This disease was called the flashy term “HELLP syndrome” for a reason. If such a diagnosis was made during pregnancy, then it’s time to sound the alarm: urgent medical attention is needed. The body seems to refuse to perform the reproductive function, and all systems begin to fail, threatening the life of the expectant mother and her baby. What is the disease, and what actions should be taken to prevent its development?

What is HELLP syndrome

HELLP syndrome is very dangerous. In short, this is gestosis in a complicated form, caused by the autoimmune reaction of the woman’s body to pregnancy. It includes a whole range of health problems - malfunctions of the liver and kidneys, bleeding, poor blood clotting, increased blood pressure, swelling and much more. As a rule, it develops in the third trimester or in the first two days after birth and requires emergency medical attention. Moreover, clinical manifestations occur before childbirth in 31% of cases, and in the postpartum period - in 69%.

Explanation of the abbreviation HELLP:

• H - Hemollysis - hemolysis;
• EL - elevated liver enzymes - excess activity of liver enzymes;
• LP - Low platelet count - thrombocytopenia.

Doctors are afraid of the syndrome because of its rapid progression and frequent deaths. Fortunately, it is rare: approximately 1–2 cases per 1 thousand pregnancies.

This disease was first described at the end of the 19th century. But it wasn't until 1985 that his symptoms were linked together and named under the umbrella term "HELLP." It is interesting that Soviet medical reference books say almost nothing about this syndrome, and only rare Russian resuscitators mentioned the disease in their works, calling it “an obstetrician’s nightmare.”

HELLP syndrome has not yet been fully studied, so it is difficult to name specific reasons for its development. Today, doctors suggest that the likelihood of the disease increases with:

• repeat pregnancy;
• drug and viral hepatitis;
• unstable emotional and mental state;
• genetic abnormalities in liver function;
• pregnancy in adulthood (28 years and above);
• advanced cases of gestosis;
• disorders of the liver and gallbladder;
• gallstone and urolithiasis;
• systemic lupus;
• gastritis;
• blood clotting disorders.

Clinical picture of the disease

Diagnosing HELLP syndrome is quite difficult, since its symptoms do not always manifest themselves in full force. In addition, many signs of the disease are common during pregnancy and have nothing to do with this serious condition. The development of complicated gestosis can be indicated by:

• nausea and vomiting, sometimes with blood (in 86% of cases);
• pain in the upper abdomen and under the ribs (in 86% of cases);
• swelling of the arms and legs (in 67% of cases);
• pain in the head and ears;
• high blood pressure (over 200/120);
• the appearance of protein and traces of blood in the urine;
• changes in blood composition, anemia;
• yellowness of the skin;
• bruises at injection sites, nosebleeds;
• blurred vision;
• convulsions.

It is worth noting that changes in urine and blood parameters usually appear long before the clinical manifestation of the disease, so every pregnant woman needs to visit her gynecologist in a timely manner and take all the tests he prescribes.

Many of the described symptoms also occur with gestosis. However, HELLP syndrome is characterized by a rapid increase in symptoms that develop within 4–5 hours. If the expectant mother feels such changes in her body, she should immediately call an ambulance.

According to statistics, 6–8 hours pass from the first manifestations of the syndrome to death in the absence of necessary medical care. Therefore, if you suspect an illness, it is very important to consult a doctor as soon as possible.

Preeclampsia, preeclampsia, eclampsia or HELLP syndrome?

If HELLP syndrome is suspected, the doctor has no more than 2–4 hours to conduct research and decide on further treatment tactics. He makes a diagnosis based on examination, ultrasound results, liver tests and blood tests. Sometimes pregnant women are prescribed a CT scan to rule out hemorrhage in the liver.

The term “preeclampsia” is used in Russian and Ukrainian medical documents and literature. In the international classification of diseases it is called preeclampsia. If it is accompanied by convulsions, it is called eclampsia. HELLP syndrome is the most severe form of gestosis, which differs in severity and number of clinical symptoms.

Liver pain

HELLP syndrome is a serious disease. According to various sources, maternal mortality with it ranges from 24 to 75%. The outcome of pregnancy, the health of the woman and the fetus mainly depend on when the disease was detected.

Statistics of complications in HELLP syndrome (per 1 thousand patients) - table

Obstetric tactics

If HELLP syndrome is suspected, hospitalization is indicated for the patient. It is important to quickly conduct an examination and relieve life-threatening symptoms in order to stabilize the condition of the expectant mother. In the case of premature pregnancy, measures are required to prevent possible complications in the fetus.

The only effective treatment for HELLP syndrome is termination of pregnancy. Natural childbirth is indicated provided that the uterus and cervix are ripe. In this case, doctors use drugs that stimulate labor. If a woman’s body is not physiologically ready for childbirth, then an emergency caesarean section is performed.

With HELLP syndrome, pregnancy must be terminated, regardless of its duration, within 24 hours. Natural birth is possible only after 34 weeks. In other cases, surgery is indicated.

Immediately upon admission to the hospital, the patient is prescribed corticosteroids (for example, dexamethasone). They significantly reduce the risk of liver damage. In addition, other drugs are used, including droppers, to restore water-salt metabolism, improve blood flow in the uterus and placenta, and calm the nervous system.

Often women undergo transfusions and undergo plasmapheresis - blood filtration using special devices. It cleanses the blood of toxins and helps avoid further complications. It is prescribed for disorders of fat metabolism, a history of repeated gestosis, hypertension, and pathologies of the kidneys and liver.

The newborn also needs help immediately after birth, since HELLP syndrome causes many diseases in infants.

What complications can occur as a result of HELLP syndrome for a mother and her baby?

The consequences of HELLP syndrome are serious for both the woman and her child. There is a risk for the expectant mother:

• pulmonary edema;
• acute renal failure;
• cerebral hemorrhages;
• hematoma formation in the liver;
• liver rupture;
• premature placental abruption;
• lethal outcome.

High blood pressure disrupts blood circulation in the placenta, as a result of which the fetus does not receive the necessary oxygen. This leads to the following complications for the baby:

• hypoxia, or oxygen starvation;
• cerebral hemorrhage during childbirth;
• developmental delay (50% of newborns);
• damage to the nervous system;
• breathing problems in a newborn;
• suffocation;
• thrombocytopenia - a blood disease in which the number of platelets sharply decreases (25% of newborns);
• of death.

Recovery after surgery

Most complications can be avoided thanks to a timely caesarean section. The operation is performed under endotracheal anesthesia - a combined method of anesthesia, in which painkillers enter both the blood and the woman’s respiratory tract. It protects the patient from pain, shock and respiratory failure.

After the operation, the young mother is carefully monitored. Especially in the first two days. At this time, there is still a high risk of complications. With proper treatment, all symptoms disappear within 3–7 days. If after a week all blood, liver and other organ parameters are restored, the patient can be discharged home.

The timing of discharge depends on the condition of the woman and her child.

To prevent HELLP syndrome or minimize severe consequences, follow these recommendations:

• plan and prepare for conception, get examined in advance, lead a healthy lifestyle;
• register for pregnancy on time, follow the doctor’s instructions;
• eat right;
• try to lead an active lifestyle, spend more time outdoors;
• give up bad habits;
• avoid stress;
• from the 20th week, keep a pregnancy diary, enter into it everything that happens to the body (weight changes, pressure surges, fetal movements, the appearance of edema);
• regularly take tests prescribed by your doctor;
• Pay attention to unusual symptoms - abdominal pain, tinnitus, dizziness and others.

Preeclampsia and its complications during pregnancy - video

HELLP syndrome is a fairly rare complication. To detect the disease in a timely manner, take the necessary tests prescribed by the doctor and listen to your condition. If dangerous symptoms occur, consult a doctor immediately. Modern diagnostics and correct treatment tactics in most cases bring positive results.

There inevitably comes a time in every person’s life that forces them to seek outside help. Often health workers act as assistants in such situations. This happens if the human body is occupied by an insidious disease, and it is not possible to cope with it independently. Everyone knows that a happy state of pregnancy is not a disease, but it is expectant mothers who especially need medical and psychological help.

“Help!”, or Where did the name of the disease come from?

The call for help sounds different in different languages. For example, in English the desperate Russian “Help!” pronounced "help". It is no coincidence that HELLP syndrome is practically in tune with the already international plea for help.

The symptoms and consequences of this complication during pregnancy are such that urgent medical intervention is essential. The abbreviation HELLP stands for a whole range of health problems: liver function, blood clotting and an increased risk of bleeding. In addition to the above, HELLP syndrome causes malfunction of the kidneys and blood pressure disorders, thereby significantly aggravating the course of pregnancy.

The picture of the disease can be so severe that the body denies the very fact of childbirth, and an autoimmune failure occurs. This situation occurs when the female body is completely overloaded, when defense mechanisms refuse to work, severe depression sets in, and the will to achieve life’s achievements and further struggle disappears. The blood does not clot, the wounds do not heal, the bleeding does not stop, and the liver is not able to perform its functions. But this critical condition is amenable to medical correction.

Disease history

HELP syndrome was described at the end of the 19th century. But it was not until 1978 that Goodlin linked this autoimmune pathology to preeclampsia during pregnancy. And in 1985, thanks to Weinstein, the disparate symptoms were united under one name: HELLP syndrome. It is noteworthy that this serious problem is practically not described in domestic medical sources. Only a few Russian anesthesiologists and resuscitation specialists examined this formidable complication of gestosis in more detail.

Meanwhile, HELP syndrome during pregnancy is rapidly gaining momentum and claiming many lives.

We will describe each complication separately.

Hemolysis

HELP syndrome primarily involves intravascular hemolysis. This terrible disease is characterized by total cellular destruction. The destruction and aging of red blood cells causes fever, yellowing of the skin, and the appearance of blood in urine tests. The most life-threatening consequences are the risk of heavy bleeding.

Danger of thrombocytopenia

The next component of the abbreviation for this syndrome is thrombocytopenia. This condition is characterized by a decrease in platelets in the blood count, which over time causes spontaneous bleeding. This process can only be stopped in a hospital setting, and during pregnancy this condition is especially dangerous. The cause may be severe immune disorders, resulting in an anomaly in which the body fights itself, destroying healthy blood cells. A blood clotting disorder caused by a change in platelet count poses a threat to life.

An ominous harbinger: increased liver enzymes

The complex of pathologies included in HELP syndrome is crowned by such an unpleasant symptom as an increase in liver enzymes. For expectant mothers, this means that serious malfunctions occur in one of the most important organs of the human body. After all, the liver not only cleanses the body of toxins and helps with digestive function, but also affects the psycho-emotional sphere. Often such an undesirable change is detected during a routine blood test, which is prescribed to a pregnant woman. In gestosis complicated by HELP syndrome, the indicators differ significantly from the norm, revealing a threatening picture. Therefore, medical consultation is the first mandatory procedure.

Features of the third trimester

The 3rd trimester of pregnancy is very important for further gestation and delivery. Common complications include swelling, heartburn and digestive dysfunction.

This occurs due to disturbances in the functioning of the kidneys and liver. An enlarged uterus puts serious pressure on the digestive organs, which is why they begin to malfunction. But with gestosis, conditions called preeclampsia and eclampsia can occur, which aggravate pain in the epigastric region and provoke nausea, vomiting, edema, and high blood pressure. Convulsive seizures may occur against the background of neurological complications. Dangerous symptoms increase, sometimes almost at lightning speed, causing enormous harm to the body, threatening the life of the expectant mother and fetus. Due to the severe course of gestosis, which often occurs during the 3rd trimester of pregnancy, a syndrome with the self-explanatory name HELP often occurs.

Vivid symptoms

HELLP syndrome: clinical picture, diagnosis, obstetric tactics - the topic of today's conversation. First of all, it is necessary to identify a number of main symptoms that accompany this formidable complication.

HELP syndrome is characterized by a variety of symptoms:

• unpleasant sensations in the liver area;
• vomiting;
• acute headaches;
• convulsive seizures;
• feverish condition;
• disturbance of consciousness;
• insufficiency of urination;
• swelling of tissues;
• pressure surges;
• multiple hemorrhages at the sites of manipulation;
• jaundice.

In laboratory tests, the disease is manifested by thrombocytopenia, hematuria, detection of protein in the urine and blood, decreased hemoglobin, and increased bilirubin content in a blood test. Therefore, to clarify the final diagnosis, it is necessary to conduct a full range of laboratory tests.

How to recognize complications in time?

In order to identify and prevent dangerous complications in a timely manner, a medical consultation is carried out, to which expectant mothers are advised to attend regularly. The specialist registers the pregnant woman, after which the changes occurring in the woman’s body throughout the entire period are closely monitored. Thus, the gynecologist will promptly record unwanted deviations and take appropriate measures.

Pathological changes can be detected using laboratory tests. For example, a urine test will help detect protein, if any. An increase in protein levels and the number of leukocytes indicates pronounced disturbances in the functioning of the kidneys. Among other things, there may be a sharp decrease in the amount of urine and a significant increase in edema.

Problems in the functioning of the liver are manifested not only by pain in the right hypochondrium, vomiting, but also by changes in blood composition (an increase in the number of liver enzymes), and upon palpation an enlarged liver is clearly felt.

Thrombocytopenia is also detected during laboratory testing of the blood of a pregnant woman for whom the threat of HELP syndrome is real.

If you suspect the occurrence of eclampsia and HELP syndrome, blood pressure control is mandatory, since due to vasospasm and blood thickening, its levels can seriously increase.

Differential diagnosis

The now fashionable diagnosis of HELP syndrome in obstetrics has gained popularity, so it is often mistakenly diagnosed. It often hides completely different diseases, no less dangerous, but more prosaic and widespread:

• gastritis;
• viral hepatitis;
• systemic lupus;
• urolithiasis disease;
• obstetric sepsis;
• liver diseases (fatty degeneration, cirrhosis);
• thrombocytopenic purpura of unknown etiology;
• renal failures.

Therefore, diff. diagnosis must take into account the variety of options. Accordingly, the triad indicated above - liver hyperfermentemia, hemolysis and thrombocytopenia - does not always indicate the presence of this complication.

Causes of HELP syndrome

Unfortunately, the risk factors have not been sufficiently studied, but there are suggestions that the following reasons can provoke HELP syndrome:

• psychosomatic pathologies;
• drug-induced hepatitis;
• genetic enzymatic changes in liver function;
• multiple births.

In general, a dangerous syndrome occurs when there is insufficient attention to the complicated course of gestosis - eclampsia. It is important to know that the disease behaves very unpredictably: it either develops at lightning speed or disappears on its own.

Therapeutic measures

When all the tests and differentials have been completed. diagnostics, certain conclusions can be drawn. When a diagnosis of HELP syndrome is made, treatment is aimed at stabilizing the condition of the pregnant woman and the unborn child, as well as speedy delivery, regardless of the term. Medical measures are carried out with the help of an obstetrician-gynecologist, a resuscitation team, and an anesthesiologist. If necessary, other specialists are involved: a neurologist or an ophthalmologist. First of all, multiple organ failure is eliminated, and preventive measures are provided to avoid possible complications.

Among the common phenomena that complicate the course of drug intervention are:

• placental abruption;
• hemorrhages;
• cerebral edema;
• pulmonary edema;
• acute renal failure;
• fatal changes and rupture of the liver;
• uncontrollable bleeding.

With a correct diagnosis and timely professional assistance, the likelihood of a complicated course tends to a minimum.

Obstetric strategy

The tactics practiced in obstetrics in relation to severe forms of gestosis, especially those complicated by HELP syndrome, are unambiguous: the use of cesarean section. With a mature uterus, ready for natural childbirth, prostaglandins and mandatory epidural anesthesia are used.

In severe cases, during cesarean section, endotracheal anesthesia is used exclusively.

Life after childbirth

Experts have noted that the disease occurs not only during the third trimester, but can also progress within two days after getting rid of the burden.

Therefore, HELP syndrome after childbirth is a completely possible phenomenon, which speaks in favor of close monitoring of mother and child in the postpartum period. This is especially true for women in labor with severe preeclampsia during pregnancy.

Who is to blame and what to do?

HELP syndrome is a disruption of the functioning of almost all organs and systems of the female body. During the disease, there is an intense outflow of vital forces, and there is a high probability of death, as well as intrauterine pathologies of the fetus. Therefore, from the 20th week, the expectant mother needs to keep a self-control diary, where she will record all the changes occurring in the body. Particular attention should be paid to the following points:

• blood pressure: its jumps upward more than three times should alert you;
• metamorphosis of weight: if it began to increase sharply, perhaps the cause was swelling;
• fetal movement: too intense or, conversely, frozen movements are a clear reason to consult a doctor;
• the presence of edema: significant tissue swelling indicates renal dysfunction;
• unusual abdominal pain: especially significant in the liver area;
• regular tests: everything that is prescribed must be performed conscientiously and on time, as this is necessary for the benefit of the mother herself and the unborn child.

You should immediately report any alarming symptoms to your doctor, since only a gynecologist is able to adequately assess the situation and make the only correct decision.

Hellp syndrome is a rare but serious complication that affects pregnant women. This is a variant of preeclampsia. HELLP syndrome means the following signs and symptoms:

• H – hemolysis (decomposition of red blood cells);
• EL - Elevated liver enzyme;
• LP-Low platelet count.

The condition affects about 0.5-0.9% of pregnant women. Occurs later in pregnancy or maybe even after childbirth.

The exact cause of the syndrome is unknown. It is considered a symptom of an underlying disorder and not in itself. It is a complication of preeclampsia, a disorder in pregnant women with high blood pressure and protein in the urine (proteinuria).

Other risk factors include:

• obesity;
• Poor nutrition;
• Diabetes;
• Age of pregnant women (over 35 years);
• Multiple pregnancy;
• History of preeclampsia.

Signs and symptoms

It is accompanied by a series of symptoms:

• Fatigue and malaise;
• Fluid retention;
• Increase in excess weight;
• Nausea and vomiting worsen over time;
• Paresthesia (tingling sensation in the limbs);
• Visual disturbances;
• swelling, especially in the legs;
• Nose bleed;
• Cramps.

Diagnostics

Symptoms associated with Hellp syndrome often mimic other diseases or complications. A physical examination followed by confirmation from blood and urine tests is carried out to diagnose it.

• During a physical examination, the doctor will look for an enlarged liver or excess swelling, especially in the legs.

Blood tests

• CBC (complete blood count) contains information about red blood cells, white blood cells, platelet count. Hemolysis, the breakdown of red blood cells, is a characteristic feature of help syndrome. An abnormal peripheral smear with a low platelet count indicates a problem.
• LDH (lactate dehydrogenase) is an enzyme that helps body tissues produce energy. LDH is present in almost all tissues of the body. LDH levels increase if there is damage.
• An LFT (liver function test) is a series of blood tests done to detect the presence of liver disease. Liver enzymes are high due to liver damage, excessive breakdown of red blood cells.

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Other studies

• A urine test detects the presence of excess protein along with elevated uric acid levels.
• Blood pressure, if high, means Help syndrome.
• An MRI or CT scan is recommended to check for internal bleeding, especially in the liver.
• Fetal monitoring tests include sonograms, stress-free tests, and fetal movement assessments to check the baby's health.

Treatment

Having a child is the final treatment. This will prevent further complications. Most women stop experiencing symptoms 4-5 days after giving birth. Delivery should be considered after completion of 34 weeks of pregnancy.

• Corticosteroids are prescribed to help the baby and mother. If birth can be delayed, corticosteroids should be given to promote fetal maturation.
• During pregnancy, women with low platelet counts may need blood. Consequently, blood transfusion occurs. Transfusion of red blood cells, platelets, and fresh frozen plasma is required.
• It is necessary to take medications to control blood pressure. Antihypertensive drugs such as labetalol, nifedipine are prescribed.
• Magnesium sulfate is prescribed to prevent episodes of seizures.

Forecast

Early diagnosis is the key to reducing morbidity and mortality. If the condition is treated early, most women make a full recovery.

If Hellp syndrome remains undiagnosed, about 25% of women develop serious complications such as blood clots, placental abruption, kidney failure, and liver damage.

The condition cannot be completely prevented. However, if a woman is diagnosed with preeclampsia, precautions can be taken to reduce the risk of Hellp syndrome.

• Maintain a healthy lifestyle that includes regular exercise and weight control that is appropriate for your height
• Follow a balanced diet consisting of fresh vegetables, fruits, and protein.

Which doctor should I contact?

If you find any signs associated with Hellp syndrome, contact your obstetrician or gynecologist.

Is it always associated with preeclampsia?

No. Although Help syndrome is a complication of preeclampsia, only about 10-20% of preeclampsia cases develop it.

What happens when placental abruption occurs?

The placenta is the structure responsible for feeding the developing baby. In placental abruption, the placental lining separates from the inner lining of the uterus before birth.

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How is preeclampsia treated?

In most cases, preeclampsia resolves after delivery. Corticosteroids are used in severe preeclampsia to improve liver and platelet function. Magnesium sulfate is the best choice for treatment.

How does it affect children?

Hellp syndrome can affect a baby's survival after birth, as women experience preterm birth. For example, if a baby is born weighing more than 1000 g, the survival rate and health of the baby is the same as that of a normal newborn.

However, if the weight is less than 1000g, then the child will need to be monitored in hospital. Additional tests will be required to ensure its safety.

Is there a risk of it appearing in the future?

There is a 20% chance of developing Help syndrome in future pregnancies.

When does it occur?

It can occur any time after the second trimester to six months after birth. Mostly occurs in the third trimester, or 24-48 hours after birth.

Pregnancy is accompanied by hormonal changes, increased stress on the mother's body, toxicosis and edema. But in rare cases, a woman’s discomfort is not limited to these phenomena. More serious diseases or complications may occur, the consequences of which can be extremely severe. These include HELLP syndrome.

What is HELLP syndrome in obstetrics?

HELLP syndrome most often develops against the background of severe forms of gestosis (after 35 weeks of pregnancy). Late toxicosis (as gestosis is sometimes called) is characterized by the presence of protein in the urine, high blood pressure and is accompanied by edema, nausea, headache, and decreased visual acuity. In this condition, the body begins to produce antibodies to its own red blood cells and platelets. Impaired blood function causes destruction of the walls of blood vessels, which is accompanied by the formation of blood clots, which lead to a malfunction of the liver. The frequency of diagnosing HELLP syndrome ranges from 4 to 12% of established cases of gestosis.

A number of symptoms that often led to the death of the mother and (or) child were first collected and described as a separate syndrome by J. A. Pritchard in 1954. The abbreviation HELLP is made up of the first letters of the Latin names: H - hemolysis (hemolysis), EL - elevated liver enzymes (increased activity of liver enzymes), LP - low platelet count (thrombocytopenia).

The causes of HELLP syndrome in pregnant women have not been identified. But presumably it can be provoked by:

• the expectant mother's use of medications such as tetracycline or chloramphenicol;
• abnormalities of the blood coagulation system;
• liver enzyme disorders, which may be congenital;
• weakness of the body's immune system.

Risk factors for HELLP syndrome include:

• light skin tone of the expectant mother;
• previous repeated births;
• severe illness in the fetal carrier;
• cocaine addiction;
• multiple pregnancy;
• woman's age is 25 years and above.

First signs and diagnosis

Laboratory blood tests allow you to diagnose HELLP syndrome even before the appearance of its characteristic clinical signs. In such cases, you may find that the red blood cells are deformed. The following symptoms are the reason for further examination:

• yellowness of the skin and sclera;
• noticeable enlargement of the liver upon palpation;
• sudden bruising;
• reduction in breathing rate and heart rate;
• increased anxiety.

Although the period of pregnancy at which HELLP syndrome most often occurs begins at 35 weeks, cases have been recorded in which the diagnosis was made at 24 weeks.

If HELLP syndrome is suspected, the following is carried out:

• Ultrasound (ultrasound examination) of the liver;
• MRI (magnetic resonance imaging) of the liver;
• ECG (electrocardiogram) of the heart;
• laboratory tests to determine the number of platelets, the activity of blood enzymes, the concentration of bilirubin, uric acid and haptoglobin in the blood.

Symptoms of the disease most often (69% of all diagnosed cases of HELLP syndrome) appear after delivery.

They begin with nausea and vomiting, soon unpleasant sensations in the right hypochondrium, restless motor skills, obvious swelling, fatigue, headache, increased reflexes of the spinal cord and brain stem.

What can mother and child expect?

It is impossible to give accurate forecasts of the consequences of HELLP syndrome. It is known that in a favorable scenario, the signs of complications in the mother disappear on their own within a period of three to seven days. In cases where the level of platelets in the blood is excessively low, the woman in labor is prescribed corrective therapy aimed at restoring water and electrolyte balance. After it, the indicators return to normal around the eleventh day.

The likelihood of recurrence of HELLP syndrome in subsequent pregnancies is approximately 4%.

Fatalities reported for HELLP syndrome range from 24 to 75%. In most cases (81%), labor occurs prematurely: this may be a physiological phenomenon or a medical termination of pregnancy in order to reduce the risk of irreversible complications for the mother.

Intrauterine fetal death, according to studies conducted in 1993, occurs in 10% of cases. The death of a child within seven days after birth has the same probability.

• In surviving children whose mother suffered from HELLP syndrome, in addition to somatic pathologies, certain abnormalities are observed:
• blood clotting disorder - in 36%;
• instability of the cardiovascular system - in 51%;

Obstetric tactics in case of diagnosing HELLP syndrome

A common medical solution for established HELLP syndrome is emergency delivery. In late pregnancy, the likelihood of a live birth is quite high.

After preliminary procedures (cleansing the blood of toxins and antibodies, plasma transfusion, platelet infusion), a caesarean section is performed.

As further treatment, hormonal therapy (glucocorticosteroids) and drugs are prescribed that are designed to improve the condition of liver cells damaged as a result of gestosis. In order to reduce the activity of enzymes that break down protein, protease inhibitors are prescribed, as well as immunosuppressants to suppress the immune system. A hospital stay is necessary until the clinical and laboratory signs of HELLP syndrome completely disappear (the peak of red blood cell destruction often occurs within 48 hours after birth).

• Indications for emergency delivery at any stage:
• progressive thrombocytopenia;
• signs of a sharp deterioration in the clinical course of gestosis;
• disturbances of consciousness and severe neurological symptoms;
• progressive decline in liver and kidney function;

distress (intrauterine hypoxia) of the fetus.

• Consequences that increase the likelihood of maternal death include:
• DIC syndrome and uterine bleeding caused by it;
• acute liver and kidney failure;
• cerebral hemorrhage;
• pleural effusion (fluid accumulation in the lung area);
• subcapsular hematoma in the liver, which entails subsequent rupture of the organ;

retinal disinsertion.

Complication of pregnancy - video

A severe complication of pregnancy, which is characterized by a triad of symptoms: hemolysis, damage to the liver parenchyma and thrombocytopenia. It is clinically manifested by rapidly increasing symptoms - pain in the liver and abdomen, nausea, vomiting, swelling, jaundice of the skin, increased bleeding, impaired consciousness up to coma. Diagnosed on the basis of a general blood test, studies of enzyme activity and the state of hemostasis. Treatment involves emergency delivery, the prescription of active plasma replacement, hepatostabilizing and hepatoprotective therapy, and drugs that normalize hemostasis.

General information

Although HELLP syndrome has been observed infrequently in recent years, it complicates the course of severe gestosis in 4-12% of cases and, in the absence of adequate treatment, has high rates of maternal and child mortality. The syndrome as a separate pathological form was first described in 1954. The name of the disorder is formed by the first letters of terms that define the key manifestations of the disease: H - hemolysis (hemolysis), EL - elevated liver enzymes (increased activity of liver enzymes), LP - low level platelet (thrombocytopenia). HELLP syndrome usually occurs in the 3rd trimester of pregnancy at 33-35 weeks. In 30% of cases it develops 1-3 days after birth. According to the results of observations, the risk group consists of fair-skinned pregnant women over 25 years of age with severe somatic disorders. With each subsequent pregnancy, the likelihood of developing the disease increases, especially if we are talking about bearing two or more fetuses.

Causes

To date, the etiology of the disorder has not been definitively determined. Specialists in the field of obstetrics and gynecology have proposed more than 30 theories of the occurrence of this acute obstetric pathology. Most likely, it develops due to a combination of a number of factors, aggravated by the course of gestosis. Some authors consider pregnancy as one of the options for allotransplantation, and HELLP syndrome as an autoimmune process. Among the most common causes of the disease are:

• Immune and autoimmune disorders. In the blood of patients, depression of B- and T-lymphocytes is noted, antibodies to platelets and vascular endothelium are determined. The ratio in the prostacyclin/thromboxane pair is reduced. Sometimes the disease complicates the course of another autoimmune pathology - antiphospholipid syndrome.
• Genetic abnormalities. The basis for the development of the syndrome may be a congenital failure of liver enzyme systems, which increases the sensitivity of hepatocytes to the action of damaging factors that occur during an autoimmune response. A number of pregnant women also have congenital disorders of the coagulation system.
• Uncontrolled use of certain medications. The likelihood of developing pathology increases with the use of pharmacological drugs that have a hepatotoxic effect. First of all, we are talking about tetracycline and chloramphenicol, the damaging effect of which increases with the immaturity of enzyme systems.

Pathogenesis

The triggering point in the development of HELLP syndrome is a decrease in the production of prostacyclin against the background of an autoimmune reaction that occurs as a result of the effect of antibodies on cellular elements of the blood and endothelium. This leads to microangiopathic changes in the inner lining of the blood vessels and the release of placental thromboplastin, which enters the mother's bloodstream. In parallel with damage to the endothelium, vascular spasm occurs, provoking placental ischemia. The next stage in the pathogenesis of HELLP syndrome is the mechanical and hypoxic destruction of red blood cells, which pass through the spasmodic vascular bed and are actively attacked by antibodies.

Against the background of hemolysis, platelet adhesion and aggregation increases, their overall level decreases, the blood thickens, multiple microthrombosis occurs, followed by fibrinolysis, and disseminated intravascular coagulation syndrome develops. Impaired perfusion in the liver leads to the formation of hepatosis with necrosis of the parenchyma, the formation of subcapsular hematomas and an increase in the level of enzymes in the blood. Blood pressure increases due to vasospasm. As other systems become involved in the pathological process, signs of multiple organ failure increase.

Classification

There is no unified systematization of the forms of HELLP syndrome yet. Some foreign authors suggest taking into account laboratory data when determining the variant of a pathological condition. In one of the existing classifications, there are three categories of laboratory indicators that correspond to hidden, suspected and obvious signs of intravascular coagulation. A more accurate option is based on determining platelet concentration. According to this criterion, three classes of the syndrome are distinguished:

• 1st class. The level of thrombocytopenia is less than 50×10 9 /l. The clinic is characterized by a severe course and a serious prognosis.
• 2nd class. The blood platelet content ranges from 50 to 100×10 9 /l. The course of the syndrome and prognosis are more favorable.
• 3rd grade. There are moderate manifestations of thrombocytopenia (from 100 to 150×10 9 /l). The first clinical signs are observed.

Symptoms

The initial manifestations of the disease are nonspecific. A pregnant woman or woman in labor complains of pain in the epigastrium, right hypochondrium and abdominal cavity, headache, dizziness, a feeling of heaviness in the head, pain in the muscles of the neck and shoulder girdle. Weakness and fatigue increase, vision deteriorates, nausea and vomiting, and swelling occur. Clinical symptoms progress very quickly. As the condition worsens, areas of hemorrhage form at the injection sites and on the mucous membranes, and the skin becomes jaundiced. There is lethargy and confusion. In severe cases of the disease, convulsive seizures and the appearance of blood in the vomit are possible. In the terminal stages, a coma develops.

Complications

HELLP syndrome is characterized by multiple organ disorders with decompensation of the basic vital functions of the body. In almost half of the cases, the disease is complicated by disseminated intravascular coagulation syndrome, every third patient develops signs of acute renal failure, and every tenth has cerebral or pulmonary edema. Some patients develop exudative pleurisy and pulmonary distress syndrome. In the postpartum period, profuse uterine bleeding with hemorrhagic shock is possible. In rare cases, in women with HELLP syndrome, the tissue peels off and a hemorrhagic stroke occurs. In 1.8% of patients, subcapsular hematomas of the liver are detected, the rupture of which usually leads to massive intra-abdominal bleeding and death of the pregnant or parturient woman.

HELLP syndrome is dangerous not only for the mother, but also for the child. If pathology develops in a pregnant woman, the likelihood of premature birth or placental abruption with coagulopathic bleeding increases. In 7.4-34.0% of cases, the fetus dies in utero. Almost a third of newborns experience thrombocytopenia, leading to hemorrhages in the brain tissue and subsequent neurological disorders. Some children are born in a state of asphyxia or with respiratory distress syndrome. A serious, although infrequent, complication of the disease is intestinal necrosis, detected in 6.2% of infants.

Diagnostics

Suspicion of the development of HELLP syndrome in a patient is the basis for urgent laboratory tests to verify damage to the hemostatic system and hepatic parenchyma. Additionally, control of basic vital parameters is provided (respiration rate, pulse temperature, blood pressure, which is elevated in 85% of patients). The most valuable diagnostic tests are the following:

• General blood analysis. A decrease in the number of red blood cells and their polychromasia, deformed or destroyed red blood cells is determined. Thrombocytopenia less than 100×10 9 /l is considered one of the diagnostically reliable criteria. The number of leukocytes and lymphocytes is usually not changed; there is a slight decrease in ESR. Hemoglobin levels drop.
• Liver tests. Violations of enzyme systems typical for liver damage are detected: aminotransferase activity (AST, AlT) is increased 12-15 times (up to 500 U/l). The activity of alkaline phosphatase increases 3 times or more. The level of bilirubin in the blood exceeds 20 µmol/l. Protein and haptoglobin concentrations are reduced.
• Assessment of the hemostasis system. Laboratory signs of consumption coagulopathy are characteristic - the content of coagulation factors synthesized in the liver with the participation of vitamin K decreases. The level of antithrombin III is reduced. Blood coagulation disorders are also indicated by prolongation of thrombin time, decrease in aPTT and fibrinogen concentration.

It should be noted that typical laboratory signs of HELLP syndrome may deviate unevenly from standard indicators; in such cases, they speak of variants of the disease - ELLP syndrome (no hemolysis of red blood cells) and HEL syndrome (platelet content is not impaired). To quickly assess the condition of the liver, an ultrasound examination is performed. Since in severe forms of the disease renal function is impaired, a decrease in the daily amount of urine, the appearance of proteinuria and an increase in the content of nitrogenous substances (urea, creatinine) in the blood is considered an unfavorable prognostic factor. Taking into account the pathogenesis of the disease, ECG, ultrasound of the kidneys, and fundus examination are recommended. In the prenatal period, CTG, ultrasound of the uterus, and Dopplerometry are performed to monitor the condition of the fetus, hemodynamics of the fetus and mother.

Given the seriousness of the prognosis of the disease, its overdiagnosis has recently been noted. HELLP syndrome must be differentiated from severe gestosis, fatty hepatosis of pregnant women, viral and drug-induced hepatitis, hereditary thrombocytopenic purpura, hemolytic uremic syndrome, intrahepatic cholestasis, Dabin-Johnson syndrome, Budd-Chiari syndrome, systemic lupus erythematosus, cytomegalovirus infection, infectious mononucleosis and other pathological conditions. In complex clinical cases, a hepatologist, anesthesiologist-reanimatologist, neurologist, ophthalmologist, infectious diseases specialist, immunologist, therapist, rheumatologist, surgeon, nephrologist are involved in the diagnostic search.

Treatment of HELLP syndrome

Medical tactics when identifying a disease in a pregnant woman are aimed at terminating the pregnancy within 24 hours from the moment of diagnosis. For patients with a mature cervix, vaginal delivery is recommended, but more often an emergency cesarean section is performed under endotrachial anesthesia using non-hepatotoxic anesthetics and prolonged mechanical ventilation. At the stage of intensive preoperative preparation, due to the introduction of fresh frozen plasma, crystalloid solutions, glucocorticoids, fibrinolysis inhibitors, the woman’s condition is maximally stabilized, and, if possible, impaired multiple organ disorders are compensated.

Complex drug therapy aimed at eliminating angiopathies, microthrombosis, hemolysis, influencing various parts of the pathogenesis, restoring the function of the liver and other organs and systems actively continues in the postoperative period. To treat the syndrome, prevent or eliminate its possible consequences, the following are recommended:

• Infusion and blood replacement therapy. The administration of blood plasma and its substitutes, platelet concentrates, and complex saline solutions makes it possible to replenish destroyed formed elements and fluid deficiency in the intravascular bed. An additional effect of such therapy is improvement of rheological parameters and stabilization of hemodynamics.
• Hepatostabilizing and hepatoprotective drugs. To stabilize hepatic cytolysis, parenteral administration of glucocorticoids is prescribed. The use of hepatoprotectors is aimed at improving the functioning of hepatocytes, protecting them from toxic metabolites, and stimulating the restoration of destroyed cellular structures.
• Means for normalizing hemostasis. To improve the parameters of the blood coagulation system, reduce the manifestations of hemolysis and prevent microthrombosis, low molecular weight heparins, other disaggregants and anticoagulants, and drugs with vasoactive effects are used. The administration of protease inhibitors is effective.

Taking into account hemodynamic parameters, patients with HELLP syndrome are given individualized antihypertensive therapy, supplemented with antispasmodics. To prevent possible infectious complications, antibiotics are used with the exception of aminoglycosides, which have hepato- and nephrotoxic effects. According to indications, nootropic and cerebroprotective drugs, vitamin and mineral complexes are prescribed. If manifestations of acute renal failure occur, depending on the severity of the disorder, hemodialysis is also performed.

Prognosis and prevention

The prognosis of HELLP syndrome is always serious. In the past, the mortality rate for the disease reached 75%. Currently, thanks to timely diagnosis and pathogenetic methods of therapy, maternal mortality has been reduced to 25%. For preventive purposes, multiparous women with chronic somatic diseases are recommended to register early with a antenatal clinic and be constantly monitored by an obstetrician-gynecologist. If signs of gestosis are detected, it is important to carefully follow the doctor’s prescriptions, normalize the diet, and adhere to sleep and rest patterns. Rapid deterioration of a pregnant woman's condition with the appearance of symptoms of severe eclampsia and preeclampsia is an indication for emergency hospitalization in an obstetric hospital.