normal pregnancy concerns or other pregnancy conditions. These symptoms may include: •Visual disturbances

•High blood pressure

•Protein in urine

•Edema (swelling)

•Severe headaches

•Bleeding

The physical examination may be normal in patients with HELLP syndrome. However

.right upper quadrant tenderness is present in as many as 90 percent of affected women. .Edema is not a useful marker because swelling

is a factor in up to 30 percent of normal pregnancies. .Hypertension and proteinuria may be absent or mild.

•How is HELLP Syndrome Diagnosed Because the symptoms of HELP can mimic many other conditions or complications, it is encouraged that physicians run a series of blood tests, including liver function, on any woman experiencing symptoms during the third trimester of pregnancy. HELLP syndrome may occur before the third trimester but it is rare. It also may occur within 48 hours of delivery, although symptoms may take up to 7 days to be evident.

Blood pressure measurements and urine tests to check for

protein are often monitored when diagnosing HELLP

syndrome.

Criteria for establishing the diagnosis of the HELLP Syndrome

Hemolysis Abnormal peripherical blood smear Elevated Bilirubin >1.2 mg/dl Elevated liver enzymes SGOT >72 U/L(NR 13-31 U/L) LDH >600 U/L (NR 80-190 U/L) S.L.A.T (SGPT)≥ 40 U/l(NR 7-35U/L) Low Platelets Platelet Count < 100 × 103 /mm3

We can also observe:

Excessive body weight increase:Increase body weight due to odema Systole pressure > 140 mmHg, but diastole pressure < 90 mmHg. Ophthalmic disorders -Minor alterations -Cortical blindness (amaurosis) -Retinal detachment -Vitreous hemorrhage. Renal function test:very important in late stage of disease B.urea(NR 3.3-7.5mmol/L) S.creatinine(NR 62-124mmol/L)

Clasification of the HELLP Syndrome based

on the platelet count ..

Class 1 – Platelet count <50 000/mm3. Class 2 - Platelet count between 50 000 - 100 000/mm3. Class 3 - Platelet count <between 100 000 - 150 000/mm3.

Another classification based on the partial or complete expression of the HELLP Syndrome

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.

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

MANAGEMENT OF THE HELLP SYNDROME

1. ANTICIPATE THE DIAGNOSIS

2. EVALUATE THE MATERNAL CONDITION

3. EVALUATE THE FETAL CONDITION

4. CONTROL THE HYPERTENSION 5. PROFILAXIS OF CONVULSIONES WITH MgSO4

6. WATER AND ELECTROLYTIC BALANCE

7. HEMOTHERAPY

8. MANAGEMENT OF LABOR AND DELIVERY

9. OPTIMIZE PERINATAL CARE

10.INTENSIVE POSTPARTUM TREATMENT OF THE PATIENT

11.BE ALERT FOR MULTIPLE ORGAN FAILURE

12.ADVISE ON FUTURE PREGNANCY

2)The Maternal Condition can be evaluated by:

CBC:. 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.Urea and Creatinine levels are variable.

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.

3)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.

4)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. Choice of hypotensive medication

Hydralazine: Bolus of 5-10 mg IV every 20-40 min. If

uneffective or unavailable, use labetalol, nifedipine or

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 is a fast acting hypotensive

agent(venous and arterial) which can be used in an

hypertinsive crisis

5)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 hours or 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.

6)Water and Electrolytic Management Objectives: -diuresis of 30-40ml/h. -Limit intake of liquids to 150ml/h. -Balance of electrolytes.

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. 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

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

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 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.

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

Therapeutic solutions

-Conservative Procedures -Surgery.

What are the Risks and Complications of HELLP Syndrome? If HELLP syndrome is undiagnosed or untreated, it can result in life threatening complications for both mother and baby. The most serious complications and risks include: •Placental Abroption

•Pulmonary Edema ( fluid buildup in the lungs) •Adult Respiratory distress syndrome (lung failure) •Diseminated intravascular coagulation (DIC—blood clotting problems that result in hemorrhage) •Ruptured liver hematoma

•Acute renal failure

•IUGR

•Infant respiratory Distress syndrome (lung failure) •Blood transfusion

prognosis: The maternal mortality rate is about 1.1% with HELLP syndrome. The infant morbidity and mortality rate is anywhere from 10-60% depending on many factors such as gestation of pregnancy, severity of symptoms and the promptness of treatment.

How can HELLP Syndrome be Prevented? Because there is not a known cause for HELLP syndrome, there is also no identified way to prevent it. Early identification and treatment is the best way to keep HELLP syndrome from getting serious. Since HELLP syndrome is believed to be related to preeclampsia, staying vigilant about diet, exercise and a healthy blood pressure can only help things!

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%.

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.

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