Help syndrome in pregnant women and its treatment. Help syndrome (hellp syndrome) is a dangerous complication in the third trimester of pregnancy: causes, diagnosis, treatment Reasons for the development of the disease in pregnant women

HELLP syndrome is a rare and dangerous pathology in obstetrics. The first letters of the abbreviated name of the syndrome indicate the following: H - hemolysis (hemolysis); EL - elevated liver enzymes (increased activity of liver enzymes); LP - 1ow platelet count (thrombocytopenia). This syndrome was first described in 1954 by J.A. Pritchard, and R.S. Goodlin et al. (1978) associated the manifestation of this syndrome with preeclampsia. In 1982, L. Weinstein for the first time combined the triad of symptoms with a special pathology - HELLP syndrome.

EPIDEMIOLOGY

In severe cases of gestosis, HELLP syndrome, in which high maternal (up to 75%) and perinatal (79 cases per 1000 children) mortality is noted, is diagnosed in 4–12% of cases.

CLASSIFICATION OF HELLP SYNDROME

Based on laboratory signs, some authors have created a classification of HELLP syndrome.

P.A Van Dam et al. Patients are divided according to laboratory parameters into 3 groups: with obvious, suspected and hidden signs of intravascular coagulation.

The classification of J.N. is based on a similar principle. Martin, in which patients with HELLP syndrome are divided into two classes.
— First class - the platelet count in the blood is less than 50×109/l.
- Second class - the concentration of platelets in the blood is 50–100×109/l.

ETIOLOGY OF HELLP SYNDROME

To date, the true cause of the development of HELLP syndrome has not been identified, but some aspects of the development of this pathology have been clarified.

Possible causes of the development of HELLP syndrome are noted.
Immunosuppression (depression of T-lymphocytes and B-lymphocytes).
Autoimmune aggression (antiplatelet, antiendothelial antibodies).
Decrease in the prostacyclin/thromboxane ratio (decreased production of prostacyclin-stimulating factor).
Changes in the hemostasis system (liver vascular thrombosis).
AFS.
Genetic defects of liver enzymes.
Use of medications (tetracycline, chloramphenicol).

The following risk factors for the development of HELLP syndrome are identified.
Light skin.
The pregnant woman is over 25 years old.
Multiparous women.
Multiple pregnancy.
The presence of severe somatic pathology.

PATHOGENESIS

The pathogenesis of HELLP syndrome is currently not fully understood (Fig. 34-1).

Rice. 34-1. Pathogenesis of HELLP syndrome.

The main stages of the development of HELLP syndrome in severe forms of gestosis are considered to be autoimmune damage to the endothelium, hypovolemia with blood thickening and the formation of microthrombi with subsequent fibrinolysis. When the endothelium is damaged, platelet aggregation increases, which, in turn, contributes to the involvement of fibrin, collagen fibers, the complement system, I- and I-M in the pathological process. Autoimmune complexes are found in the sinusoids of the liver and in the endocardium. In this regard, it is advisable to use glucocorticoids and immunosuppressants for HELLP syndrome. Destruction of platelets leads to the release of thromboxanes and imbalance in the thromboxane-prostacyclin system, generalized arteriolospasm with worsening hypertension, cerebral edema and seizures. A vicious circle develops, which is currently only possible to break through emergency delivery.

Preeclampsia is considered a syndrome of MODS, and HELLP syndrome is its extreme degree, which is a consequence of maladaptation of the mother's body when trying to ensure the normal functioning of the fetus. Macroscopically, with HELLP syndrome, an increase in the size of the liver, thickening of its consistency, and subcapsular hemorrhages are noted. The color of the liver becomes light brown. Microscopic examination reveals periportal hemorrhages, fibrin deposits, I-M, I- in the liver sinusoids, multilobular necrosis of hepatocytes.

CLINICAL PICTURE (SYMPTOMS) OF HELLP SYNDROME

HELLP syndrome usually occurs in the third trimester of pregnancy, most often at 35 weeks or more. The disease is characterized by a rapid increase in symptoms. Initial manifestations are nonspecific: nausea and vomiting (in 86% of cases), pain in the epigastric region and, especially, in the right hypochondrium (in 86% of cases), severe swelling (in 67% of cases), headache, fatigue, malaise, motor anxiety, hyperreflexia.

Characteristic signs of the disease are jaundice, vomiting blood, hemorrhages at injection sites, increasing liver failure, convulsions and severe coma. The most common clinical signs of HELLP syndrome are presented in table. 34-1.

DIAGNOSIS OF HELLP SYNDROME

LABORATORY RESEARCH

Quite often, laboratory changes occur much earlier than clinical manifestations.

One of the main laboratory symptoms of HELLP syndrome is hemolysis, which is manifested by the presence of wrinkled and deformed red blood cells and polychromasia in the blood smear. The destruction of red blood cells leads to the release of phospholipids and to intravascular coagulation, i.e. chronic disseminated intravascular coagulation syndrome, which can cause fatal obstetric hemorrhage.

If HELLP syndrome is suspected, it is necessary to immediately conduct laboratory tests, including determining the activity of ALT, AST, lactate dehydrogenase, the concentration of bilirubin, haptoglobin, uric acid, the number of platelets in the blood and assessing the state of the blood coagulation system. The fundamental criteria for diagnosing HELLP syndrome are laboratory parameters (Table 34-2).

Table 34-1. Clinical picture of HELLP syndrome

Signs	HELLP syndrome
Pain in the epigastric region and/or right hypochondrium	+++
Headache	++
Jaundice	+++
AG	+++/–
Proteinuria (more than 5 g/day)	+++/–
Peripheral edema	++/–
Vomit	+++
Nausea	+++
Brain or visual disturbances	++/–
Oliguria (less than 400 ml/day)	++
Acute tubular necrosis	++
Cortical necrosis	++
Hematuria	++
Panhypopituitarism	++
Pulmonary edema or cyanosis	+/–
Weakness, fatigue	+/–
Stomach bleeding	+/–
Bleeding at injection sites	+
Increasing liver failure	+
Hepatic coma	+/–
Convulsions	+/–
Ascites	+/–
Fever	++/–
Itchy skin	+/–
Weight loss	+

Note: +++, ++, +/– - severity of manifestations.

Table 34-2. Laboratory data

Laboratory indicators	Changes in HELLP syndrome
Content of leukocytes in blood	Within normal limits
Activity of aminotransferases in the blood (ALT, AST)	Increased to 500 units (normal is up to 35 units)
ALP activity in the blood	Marked increase (3 times or more)
Bilirubin concentration in blood	20 µmol/l or more
ESR	Reduced
The number of lymphocytes in the blood	Normal or slight decrease
Blood protein concentration	Reduced
Blood platelet count	Thrombocytopenia (less than 100×109/l)
The nature of red blood cells in the blood	Altered red blood cells with Barr cells, polychromasia
Number of red blood cells in the blood	Hemolytic anemia
Prothrombin time	Increased
Blood glucose concentration	Reduced
Clotting factors	Consumptive coagulopathy: decreased content of factors for the synthesis of which vitamin K is required in the liver, decreased concentration of antithrombin III in the blood
Concentration of nitrogenous substances in the blood (creatinine, urea)	Promoted
Haptoglobin content in blood	Reduced

INSTRUMENTAL RESEARCH

For early detection of subcapsular hematoma of the liver, ultrasound of the upper abdomen is indicated. Ultrasound of the liver in pregnant women with severe gestosis complicated by HELLP syndrome also reveals multiple hypoechoic areas, which are regarded as signs of periportal necrosis and hemorrhage (hemorrhagic liver infarction).

CT and MRI are used for differential diagnosis of HELLP syndrome.

DIFFERENTIAL DIAGNOSTICS

Despite the difficulties in diagnosing HELLP syndrome, there are a number of signs characteristic of this nosology: thrombocytopenia and impaired liver function. The severity of these disorders reaches a maximum 24–48 hours after birth, while in severe gestosis, on the contrary, regression of these indicators is observed during the first day of the postpartum period.

Signs of HELLP syndrome can also be present in other pathological conditions besides gestosis. Differential diagnosis of this condition with hemolysis of erythrocytes, increased activity of liver enzymes in the blood and thrombocytopenia that develops with the following diseases is necessary.

Cocaine addiction.
Systemic lupus erythematosus.
Thrombocytopenic purpura.
Hemolytic uremic syndrome.
Acute fatty hepatosis of pregnant women.
Viral hepatitis A, B, C, E.
CMV and infectious mononucleosis.

The clinical picture of liver damage during pregnancy is often erased and doctors sometimes consider the symptoms described above as a manifestation of another pathology.

INDICATIONS FOR CONSULTATION WITH OTHER SPECIALISTS

Consultations with a resuscitator, hepatologist, and hematologist are indicated.

EXAMPLE OF FORMULATION OF DIAGNOSIS

Pregnancy 36 weeks, cephalic presentation. Gestosis in severe form. HELLP syndrome.

TREATMENT OF HELLP SYNDROME

TREATMENT GOALS

Restoration of disturbed homeostasis.

INDICATIONS FOR HOSPITALIZATION

HELLP syndrome, as a manifestation of severe gestosis, in all cases serves as an indication for hospitalization.

NON-DRUG TREATMENT

Emergency delivery is carried out against the background of infusion-transfusion therapy under anesthesia.

DRUG TREATMENT

Along with infusion-transfusion therapy, protease inhibitors (aprotinin), hepatoprotectors (vitamin C, folic acid), lipoic acid 0.025 g 3-4 times a day, fresh frozen plasma at a dose of at least 20 ml/kg body weight per day, transfusion are prescribed platelet concentrate (at least 2 doses when the platelet count is less than 50×109/l), glucocorticoids (prednisolone at a dose of at least 500 mg/day intravenously). In the postoperative period, under the control of clinical and laboratory parameters, the administration of fresh frozen plasma is continued at a dose of 12–15 ml/kg body weight in order to replenish the content of plasma coagulation factors, and it is also recommended to carry out plasmapheresis in combination with replacement transfusion of fresh frozen plasma, elimination of hypovolemia, antihypertensive and immunosuppressive therapy. Mayenne et al. (1994) believe that the administration of glucocorticoids improves maternal outcome in women with preeclampsia and HELLP syndrome.

Pregnancy is a happy time for every woman. However, this joyful period may be overshadowed by the development of HELLP syndrome. Such a pathology requires urgent medical attention. How to recognize a dangerous condition and avoid negative consequences?

What is HELLP syndrome?

Doctors designate the pathology as a dangerous and severe complication of gestosis - late toxicosis in the last months of pregnancy. In obstetrics, the syndrome was named according to the basic symptoms that make up the clinical picture of the disease:

• H - hemolysis (decomposition of erythrocytes - red blood cells that deliver oxygen to all tissues of the body);
• EL - increased levels of liver enzymes, which may indicate a disease of this organ;
• LP - thrombocytopenia - decreased platelet formation and, as a result, poor blood clotting.

In addition, the syndrome causes multiple damage to the organs and body systems of a pregnant woman, thereby aggravating the course of pregnancy.

Although the pathology is quite dangerous, fortunately, it is rare. The disease is detected in 0.9% of pregnant women, and more often HELLP syndrome is diagnosed in those women who suffer from severe gestosis (from 4–12%).

The diagnosis of “HELLP syndrome” is made in 70% of cases in the third trimester of pregnancy (after 35 weeks) and in the first two weeks after birth.

Causes and risk factors

It is still not known exactly what causes the pathology. Obstetricians identify several probable reasons:

• taking tetracycline antibiotics;
• thrombosis - the formation of blood clots in arterial or venous vessels;
• destruction by the body of red blood cells (cells that are responsible for the delivery of oxygen) and platelets (bodies that influence blood clotting);
• hereditary liver diseases;
• severe form of gestosis (complications in the second half of pregnancy).

Studies have shown that the risk group includes women who suffered from HELLP syndrome in previous pregnancies. The probability that the situation will repeat itself is about 25%.

In addition, the development of pathology is influenced by:

• too pale skin;
• age of the expectant mother after 25 years;
• multiple pregnancy;
• severe autoimmune diseases.

Often the syndrome occurs in women whose pregnancy was difficult from the first days of conception. This is indicated by early toxicosis, high blood pressure, threat of failure, placental insufficiency and other undesirable conditions.

Clinical picture

For HELLP syndrome, the initial symptoms are nonspecific. A pregnant woman appears:

• headache;
• vomit;
• pain under the right rib;
• fatigue;
• severe swelling (in 67%);
• motor restlessness.

After some time, the following symptoms appear:

• yellowness of the skin;
• nausea and vomiting;
• convulsions;
• hematomas (bruising) at injection sites;
• visual disturbances;
• anemia;
• heart rhythm failure;
• increasing renal and liver failure.

In severe forms of the disease, the functioning of the brain centers is disrupted, brain swelling, and profound disruption of organ function occur, which can lead to coma. If several signs appear, you should immediately seek medical help.

Diagnostics

The following methods are used to diagnose pathology:

• Ultrasound of the upper abdomen;
• biochemical and clinical blood tests;
• MRI and CT.

A doctor can diagnose “HELLP syndrome” if research reveals:

• insufficient platelet count - less than 100 x 10 9 /l;
• reduced amount of protein and lymphocytes;
• increased level of bilirubin (bile pigment) - from 20 µmol or more;
• deformation and low erythrocyte (red blood cell) counts;
• increased concentration of urea and creatinine in the blood.

Timely detection of a dangerous condition increases the effectiveness of therapy and increases the chances of recovery.

HELLP syndrome should be distinguished from the following diseases:

• viral hepatitis;
• liver failures;
• liver pathologies;
• gastritis.

In severe cases of gestosis, as well as to clarify the diagnosis, the doctor may prescribe additional studies:

• Ultrasound of the liver and kidneys;
• Fetal ultrasound;
• Doppler ultrasound is a method for studying blood flow in the vessels of the placenta, uterus and child;
• cardiotocography - assessment of fetal heart rate.

HELLP syndrome is a severe pathological complication of gestosis, which requires professional treatment and observation in a hospital.

Obstetric tactics

If HELLP syndrome is confirmed, obstetricians follow a clear plan, which includes:

1. Possible stabilization of the pregnant woman's condition.
2. Prevention of complications for the expectant mother and fetus.
3. Normalization of blood pressure.
4. Delivery.

Doctors say that the only and correct method of treatment is a caesarean section or emergency birth (depending on the duration of pregnancy and the severity of the symptoms of the pathology).

Most obstetricians say that pregnancy should be terminated within 24 hours of diagnosis (regardless of the date).

All other therapeutic and organizational therapy is preparation for childbirth.

Drug therapy

Additionally, drug treatment is provided, which includes:

• plasmaphoresis - a procedure for cleaning plasma from aggressive substances;
• administration of fresh frozen plasma;
• transfusion of platelet concentrate.

The following are administered intravenously:

• protease inhibitors - substances that prevent protein breakdown;
• hepaprotectors - to improve the condition of the liver;
• glucocorticoids - hormones to stabilize the adrenal glands.

In the postoperative period the following is prescribed:

• fresh frozen plasma to normalize blood clotting;
• glucocorticoids;
• immunosuppressive and antihypertensive (to lower blood pressure) therapy.

Treatment prognosis

With early detection of pathology and timely provision of medical care, the prognosis is quite favorable. On days 3–7 after delivery, all blood parameters usually return to normal, with the exception of thrombocytopenia (special therapy is required).

The time spent in the hospital depends on the health of the mother and child, as well as the presence of complications.

Possible complications

The consequences of HELLP syndrome for mother and child are quite serious. That is why much attention is paid to solving this problem.

Possible complications in a pregnant woman - table

Possible complications in newborns - table

Prevention

To prevent the disease, expectant mothers are advised to:

• regularly take tests and visit a doctor;
• give up bad habits;
• lead a healthy lifestyle;
• register with the antenatal clinic in a timely manner;
• normalize physical activity;
• avoid stressful situations.

Gestosis in women during pregnancy - video

HELLP syndrome is a dangerous pathology that occurs in the second half of pregnancy and can lead to serious consequences. Only timely medical assistance and compliance with all recommendations will help the expectant mother avoid serious complications and give birth to a healthy baby.

A severe complication of pregnancy, which is characterized by a triad of symptoms: hemolysis, damage to the liver parenchyma and thrombocytopenia. Clinically manifested by rapidly increasing symptoms - pain in the liver and abdomen, nausea, vomiting, swelling, yellowness 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.

Reasons

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 DIC 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 test. 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 disease 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 administration 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 angiopathy, microthrombosis, hemolysis, influencing various parts of pathogenesis, restoring the function of the liver and other organs and systems is actively continued 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.

HELP syndrome is a rare pathology in women in late pregnancy. It is almost always discovered about a month before the onset of labor. In some women, signs of this syndrome appeared after childbirth. George Pritchard was the first to describe this pathology. It must be said that this rare syndrome occurs in only seven percent of women, but 75% of cases are fatal.

The name HELLP is an abbreviation of English words. Each letter is deciphered as follows:

• H – destruction of red blood cells.
• EL – increase in liver enzyme levels.
• LP - a decrease in the level of red blood cells, which serve for blood clotting.

In medical practice, namely in obstetrics, HELP syndrome is understood as some abnormalities in the female body that do not allow a woman to become pregnant or bear a healthy baby without pathologies.

Etiology

To date, the exact causes of this syndrome have not yet been clarified. However, scientists continue to put forward various theories of its origin. Today there are already more than thirty theories, but not one can point to the fact that affects the appearance of pathology. Experts have noticed one pattern - such a deviation appears against the background of something that appeared late.

A pregnant woman suffers from swelling, which begins in the arms and legs, then moves to the face, then to the whole body. The level of protein in the urine increases, and blood pressure also increases. This condition is extremely unfavorable for the fetus, because aggressive antibodies to it are formed in the mother’s body. They negatively affect red blood cells and platelets, destroying them. In addition, the integrity of blood vessels and liver tissue is compromised.

As mentioned earlier, HELP syndrome occurs for currently unknown reasons.

However, you can pay attention to some factors that increase the risk of pathology:

• diseases of the immune system;
• heredity, when there is a lack of enzymes in the liver, that is, a congenital pathology;
• changes in the number and purpose of lymphocytes;
• formation in the blood vessels of the liver;
• long-term use of medications without medical supervision.

When observing pathology, we can identify certain factors that are called provoking:

• numerous births in the past;
• the age of the woman in labor is more than twenty-five years;
• pregnancy with multiple fetuses.

The hereditary factor has not been established.

Classification

Based on exactly what signs HELP syndrome exhibits, some experts have created the following classification:

• obvious symptoms of intravascular thickening;
• suspect signs;
• hidden.

The classification of J. N. Martin has a similar principle: here the syndrome with the same name HELP is divided into two classes.

Symptoms

The first signs that appear are nonspecific, so it is impossible to diagnose the disease based on them.

A pregnant woman experiences symptoms such as:

• nausea;
• often vomiting;
• dizziness;
• pain in the side;
• causeless anxiety;
• fatigue;
• pain in the upper abdomen;
• change in skin color to yellow;
• the appearance of shortness of breath even with light exertion;
• blurred vision, brain activity, fainting.

The first manifestations are observed against the background of large edema.

During the rapid development of the disease or in the case when medical care was provided too late, it develops, appears, the process of urination is disrupted, convulsions occur, and body temperature rises. In some situations, a woman may fall into a coma. A doctor can make an accurate diagnosis of HELP syndrome only based on laboratory test results.

There is also a pathology that appears after the birth resolution. The risk of its development increases when a woman had severe late toxicosis during pregnancy. In addition, a cesarean section or difficult labor may also be triggers. If a woman in labor has previously experienced the symptoms described above, then she should be taken under close supervision. This should be done by the medical staff of the maternity hospital.

Diagnostics

If a doctor suspects a pregnant woman has such a disease, he must write her a referral for laboratory tests, such as:

• urine analysis - it can be used to find out the level and presence of protein, in addition, the functioning of the kidney is diagnosed;
• taking blood for analysis to find out the level of hemoglobin, platelets and red blood cells, and the bilirubin indicator is also important;
• ultrasound examination of the condition of the placenta, peritoneum, liver and kidneys;
• computed tomography to avoid making a false diagnosis that has similar symptoms;
• cardiotocography - determines the viability of the fetus and evaluates its heartbeat.

In addition to these studies, a visual examination of the patient and anamnesis is performed. The presence of signs such as yellow color of the skin, bruises from injections will help to more accurately establish the diagnosis.

Doctors often resort to the help of their colleagues working in other areas, for example, a resuscitator, gastroenterologist, or hepatologist.

When diagnosing this pathology, it is necessary to exclude the following diseases:

• exacerbation;
• different shapes (A, B, C);
• cocaine addiction;
• red and others.

Based on the diagnostic results, treatment tactics are determined.

Treatment

When a pregnant woman is diagnosed with HELP pathology, this is already an indication for urgent hospitalization. The main method of therapy is termination of pregnancy, because it is precisely because of this that this pathology arises.

However, there is a chance to save the child, because this condition in pregnant women manifests itself in the later stages, so the woman is induced for labor. In a situation where the uterus is ready and the gestation period is more than thirty-five weeks, a caesarean section is prescribed.

If the pregnancy is shorter, then the woman is prescribed glucocorticosteroids: they will help the fetal lungs open. But if there are signs such as heavy bleeding, high blood pressure, or bleeding in the brain, then an urgent caesarean section is necessary, and it does not matter at what stage of pregnancy the woman is. The therapy provided stabilizes the woman’s condition and helps restore the baby’s health after surgery.

If the therapy method is correct, then the mother’s health will improve within a couple of days after the operation.

After this, the doctor must:

• stabilize the patient's condition;
• treat with antibiotics to prevent infectious diseases;
• prescribe medications to normalize the functioning of the kidneys and liver, as well as to prevent the formation of blood clots;
• stabilize blood pressure.

Before the expectant mother undergoes a cesarean section, she may be prescribed a procedure such as plasmapheresis - plasma is removed from the blood, but only a clearly designated volume is used.

This is done with a special sterile, and disposable, device that separates the plasma. This is a harmless procedure that does not cause any discomfort to the woman. The duration takes approximately two hours. After this, a blood transfusion is necessary.

Also, in preparation for the operation and immediately after it, the woman is prescribed medications to reduce blood pressure, liver failure, and kidney failure.

Only complex therapy will help with this, which will include drugs such as:

• hormonal medications;
• means for stabilizing the functioning of the liver;
• medications that artificially reduce immunity.

After the operation, blood transfusion continues. The doctor also prescribes the use of lipoic and folic acid, vitamin C. If therapy is started on time and the operation is successful, then the prognosis is quite favorable. After delivery, all signs of pathology begin to disappear, however, relapse of the disease is high in all subsequent pregnancies.

Possible complications

The occurrence of complications from this pathology is quite common. Unfortunately, deaths cannot be ruled out. This applies not only to the mother, but also to the fetus.

The disease is dangerous due to the formation of blood clots and heavy bleeding in any location. In severe cases, hemorrhages may occur in the brain, and this is a malfunction of the central nervous system.

Disturbances in the kidneys and liver are also scary, because the consequences are such that the body is poisoned. Some cases of pathology end in coma, and it is not easy to bring a woman out of this state.

It should be noted that pathologies may also occur in the fetus, because with this syndrome occurs.

This disease causes the following symptoms in a woman:

• pain in the upper abdomen;
• a sharp decrease in blood pressure;
• dyspnea;
• severe weakness.

The fetus experiences oxygen starvation, which leads to deviations in development, height and weight. In addition, the mother’s emerging ailments lead to diseases of the baby’s nervous system. Such children suffer, lag behind in physical and mental development, in addition, they...

When one-third of the placenta is separated, the fetus dies.

Prevention

Even if the health of the expectant mother before pregnancy is excellent, there is still a risk of pathology.

Therefore, a woman must follow the following rules of prevention:

• consciously plan pregnancy, avoid unwanted conception;
• carrying out therapeutic actions in identifying any diseases;
• exercise more, maintain a clear daily routine;
• after establishing the fact of pregnancy, visit the doctor according to the schedule;
• regular health monitoring, that is, testing;
• timely treatment of late toxicosis;
• healthy eating;
• drinking the required amount of fluid per day;
• refuse heavy physical work, avoid;
• compliance with the work/rest schedule;
• Be sure to notify your doctor about taking medications for chronic diseases.

Self-medication is unacceptable.

HELLP syndrome is a rare and dangerous disease that complicates the course of pregnancy and is a consequence of severe gestosis with hypertension, edema and proteinuria. This obstetric pathology develops in the last trimester of pregnancy - at 35 or more weeks. HELLP syndrome can occur in women in labor who have a history of late toxicosis. Obstetricians pay special attention to young mothers from this category and actively monitor them for 2-3 days after birth.

The abbreviation HELLP syndrome in obstetrics stands for:

• H - hemolysis,
• EL - elevated levels of liver enzymes,
• LP - thrombocytopenia.

Pritchard first described the HELLP syndrome in 1954, and Goodlin and his co-authors in 1978 associated the manifestations of the pathology with gestosis in pregnant women. Weinstein in 1982 identified the syndrome as a separate nosological unit.

In pregnant women, the disease manifests itself with signs of damage to internal organs- liver, kidneys and blood system, dyspepsia, pain in the epigastrium and right hypochondrium, edema, hyperreflexia. The high risk of bleeding is due to dysfunction of the blood coagulation system. The body denies the very fact of childbirth, and an autoimmune failure occurs. When compensatory mechanisms fail, the pathology begins to rapidly progress: women’s blood stops clotting, wounds do not heal, bleeding does not stop, and the liver does not perform its functions.

HELLP syndrome is a rare disease diagnosed in 5–10% of pregnant women with severe gestosis. Providing emergency medical care helps prevent the development of dangerous complications. Timely consultation with a doctor and adequate therapy are necessary to save two lives at once.

Currently, HELP syndrome during pregnancy claims many lives. The mortality rate among women with severe gestosis remains quite high and amounts to 75%.

Etiology

Currently, the causes of the development of HELLP syndrome remain unknown to modern medicine. Among the possible etiopathogenetic factors of the disease are the following:

The high-risk group for the development of this pathology includes:

• Light-skinned women
• Pregnant women aged 25 years or more,
• Women who have given birth more than twice
• Pregnant women with multiple births,
• Patients with signs of severe psychosomatic pathology,
• Pregnant women with eclampsia.

Most scientists believe that late toxicosis occurs more severely in those women whose pregnancy developed unfavorably from the first weeks: there was a threat of miscarriage or there was fetoplacental insufficiency.

Pathogenesis

Pathogenetic links of HELLP syndrome:

1. Severe gestosis,
2. Production of immunoglobulins to red blood cells and platelets that bind foreign proteins,
3. Production of immunoglobulins to the vascular endothelium,
4. Autoimmune endothelial inflammation,
5. platelet aggregation,
6. Destruction of red blood cells,
7. Release of thromboxane into the bloodstream,
8. Generalized arteriolospasm,
9. cerebral edema,
10. Convulsive syndrome,
11. Hypovolemia with blood thickening,
12. Fibrinolysis,
13. Thrombosis,
14. The appearance of CEC in the capillaries of the liver and endocardium,
15. Damage to liver and heart tissue.

The pregnant uterus puts pressure on the organs of the digestive system, which leads to disruption of their functioning. Patients experience epigastric pain, nausea, heartburn, flatulence, vomiting, edema, and hypertension. The lightning-fast increase in such symptoms threatens the life of the woman and the fetus. This is how a syndrome with the characteristic name HELP develops.

HELLP syndrome is an extreme degree of gestosis in pregnant women, resulting from the inability of the mother’s body to ensure the normal development of the fetus.

Morphological signs of HELLP syndrome:

• Hepatomegaly,
• Structural changes in the liver parenchyma,
• Hemorrhages under the membranes of the organ,
• "Light" liver,
• Hemorrhages in periportal tissue,
• Polymerization of fibrinogen molecules into fibrin and its deposition in the liver sinusoids,
• Large nodular necrosis of hepatocytes.

HELP syndrome components:

The further development of these processes can only be stopped under stationary conditions. For pregnant women, they are especially dangerous and life-threatening.

Symptoms

The main clinical signs of HELP syndrome increase gradually or develop at lightning speed.

The initial symptoms include signs of asthenia of the body and hyperexcitation:

• Dyspeptic symptoms
• Pain in the hypochondrium on the right,
• Edema,
• Migraine,
• Fatigue,
• Heaviness in the head
• Weakness,
• Myalgia and arthralgia,
• Motor restlessness.

Many pregnant women do not take such signs seriously and often attribute them to a general malaise that is typical for all expectant mothers. If measures are not taken to eliminate them, the woman’s condition will rapidly deteriorate, and typical manifestations of the syndrome will appear.

Characteristic symptoms of the pathology:

1. Yellowness of the skin,
2. Bloody vomiting
3. Hematomas at the injection site,
4. Hematuria and oliguria,
5. Proteinuria,
6. Dyspnea,
7. Interruptions in the functioning of the heart,
8. confusion,
9. visual impairment,
10. Feverish state
11. Convulsive seizures
12. Coma.

If specialists do not provide medical assistance to the woman within 12 hours from the moment the first signs of the syndrome appear, life-threatening complications will develop.

Complications

Complications of pathology that develop in the mother’s body:

• Acute pulmonary failure,
• Persistent kidney and liver dysfunction,
• Hemorrhagic stroke,
• Rupture of hepatic hematoma,
• Hemorrhage into the abdominal cavity,
• Premature placental abruption,
• Convulsive syndrome,
• Disseminated intravascular coagulation syndrome,
• Fatal outcome.

Severe consequences that occur in the fetus and newborn:

1. Intrauterine growth restriction,
2. Suffocation,
3. Leukopenia,
4. Neutropenia,
5. Intestinal necrosis,
6. Intracranial hemorrhages.

Diagnostics

Diagnosis of the disease is based on complaints and anamnestic data, among which the main ones are pregnancy 35 weeks, gestosis, age over 25 years, severe psychosomatic diseases, multiple births, multiple births.

During the examination of the patient, specialists identify hyperexcitability, yellowness of the sclera and skin, hematomas, tachycardia, tachypnea, and edema. Hepatomegaly is detected by palpation. A physical examination consists of measuring blood pressure, conducting 24-hour blood pressure monitoring, and determining the pulse.

Laboratory research methods play a decisive role in the diagnosis of HELP syndrome.

Instrumental studies:

1. Ultrasound of the abdominal cavity and retroperitoneal space can detect subcapsular hematoma of the liver, periportal necrosis and hemorrhage.
2. CT and MRI are performed to determine the condition of the liver.
3. Fundus examination.
4. Ultrasound of the fetus.
5. Cardiotocography is a method for studying fetal heart rate and uterine tone.
6. Fetal Doppler - assessment of blood flow in the fetal vessels.

Treatment

Obstetrician-gynecologists, resuscitators, hepatologists, and hematologists treat HELP syndrome in pregnant women. The main therapeutic goals are: restoration of impaired homeostasis and functions of internal organs, elimination of hemolysis and prevention of thrombus formation.

Hospitalization is indicated for all patients with HELLP syndrome. Non-drug treatment consists of emergency delivery against the background of intensive care. The only way to prevent further progression of the disease is to terminate the pregnancy as soon as possible. For caesarean section, epidural anesthesia is used, and in severe cases, endotracheal anesthesia is used exclusively. If the uterus is mature, then childbirth takes place naturally with mandatory epidural anesthesia. A successful outcome of the operation is accompanied by a decrease in the severity of the main symptoms of the pathology. The hemogram data gradually returns to normal. Complete restoration of the normal platelet count occurs within 7-10 days.

Drug therapy is carried out before, during and after cesarean section:

Physiotherapeutic methods are relevant in the postoperative period. Women are prescribed plasmapheresis, ultrafiltration, hemosorption.

With adequate treatment, the woman’s condition normalizes 3-7 days after delivery. If you have HELLP syndrome, it is impossible to maintain a pregnancy. Timely diagnosis and pathogenetic therapy reduce the mortality rate from pathology by 25%.

Prevention

There is no specific prevention of HELP syndrome. Preventive measures to avoid the development of HELLP syndrome:

1. Timely detection and competent treatment of late gestosis,
2. Preparing a married couple for pregnancy: identifying and treating existing diseases, combating bad habits,
3. Registration of a pregnant woman up to 12 weeks,
4. Regular attendance at consultations with the doctor who is managing the pregnancy,
5. Proper nutrition that meets the needs of the pregnant woman’s body,
6. Moderate physical stress
7. Optimal work and rest regime,
8. Full sleep
9. Elimination of psycho-emotional stress.

Timely and correct treatment makes the prognosis of the disease favorable: the main symptoms quickly and irrevocably regress. Relapses are extremely rare and account for 4% of women at high risk. The syndrome requires professional treatment in a hospital.

HELLP syndrome is a dangerous and serious disease that occurs exclusively in pregnant women. In this case, the functions of all organs and systems are disrupted, a decline in vitality and energy is observed, and the risk of intrauterine fetal death and maternal death increases significantly. Strict compliance with all medical recommendations and prescriptions will help prevent the development of this dangerous pregnancy complication.

Video: lecture on obstetrics – HELP syndrome