Help syndrome after childbirth treatment. HELLP syndrome: causes and development, symptoms, diagnosis, treatment, prognosis

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. Disorders 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. Prescription 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.

During pregnancy, a woman's body experiences enormous stress. All systems ensure the health of not only the mother, but also the baby. The development of pathologies during this period of a person’s life occurs in its most severe form. This is due to the limited “safety margin” of the body, as well as the peculiarities of metabolism during gestation. One of the critical conditions in obstetrics is HELP syndrome. Its consonance with the English word “help” is not accidental. Identification of signs of this disorder is most often recorded in the last trimester or in the first week after birth and requires intensive care and hospitalization of the patient. Several serious violations occur at once, which often threaten not only the health of the child, but also the life of the mother.

HELLP syndrome during pregnancy is a rare pathology that manifests itself with serious hemodynamic disturbances and failure of normal liver function. The mortality rate of women in the absence of medical care reaches 100%. If a patient is diagnosed with such a disease, urgent delivery is required, otherwise both mother and child may die. If the syndrome has formed at a late stage of gestosis, they resort to drug stimulation. At earlier stages, a caesarean section is required. Otherwise the consequences are fatal.

Reasons for the development of the disease in pregnant women

HELLP syndrome in obstetrics has not been fully studied. The exact pathogenesis of its occurrence is unknown. Reasons that can trigger the development of complications include:

1. Autoimmune processes that lead to the destruction of the body's own cells. There is a decrease in the number of platelets and red blood cells, which is accompanied by serious hemodynamic disorders.
2. Congenital abnormalities of the functioning of the liver, consisting of failures in the production of enzymes.
3. Thrombosis of blood vessels of the hepatobiliary system.
4. Antiphospholipid syndrome is classified as a separate nosological entity, although in essence it is an autoimmune process. Excessive destruction of the lipid structures of the cell membranes of the body occurs by antibodies.

The development of HELP syndrome is common due to lack of attention to pregnancy complications, for example, preeclampsia. If a woman is not registered with a gynecologist and does not control her own health and the condition of the baby, such a disorder can progress. A direct connection between the disease and a serious increase in blood pressure has not been established. Moreover, the development of HELLP syndrome is often recorded simultaneously with eclampsia.

Risk factors

Some features of a woman’s body also predispose to the occurrence of pathology, such as:

1. First-time mothers rarely face this problem. But recurrence of gestosis can be complicated by HELP syndrome.
2. Multiple pregnancies more often lead to the formation of such disorders than the development of only one child in the uterus.
3. The patient has a history of severe chronic lesions of the cardiovascular system, liver and kidneys.
4. Age over 25 years is a risk factor for gestosis in relation to the further development of hemodynamic disorders.
5. HELP syndrome is more often recorded in women with fair skin than in dark-skinned patients.

Main symptoms

The clinical picture of the disease is associated with the main pathological processes occurring in the body. Decoding the abbreviation HELLP implies the formation of the following problems:

1. H - hemolysis. Hemolysis is the process of breakdown of red blood cells directly in the bloodstream.
2. EL – elevated liver enzymes. An increase in the level of liver enzymes accompanies serious dysfunction of the organ. An increase in enzyme concentration indicates the death of hepatocytes.
3. LP – low platelet levels. A decrease in the level of platelets - cells that stop bleeding. Such a problem can be either a consequence of the formation of pathological clots and destruction of structures in blood vessels, or it can occur due to insufficient production of platelets by the red bone marrow.

A similar cascade of reactions is accompanied by the following symptoms:

1. Nausea and vomiting normally occur with toxicosis in early pregnancy. However, with HELP syndrome, they can recur in the last trimester.
2. Migraine and dizziness are common symptoms that are often the first signal of the development of preeclampsia and other dangerous hemodynamic disorders.
3. At later stages, icteric staining of the mucous membranes appears. This is due to the active release of the pigment bilirubin, which is found in red blood cells and liver cells, into the blood.
4. The appearance of hematomas and petechiae at the site of minor injuries, such as abrasions or injections. Such a clinical sign indicates disturbances in the coagulation system.
5. The most severe symptom of HELP syndrome is the development of seizures. It is associated with a violation of oxygen transport to brain cells, since there is a decrease in the level of red blood cells that perform this function.

Diagnostics

After the symptoms of the disease appear, doctors have very little time left to save the woman and child. Significant deterioration and death may occur as early as 12 hours after the onset of clinical signs. The diagnosis is made on the basis of anamnesis and hematological tests, which reveal changes characteristic of the problem.

HELP syndrome in pregnant women requires visual diagnosis. Ultrasound allows you to assess the presence of organic damage to the liver and thrombosis of its vessels. Ultrasound examination of the fetus is also recommended.

The difficulty in confirming the occurrence of the disease comes down to the fact that diagnosis is often based on different criteria. Although there are special recommendations both for confirming HELLP syndrome and for its treatment, in many sources the authors refer to various pathological changes. Some argue that the diagnosis is made solely on the basis of characteristic abnormalities in the biochemical blood test, which include increased levels of liver enzymes and bilirubin. Others are inclined to believe that to confirm HELLP syndrome, a combination of pronounced severe preeclampsia with hematological parameters characteristic of this disorder is required. However, in a number of studies describing the problem, there was no indication of suspicion or confirmation of the presence of hemolysis in women with this disease. That is, in some patients, when the disorder develops, the breakdown of red blood cells in the bloodstream is completely absent.

Diagnosis of HELP syndrome requires an integrated approach, although one should focus not only on the clinical manifestations of the disease and the patient’s medical history, but also on the presence of characteristic abnormalities in laboratory tests.

Treatment methods

The problem in gynecology is considered an emergency, so special attention is paid to it in the educational process of doctors. Doctors either stimulate natural labor by administering appropriate medications, or resort to surgery to remove the fetus from the uterus.

Obstetric tactics depend on the time of development of gestosis:

1. If the period exceeds 34 weeks, then prostaglandins and epidural anesthesia are used, since the natural process is preferred. There is no point in waiting: a woman’s condition can worsen at any moment. In severe cases, the patient is placed in the intensive care unit.
2. When HELP syndrome is detected between 27 and 34 weeks, the mother’s condition is stabilized, as well as the fetus is prepared for a cesarean section. Indications for postponing surgery are eclampsia, the formation of disseminated intravascular coagulation, and bleeding.
3. If the pathology develops before 27 weeks, after the use of glucocorticoids, surgery is performed to adapt the baby’s underdeveloped lungs.

HELP syndrome can also occur after childbirth. In such cases, treatment is simplified by the fact that only the mother needs to be saved.

Complications

In the absence of medical care or non-compliance with doctors' recommendations, dysfunction of the mother's liver, kidneys and lungs occurs. The child suffers from developmental delays, respiratory distress syndrome and asphyxia. In 20% of cases, the fetus dies even with timely assistance if there are significant changes in the hemodynamics of the female body.

Recovery process after surgery

After delivery, monitoring of the patient's condition is required, since HELLP syndrome may develop later. Symptomatic treatment is carried out, hormonal drugs are used to normalize blood counts. The timing of a woman’s discharge from the hospital depends on her well-being and the health of the baby.

Prevention and prognosis

Despite the insignificant frequency of detection of HELP syndrome in pregnant women, much attention is paid to it. Preventing the formation of the disease comes down to following the rules of a healthy lifestyle and timely consultation with a doctor. The prognosis depends on the duration of gestosis, as well as on the presence of chronic diseases in the woman.

HELLP syndrome (abbreviation from English: H - hemolysis - hemolysis, EL - elevated liver enzymes - increased activity of liver enzymes, LP - low platelet count - thrombocytopenia) is a variant of severe preeclampsia, characterized by the presence of hemolysis of red blood cells, increased levels of liver enzymes and thrombocytopenia. This syndrome occurs in 4-12% of women with severe preeclampsia. Severe arterial hypertension does not always accompany HELLP syndrome; The degree of hypertension rarely reflects the severity of the woman’s condition as a whole. HELLP syndrome is most common in primigravidas and multiparous women, and is also associated with a high rate of perinatal mortality.

Criteria for HELLP syndrome (presence of all of the following criteria).
Hemolysis:
- pathological blood smear with the presence of fragmented red blood cells;
- lactate dehydrogenase level >600 IU/l;
- bilirubin level >12 g/l.

Increased liver enzyme levels:
- aspartate aminotransferase >70 IU/l.

Thrombocytopenia:
- platelet count
HELLP syndrome may be accompanied by mild symptoms of nausea, vomiting, and pain in the epigastric region/upper outer quadrant of the abdomen, and therefore diagnosis of this condition is often delayed.

Severe epigastric pain that is not relieved by taking antacids should cause high suspicion. One of the characteristic symptoms (often late) of this condition is the syndrome of “dark urine” (the color of Coca-Cola).

The clinical picture of HELLP syndrome is variable and includes the following symptoms:
- pain in the epigastric region or right upper quadrant of the abdomen (86-90%);
- nausea or vomiting (45-84%);
- headache (50%);
- sensitivity to palpation in the right upper quadrant of the abdomen (86%);
- distolic blood pressure above 110 mmHg. (67%);
- massive proteinuria >2+ (85-96%);
- swelling (55-67%);
- arterial hypertension (80%).Epidemiology

The frequency of HELLP syndrome in the general population of pregnant women is 0.50.9%, and in severe preeclampsia and eclampsia - 10-20% of cases. In 70% of cases, HELLP syndrome develops during pregnancy (in 10% - before 27 weeks, in 50% - 27-37 weeks, and in 20% - after 37 weeks).

In 30% of cases, HELLP syndrome manifests itself within 48 hours after birth.

In 10-20% of cases, HELLP syndrome is not accompanied by arterial hypertension and proteinuria, which once again indicates more complex mechanisms of its formation. Excessive weight gain and edema precede the development of HELLP syndrome in 50% of pregnant women. HELLP syndrome is one of the most severe types of liver damage and acute liver failure associated with pregnancy: perinatal mortality reaches 34%, and mortality in women up to 25%. Depending on the set of symptoms, complete HELLP syndrome and its partial forms are distinguished: in the absence of hemolytic anemia, the developed symptom complex is designated as ELLP syndrome, and only in the case of thrombocytopenia - LP syndrome. Partial HELLP syndrome, in contrast to complete syndrome, is characterized by a more favorable prognosis. In 80-90%, severe gestosis (preeclampsia) and HELLP syndrome are combined with each other and are considered as a single whole.

Pathogenesis

The pathogenesis of HELLP syndrome has much in common with the pathogenesis of preeclampsia, DIC syndrome and antiphospholipid syndrome:
- violation of vascular tone and permeability (vasospasm, capillary leakage);
- activation of neutrophils, imbalance of cytokines (IL-10, IL-6 receptor, and TGF-β3 are increased, and CCL18, CXCL5, and IL-16 are significantly decreased);
- fibrin deposition and microthromb formation in microcirculation vessels;
- increase in plasminogen activator inhibitors (PAI-1);
- disturbance of fatty acid metabolism [deficiency of long chain 3-hydroxyacyl-CoA dehydrogenase], characteristic of fatty hepatosis. Of great importance in the development of HELLP syndrome is antiphospholipid syndrome and other variants of thrombophilias, various genetic abnormalities that also play a role in the development of preeclampsia. In total, 178 genes have been identified that are related to preeclampsia and HELLP syndrome. HELLP syndrome can recur in subsequent pregnancies with a frequency of 19%.

Diagnostics
Signs of HELLP syndrome include abdominal pain as a manifestation of stretching of the liver capsule and intestinal ischemia, an increase in fibrin/fibrinogen degradation products as a reflection of disseminated intravascular coagulation, a decrease in hemoglobin levels, metabolic acidosis, an increase in the level of indirect bilirubin, lactate dehydronase and the detection of red blood cell debris (schizocytes). ) in a blood smear, as a reflection of hemolysis. Hemoglobinemia and hemoglobinuria are macroscopically detected in only 10% of patients with HELLP syndrome. An early and specific laboratory sign of intravascular hemolysis is a low haptoglobin content (less than 1.0 g/l).

The most important predictors and criteria for the severity of HELLP syndrome include thrombocytopenia, the progression and severity of which directly correlates with hemorrhagic complications and the severity of DIC. The severity of acute liver failure and hepatic encephalopathy is assessed using generally accepted scales.

Complications for the mother:
- DIC syndrome 5-56%;
- placental abruption 9-20%;
- acute renal failure 7-36%;
- massive ascites 4-11%,
- pulmonary edema in 3-10%.
- intracerebral hemorrhages from 1.5 to 40%. Less common are eclampsia 4-9%, cerebral edema 1-8%, subcapsular hematoma of the liver 0.9-2.0% and liver rupture 1.8%.

Perinatal complications:
- fetal development delay 38-61%;
- premature birth 70%;
- thrombocytopenia of newborns 15-50%;
- acute respiratory distress syndrome 5.7-40%.

Perinatal mortality ranges from 7.4 to 34%. HELLP syndrome is quite complex. Diseases with which the differential diagnosis must be differentiated from HELLP syndrome include gestational thrombocytopenia, acute fatty liver, viral hepatitis, cholangitis, cholecystitis, urinary tract infection, gastritis, gastric ulcer, acute pancreatitis, immune thrombocytopenia, folic acid deficiency, systemic lupus erythematosus, antiphospholipid syndrome, thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome. Management of childbirth. Treatment

The clinical picture of HELLP syndrome can unfold rapidly and it is necessary to be prepared for a wide variety of course options. In principle, there are three options for treatment tactics in patients with HELLP syndrome.
If the pregnancy is more than 34 weeks, urgent delivery is required. The choice of method of delivery is determined by the obstetric situation.
At a gestational age of 27-34 weeks, in the absence of life-threatening signs, it is possible to prolong pregnancy for up to 48 hours to stabilize the woman’s condition and prepare the fetal lungs with corticosteroids. The method of delivery was cesarean section.
If the gestational age is less than 27 weeks and there are no life-threatening signs (see above), it is possible to prolong pregnancy to 48-72 hours. In these conditions, corticosteroids are also used. The method of delivery was cesarean section. HUS - hemolytic-uremic syndrome; TTP - thrombotic thrombocytopenic purpura; SLE - systemic lupus erythematosus; APS - antiphospholipid syndrome; AHF - acute fatty hepatosis of pregnancy.

Drug therapy is carried out by an anesthesiologist-resuscitator. Corticosteroid therapy in women with HELLP syndrome (betamethasone 12 mg every 24 hours, dexamethasone 6 mg every 12 hours, or high-dose dexamethasone 10 mg every 12 hours) used either before or after delivery has not been shown to be effective in preventing maternal and perinatal complications of HELLP syndrome. The only effects of corticosteroids are an increase in the woman's platelet count and a lower incidence of severe RDS in newborns. Corticosteroids are prescribed when the platelet count is less than 50,0009/L.

Therapy for preeclampsia. When HELLP syndrome develops against the background of severe preeclampsia and/or eclampsia, magnesium sulfate therapy at a dose of 2 g/hour intravenously and antihypertensive therapy are mandatory for blood pressure above 160/110 mm Hg. Treatment for gestosis (preeclampsia) should continue for at least 48 hours after delivery.

Correction of coagulopathy. Replacement therapy with blood components (cryoprecipitate, packed red blood cells, platelet mass, recombinant factor VII, prothrombin complex concentrate) will be required in 3293% of cases of patients with HELLP syndrome complicated by bleeding and disseminated intravascular coagulation. The absolute indication for replacement therapy with blood components and blood coagulation factors (concentrates) is the sum of points on the scale for diagnosing overt DIC syndrome of more than 5 points.

If coagulopathic bleeding develops, therapy with anti-fibrinolytics (tranexamic acid 15 mg/kg) is indicated. The use of heparin is contraindicated. If the platelet count is more than 50*109/l and there is no bleeding, prophylactic platelet mass is not transfused. Indications for platelet transfusion arise when the platelet count is less than 20*109/l and the upcoming delivery. To restore the synthesis of prothrombin complex factors in the liver, vitamin K 2-4 ml is used.

To stop bleeding, the benefits of clotting factor concentrates are used:
- the possibility of immediate administration, which allows you to anticipate the introduction of an effective dose of fresh frozen plasma (15 ml/kg) by almost 1 hour;
- immunological and infectious safety;
- the number of replacement therapy drugs decreases (cryoprecipitate, platelet mass, red blood cells).
- reduction in the incidence of post-transfusion lung damage.

There is no evidence base regarding the hemostatic effect of sodium etamsylate, vikasol and calcium chloride.

Infusion therapy. It is necessary to correct electrolyte disturbances with polyelectrolyte balanced solutions; with the development of hypoglycemia, an infusion of glucose solutions may be required; for hypoalbuminemia less than 20 g/l, an albumin infusion of 10% - 400 ml, 20% - 200 ml; for arterial hypotension, synthetic colloids (modified gelatin). Monitoring the rate of diuresis and assessing the severity of hepatic encephalopathy is necessary to prevent cerebral edema and pulmonary edema.

In general, against the background of severe preeclampsia, infusion therapy is restrictive - crystalloids up to 40-80 ml/h. With the development of massive intravascular hemolysis, infusion therapy has its own characteristics, outlined below.

Treatment of massive intravascular hemolysis. When a diagnosis of massive intravascular hemolysis (free hemoglobin in the blood and urine) is made and immediate hemodialysis is not possible, conservative tactics can ensure the preservation of kidney function. With preserved diuresis - more than 0.5 ml/kg/h and pronounced metabolic acidosis - pH less than 7.2, the administration of 4% sodium bicarbonate 200 ml is immediately started to stop metabolic acidosis and prevent the formation of hydrochloric acid hematin in the lumen of the renal tubules.

Next, intravenous administration of balanced crystalloids (sodium chloride 0.9%, Ringer's solution, Sterofundin) is started at the rate of 60-80 ml/kg body weight, with an administration rate of up to 1000 ml/h. In parallel, diuresis is stimulated with saluretics - furosemide 20-40 mg divided intravenously to maintain the diuresis rate to 150-200 ml/h. An indicator of the effectiveness of the therapy is a decrease in the level of free hemoglobin in the blood and urine. Against the background of such infusion therapy, the course of preeclampsia may worsen, but, as experience shows, such tactics will avoid the formation of acute tubular necrosis and acute pyelonephritis. With the development of arterial hypotension, an intravenous infusion of synthetic colloids (modified gelatin) in a volume of 500-1000 ml begins, and then an infusion of norepinephrine 0.1 to 0.3 mcg/kg/min or dopamine 5-15 mcg/kg/h to maintain systolic blood pressure more than 90 mmHg.

In dynamics, the color of urine, the content of free hemoglobin in the blood and urine, and the rate of diuresis are assessed. If oliguria is confirmed (diuresis rate less than 0.5 ml/kg/h within 6 hours after the start of infusion therapy, stabilization of blood pressure and stimulation of diuresis with 100 mg furosemide), an increase in creatinine level by 1.5 times, or a decrease in glomerular filtration rate >25% (or already developing renal dysfunction and failure), it is necessary to limit the volume of injected fluid to 600 ml/day and begin renal replacement therapy (hemofiltration, hemodialysis).

Method of anesthesia during delivery. In case of coagulopathy: thrombocytopenia (less than 100*109), deficiency of plasma coagulation factors, surgical delivery must be carried out under general anesthesia, using drugs such as ketamine, fentanyl, sevoflurane.

HELLP syndrome is an interdisciplinary problem and issues of diagnosis and treatment involve doctors of various specialties: obstetrician-gynecologist, anesthesiologist-resuscitator, surgeon, doctors of hemodialysis departments, gastroenterologist, transfusiologist. Difficulties in diagnosis, the symptomatic nature of treatment, and the severity of complications determine high rates of maternal (up to 25%) and perinatal (up to 34%) mortality. The only radical and effective treatment for HELLP syndrome is still only delivery, and therefore it is so important to promptly identify and take into account its slightest clinical and laboratory manifestations (especially progressive thrombocytopenia) during pregnancy.

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 includes an intravascular threatening disease 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 with such an unpleasant symptom as: 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 can occur, called which aggravate pain in the epigastric region, provoke the appearance of 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.

1. From the side of the central nervous system. The nervous system reacts to these disturbances with convulsions, intense headaches, and visual disturbances.
2. The functioning of the cardiovascular system is disrupted due to tissue swelling and decreased blood circulation.
3. Respiratory processes are generally not affected, but pulmonary edema may occur after childbirth.
4. On the part of hemostasis, thrombocytopenia and disruption of the functional component of platelet function are noted.
5. Decreased liver function, sometimes death of its cells. Rarely observed spontaneously, which entails death.
6. Disorders of the genitourinary system: oliguria, renal dysfunction.

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;
• diseases 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, preventive measures are eliminated and 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.

The term HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) - hemolysis, increased activity of liver enzymes (enzymes) and thrombocytopenia - is associated with an extremely severe form of preeclampsia and eclampsia. Back in 1893, G. Schmorl described the characteristic clinical picture of this syndrome, and the term HELLP (taking into account pathogenesis) was proposed by L. Weinstein (1985).

M.V. Mayorov, antenatal clinic of city clinic No. 5, Kharkov

Domestic literature contains very little information about HELLP syndrome, most often limited to brief mentions. This topic was considered in more detail by the luminaries of Russian anesthesiology and resuscitation A.P. Zilber and E.M. Shifman, as well as the chief obstetrician-gynecologist of the Ministry of Health of Ukraine V.V. Kaminsky.

Sad as it may be, inexorable statistics indicate the annual death of approximately 585 thousand women in the world, in one way or another related to pregnancy and childbirth. The main causes of maternal mortality in our country are: obstetric sepsis, bleeding, gestosis, as well as extragenital diseases. In severe forms of gestosis, HELLP syndrome accounts for 4 to 12% of cases and is characterized by high maternal mortality (according to various authors, from 24 to 75% of cases).

A consequence of the lack of knowledge about the clinical and laboratory manifestations of the described symptom complex in recent years is the overdiagnosis of HELLP syndrome. The clinical course of severe forms of preeclampsia can be very diverse. That is why the diagnosis of severe gestosis with HELLP syndrome is often erroneous. In reality, the described pathology may hide hepatitis, fatty hepatosis of pregnancy, hereditary thrombocytopenic purpura, etc. Often, “under the guise” of HELLP syndrome, obstetric sepsis or other pathology remains unrecognized.

Consequently, the detection of a triad in pregnant women - hemolysis, hepatic hyperenzymemia and thrombocytopenia - should not yet mean the immediate establishment of an unconditional diagnosis of “HELLP syndrome”. Only a careful and thoughtful clinical and physiological interpretation of these symptoms in each specific case allows us to differentiate it as a form of preeclampsia, which in advanced cases is a variant of severe multiple organ failure.

Differential diagnosis of HELLP syndrome, according to V.V. Kaminsky et al. , should be carried out with the following diseases:

• uncontrollable vomiting of pregnant women (in the first trimester);
• intrahepatic cholestasis (in the first trimester of pregnancy);
• cholelithiasis (at any stage of pregnancy);
• Dabin-Johnson syndrome (in the 2nd or 3rd trimester);
• acute fatty liver degeneration of pregnant women;
• viral hepatitis;
• drug-induced hepatitis;
• chronic liver disease (cirrhosis);
• Budd-Chiari syndrome;
• urolithiasis;
• gastritis;
• idiopathic thrombocytopenic purpura;
• hemolytic uremic syndrome;
• systemic lupus erythematosus.

Most researchers consider HELLP syndrome as a complication or as an atypical variant of gestosis, believing that it is based on generalized arteriolospasm, combined with hemoconcentration and hypovolemia, the development of a hypokinetic type of blood circulation, endothelial damage and the occurrence of respiratory failure, including pulmonary edema.

In typical cases, HELLP syndrome develops in multiparous women with preeclampsia, over the age of 25 years, with a burdened obstetric history. Clinical manifestations occur in 31% of cases before delivery; in the postpartum period - in 69% of cases.

Quite convincing is the point of view that pregnancy is a case of allotransplantation, and HELLP syndrome as an autoimmune reaction manifests itself as an exacerbation in the postpartum period. The autoimmune mechanism of endothelial damage, hypovolemia with blood thickening and the formation of microthrombi with subsequent fibrinolysis (disseminated intravascular coagulation (DIC)) are the main stages of the development of HELLP syndrome in severe forms of gestosis.

The destruction of platelets leads to the release of thromboxanes and imbalance of the thromboxane-prostacyclin system, which causes: generalized spasm of arterioles with increased arterial hypertension (AH), cerebral edema and convulsions; deterioration of uteroplacental blood flow; increased platelet aggregation, fibrin and red blood cell deposition, mainly in the placenta, kidneys and liver. These changes cause profound dysfunction of these organs, creating a vicious circle that can only be broken at a certain stage by terminating the pregnancy.

HELLP syndrome is characterized by the presence of multiple organ disorders, in particular from:

• Central nervous system: headache, blurred vision, hyperreflexia, convulsions. The cause of these disorders is vasospasm and hypoxia, and not cerebral edema, as previously thought.
• Respiratory system: the lungs remain intact for a long time. Edema of the upper respiratory tract and pulmonary edema may develop (usually after delivery). The development of respiratory distress syndrome is often observed.
• Of cardio-vascular system: generalized arteriolospasm leads to a decrease in circulating blood volume and tissue edema. The total peripheral vascular resistance and stroke volume increase, as a result of which the load on the left ventricle increases. Against this background, the development of diastolic dysfunction is possible.
• Hemostasis systems: Thrombocytopenia, as well as qualitative disorders of platelet function, are common. In severe cases, the development of disseminated intravascular coagulation syndrome is often observed.
• Liver: there is a decrease in the activity of liver enzymes with an increase in their level in the serum; areas of ischemia and even necrosis may develop. Spontaneous liver rupture is rare, but its outcome is almost always fatal.
• Kidney: Proteinuria indicates vascular damage to the glomerulus. Oliguria is more often associated with hypovolemia and reduced renal blood flow. Preeclampsia often progresses to acute renal failure.

Clinical signs and symptoms include complaints of spontaneous pain and tenderness on palpation in the epigastrium and right hypochondrium, jaundice, hyperbilirubinemia, proteinuria, hematuria, hypertension, anemia, nausea, vomiting; There may be hemorrhages at the injection sites.

For diagnosing HELLP syndrome The following standard laboratory data are required:

• hemolysis (determined by analyzing a peripheral blood smear);
• increased bilirubin content;
• increased levels of lactate dehydrogenase;
• elevated levels of alanine aminotransferase and aspartate aminotransferase;
• low platelet count (<100х10 9 /л).

In some cases, not the entire complex of classical signs of HELLP syndrome appears. Then, in the absence of hemolysis of erythrocytes, the name “ELLP syndrome” is used, in the absence of thrombocytopenia – “HEL syndrome”. It should be remembered that in 15% of patients with HELLP syndrome, hypertension may be absent or insignificant.

When carrying out differential diagnosis, it is necessary to take into account that thrombocytopenia and impaired liver function reach a maximum in HELLP syndrome 24-48 hours after birth, and in typical severe gestosis, on the contrary, there is a positive dynamics of these indicators during the first days of the postpartum period. Timely diagnosis of HELLP syndrome significantly improves the results of its intensive care. At the same time, according to A.P. Zilber (1999), sometimes slight thrombocytopenia or a moderate increase in the activity of liver enzymes in pregnant women “excites medical passions to diagnose HELLP syndrome.”

Early recognition of HELLP syndrome plays a very important role in preventing possible serious consequences for the life of the mother and child in the future. Treatment is carried out by an obstetrician-gynecologist together with an anesthesiologist-resuscitator, and if necessary, related specialists are involved - an ophthalmologist, a neurologist, etc.

V.V. Kaminsky et al. A detailed and clear algorithm of actions has been developed after the diagnosis of “HELLP syndrome” has been established, which allows us to answer the question: what to do? This algorithm includes:

• elimination of decompensation of multiple organ failure;
• it is possible to completely stabilize the patient’s condition;
• prevention of possible complications for the mother and fetus;
• delivery.

It should be remembered that the only pathogenetic treatment method is termination of pregnancy, i.e. delivery. Most authors emphasize that when a diagnosis of HELLP syndrome is made, pregnancy should be terminated within 24 hours, regardless of its duration. All other organizational and therapeutic measures are essentially preparation for delivery, which must be urgent, because during childbirth, as a rule, the severity of gestosis increases.

The method of delivery for a “mature” cervix is ​​through the natural birth canal, otherwise - cesarean section. After the birth of the placenta, curettage of the uterine cavity is required.

It is necessary to constantly remember possible complications of HELLP syndrome, which is fraught with maternal mortality:

• DIC syndrome and uterine bleeding;
• placental abruption;
• acute hepatic-renal failure;
• pulmonary edema;
• pleural effusion (exudative pleurisy);
• respiratory distress syndrome;
• subcapsular hematoma of the liver with its rupture and intra-abdominal bleeding;
• retinal disinsertion;
• cerebral hemorrhage.

On the part of the fetus, intrauterine growth retardation and intrauterine death are observed; newborns often develop bleeding and cerebral hemorrhages.

The main goals of pathogenetic therapy for HELLP syndrome: elimination of hemolysis and thrombotic microangiopathy, prevention of multiple organ multisystem dysfunction syndrome, optimization of neurological status and excretory function of the kidneys, normalization of blood pressure.

Intensive preoperative preparation, as well as intensive therapy after delivery, are aimed at many pathogenetic links and include the following components:

• strictly individualized antihypertensive therapy;
• reduction of hypovolemia, hypoproteinemia, intravascular hemolysis;
• correction of metabolic acidosis;
• appropriate infusion and transfusion therapy;
• antispasmodics, antiplatelet agents;
• stabilization of hemostasis indicators;
• rheocorrection of blood (anticoagulants and antiplatelet agents, in particular low molecular weight heparins [fraxiparin], pentoxifylline [trental], etc.);
• antibiotic therapy to prevent infectious complications (aminoglycosides are excluded, given their nephro- and hepatotoxicity);
• hepatostabilizing therapy, in particular large doses of glucocorticosteroids - until hepatic cytolysis is stabilized and thrombocytopenia is eliminated;
• protease inhibitors (contrical, gordox, trasylol);
• hepatoprotectors, cerebroprotectors and nootropics, vitamin complex (in high doses);
• magnesium therapy - according to the classical obstetric scheme;
• according to appropriate indications - plasmapheresis, hemodialysis.

For delivery, exclusively endotracheal anesthesia with minimal use of hepatotoxic anesthetics is recommended, as well as prolonged artificial ventilation in the postoperative period with intensive differentiated therapy.

The list of references is in the editorial office