How does help syndrome manifest itself after childbirth. Hellp - syndrome: concept, clinical forms, possible complications, medical and obstetric tactics

HELLP syndrome- a rare and dangerous pathology in obstetrics. The first letters of the abbreviated name of the syndrome indicate the following:
H - Nemolysis (hemolysis);
EL - elevated liver enzymes (increased activity of liver enzymes);
LP - low 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 - the HELLP syndrome.

EPIDEMIOLOGY

In severe gestosis, the 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

Based on laboratory features, some authors have created a classification of the 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.
According to a similar principle, the classification of J.N. Martin, in which patients with HELLP syndrome are divided into two classes.
- The first class - the content of platelets in the blood is less than 50 x 10 9 /l.
- The second class - the concentration of platelets in the blood is 50-100 x 10 9 /l.

ETIOLOGY

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 the HELLP syndrome are noted.

Immunosuppression (depression of T-lymphocytes and B-lymphocytes).
Autoimmune aggression (antiplatelet, antiendothelial antibodies).
Decreased prostacyclin/thromboxane ratio (decreased production of the prostacyclin-stimulating factor).
Changes in the hemostasis system (thrombosis of the liver vessels).
Genetic defects in liver enzymes.
The use of drugs (tetracycline, chloramphenicol). The following risk factors for the development of HELLP syndrome are distinguished.
Bright skin.
The age of the pregnant woman is over 25 years.
Multiparous women.
Multiple pregnancy.
The presence of severe somatic pathology.

PATHOGENESIS

The pathogenesis of HELLP syndrome is currently not fully understood.

The main stages in the development of the HELLP syndrome in severe preeclampsia are considered to be autoimmune damage to the endothelium, hypovolemia with blood clotting, 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, IgG and IgM 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 in HELLR syndrome. The destruction of platelets leads to the release of thromboxanes and imbalance in the thromboxane-prostacycline system, generalized arteriolospasm with aggravation of hypertension, cerebral edema and convulsions. A vicious circle is developing, which is currently only possible to break through emergency delivery.

Preeclampsia is considered a syndrome of multiple organ failure, and HELLP syndrome is its extreme degree, which is the result of maternal organism maladjustment in an attempt to ensure the normal functioning of the fetus.

Macroscopically, with HELLP syndrome, an increase in the size of the liver, a thickening of its consistency, and subcapsular hemorrhages are noted. The color of the liver becomes light brown. Microscopic examination reveals periportal hemorrhages, fibrin deposits, IgM, IgG in the sinusoids of the liver, multilobular necrosis of hepatocytes.

CLINICAL PICTURE

HELLR syndrome usually occurs in the third trimester of pregnancy, more often at 35 weeks or more. The disease is characterized by a rapid increase in symptoms. The 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), pronounced edema (in 67% of cases), headache, fatigue, malaise, motor restlessness, hyperreflexia.

The characteristic signs of the disease are jaundice, vomiting of blood, hemorrhages at the injection sites, progressive liver failure, convulsions and severe coma.

The most common clinical features of HELLP syndrome

signs	HELLP syndrome
Pain in the epigastric region and / or in the right hypochondrium	+++
Headache	++
Jaundice	+++
Arterial hypertension	+++/-
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	+/-
Fever	++/-
Skin itching	+/-
Weight loss	+
Note: +++, ++, +/- - severity of manifestations.

DIAGNOSTICS

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 erythrocytes in a blood smear, polychromasia. The destruction of erythrocytes leads to the release of phospholipids and to intravascular coagulation, i.e. chronic DIC, which is the cause of fatal obstetric bleeding.
If HELLR 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.

Laboratory indicators	Changes in HELLP syndrome
The content of leukocytes in the blood	Within normal limits
The activity of aminotransferases in the blood (ALT, AST)	Increased to 500 units (norm up to 35 units)
ALP activity in the blood	Pronounced increase (3 times or more)
The concentration of bilirubin in the blood	20 µmol/l or more
ESR	reduced
The number of lymphocytes in the blood	Norm or slight decrease
The concentration of protein in the blood	reduced
The number of platelets in the blood	Thrombocytopenia (less than 100 x 10 9 /l)
The nature of red blood cells	Altered erythrocytes with Barr cells, polychromasia
The number of erythrocytes in the blood	Hemolytic anemia
prothrombin time	enlarged
Blood glucose concentration	reduced
clotting factors	Consumption coagulopathy: a decrease in the content of factors for the synthesis of which vitamin K is needed in the liver, a decrease in the concentration of antithrombin III in the blood
The concentration of nitrogenous substances in the blood (creatinine, urea)	Increased
The content of haptoglobin in the 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 preeclampsia complicated by HELLP syndrome also reveals multiple hypoechoic areas, which are regarded as signs of periportal necrosis and hemorrhage (hemorrhagic liver infarction).
For differential diagnosis of HELLP syndrome, CT and MRI are used.

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

Signs of the HELLP syndrome may also occur in other pathological conditions besides preeclampsia. It is necessary to differentiate this condition with hemolysis of erythrocytes, increased activity of liver enzymes in the blood and thrombocytopenia, which developed with the following diseases:

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

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

Indications for consulting other specialists
Consultations of a resuscitator, hepatologist, hematologist are shown.

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

TREATMENT

Treatment Goals: Restoration of disturbed homeostasis.

Indications for hospitalization
HELLP-syndrome as a manifestation of severe gestosis in all cases is an indication for hospitalization.

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

Medical 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 of body weight per day, transfusion thromboconcentrate (at least 2 doses when the platelet count is less than 50 x 10 9 / l), glucocorticoids (prednisolone at a dose of at least 500 mg / day intravenously). In the postoperative period, under the control of clinical and laboratory indicators, fresh frozen plasma is continued at a dose of 12-15 ml/kg of body weight in order to replenish the content of plasma coagulation factors, and it is also recommended to perform plasmapheresis in combination with replacement transfusion of fresh frozen plasma, elimination of hypovolemia, antihypertensive and immunosuppressive therapy. Mayen et al. (1994) believe that the administration of glucocorticoids improves maternal outcome in women with preeclampsia and HELLP syndrome.

Terms and methods of delivery
In HELLP syndrome, emergency delivery by caesarean section is indicated against the background of correction of metabolic disorders, replacement and hepatoprotective therapy, and prevention of complications.

Possible complications in pregnant women with severe preeclampsia complicated by HELLP syndrome

For cesarean section, the most gentle methods of protecting the mother and fetus from obstetric aggression should be used. When choosing epidural or spinal anesthesia, one should not forget about the high risk of extradural and subdural bleeding in thrombocytopenia. The content of platelets less than 100 x 10 9 /l is considered a critical value for regional anesthesia in severe preeclampsia with HELLP syndrome. Subdural hematomas can also occur during regional anesthesia in pregnant women with severe preeclampsia who have been taking acetylsalicylic acid for a long time.

During delivery, special attention is paid to the condition of children. It has been established that thrombocytopenia occurs in newborns in 36% of cases, which leads to the development of hemorrhages and lesions of the nervous system. 5.6% of children are born in a state of asphyxia, and the majority of newborns are diagnosed with respiratory distress syndrome. In 39% of cases, IGR is noted, in 21% of cases - leukopenia, in 33% of cases - neutropenia, in 12.5% of cases - intracranial hemorrhages, in 6.2% of cases - intestinal necrosis.

Evaluation of the effectiveness of treatment
The success of intensive therapy for HELLP syndrome largely depends on timely diagnosis, both before delivery and in the postpartum period. Despite the extreme severity of the course of the HELLP syndrome, its addition should not serve as an excuse for the death of severe gestosis, but rather indicates untimely diagnosis and late or inadequate intensive care.

Prevention
Timely diagnosis and adequate treatment of preeclampsia.

Information for the patient
HELLP syndrome is a severe complication of preeclampsia that requires professional treatment in a hospital. In most cases, a week after delivery, the manifestations of the disease disappear.

Forecast
With a favorable course in the postpartum period, a rapid regression of all symptoms is observed. At the end of pregnancy, after 3-7 days, laboratory blood counts normalize, with the exception of cases of severe thrombocytopenia (below 50 x 10 9 /l), when, with the use of appropriate corrective therapy, the platelet count returns to normal on the 11th day, and LDH activity - after 8-10 days. The risk of recurrence during subsequent pregnancy is small and amounts to 4%, but women should be classified as a high-risk group for the development of this pathology.

Obstetrics: Handbook of the University of California / Ed. K. Niswander, L Evans. - M.: Practice, 1999. - S. 132-134.
Wallenberg H.S.S. New advances in the management of early preeclampsia and HELLP syndrome / Kh.S.S. Wallenberg // Obstetrics and Gynecology. - 1998. - No. 5. - S. 29-32.
Intensive care in obstetrics and gynecology / V.I. Kulakov and others - M .: Medical Information Agency, 1998. - S. 44-61.
Achieving better results in the treatment of mothers with postpartum HELLP syndrome using dexamethasone therapy / Martin J. et al. // AG-info. - 2000. - No. 1. - S. 20-21.
Makatsaria A.D., Bitsadze V.O. Thrombophilia and antithrombotic therapy in obstetric practice / A.D. Makatsaria, V.O. Bitsadze. - M.: Triada-X, 2003. - 904 p.
Nikolaeva E.I., Bobkova M.V. HELLP-syndrome or acute fatty hepatosis of pregnant women / E.I. Nikolaev, M.V. Bobkova // Medical assistance. - Medicine, 1994. - No. 2. - S. 23-25.
Sidorova I.S. Gestosis / I.S. Sidorov. - M., 1997. - S. 130-136.
Surov A.V. HELLP syndrome in obstetrics / A.V. Surov // Obstetrics and gynecology. -1997. - No. 6. - S. 7-9.
Savelyeva G.M., Shalina R.I., Belyakova G.I. HELLR-syndrome: etiology, pathogenesis, diagnosis, treatment / G.M. Savelyeva, R.I. Shalina, G.I. Belyakova // Bulletin of the Russian Association of Obstetricians and Gynecologists. - 1997. - No. 2. - S. 33-37.
Khadasevich L.S., Hareva O.V., Abramov A.A. Preeclampsia in pregnancy complicated by HELLP syndrome / L.S. Khadasevich, O.V. Hareva, A.A. Abramov // Archive of Pathology. -1999. - T. 61, No. 6. - S. 41-43.

This ailment was called the flashy term "HELLP-syndrome" for a reason. If during pregnancy such a diagnosis was established, then it is time to sound the alarm: urgent medical care is needed. The body, as it were, refuses to perform a reproductive function, and all systems begin to fail, threatening the life of the expectant mother and her baby. What is a disease, and what actions should be taken in order to prevent its development?

What is HELLP Syndrome

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

Explanation of the abbreviation HELLP:
H - Hemollysis - hemolysis;
EL - elevated liver enzymes - excess of liver enzymes;
LP - Low platelet count - thrombocytopenia.

Doctors fear the syndrome because of its rapid course and frequent deaths. Fortunately, it is rare: about 1-2 cases per 1 thousand pregnancies.

This disease was first described at the end of the 19th century. But it wasn't until 1985 that his symptoms were linked together and referred to by the generic term "HELLP". It is interesting that in Soviet medical reference books almost nothing is said about this syndrome, and only rare Russian resuscitators indicated in their writings about the disease, calling it "an obstetrician's nightmare."

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

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

Clinical picture of the disease

Diagnosing HELLP syndrome is quite difficult, since its symptoms do not always manifest themselves in full force. In addition, many of the symptoms of the disease often occur during pregnancy and have nothing to do with this serious condition. Indicate the development of complicated preeclampsia can:

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

It is worth noting that changes in urine and blood values usually appear long before the clinical manifestation of the disease, so every pregnant woman needs to visit her gynecologist in a timely manner and take all the tests prescribed by him. Many of the described signs are also found in gestosis. However, the HELLP syndrome is characterized by a rapid increase in symptoms that develop within 4-5 hours. If the expectant mother felt such changes in the body, you should immediately call an ambulance.

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

Preeclampsia, preeclampsia, eclampsia or HELLP syndrome?

The doctor has no more than 2-4 hours to research and make a decision on the tactics of further treatment if HELLP syndrome is suspected. He makes the diagnosis based on the physical examination, ultrasound results, liver tests, and blood tests. Sometimes pregnant women are prescribed tomography to rule out hemorrhage in the liver.

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

Distinctive symptoms in similar diseases - table

	Preeclampsia	Preeclampsia	Eclampsia	HELLP syndrome
Average pressure rise	140/90	160/110	160/110	200/120
Edema	+	+	+	+
convulsions			+	+
hemorrhages				+
Headache	+	+	+	+
Fatigue		+	+	+
Yellowness of the skin				+
Nausea, vomiting	+	+	+	+
Vomiting blood				+
Pain in the liver				+

Prognosis for HELLP syndrome

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

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

	1993	year 2000	2008	2015
Pulmonary edema	12%	14%	10%	11%
Liver hematomas	23%	18%	15%	10%
Placental abruption	28%	28%	22%	17%
preterm birth	60%	55%	51%	44%
Mother's death	11%	9%	17%	8%
The death of a child	35%	42%	41%	30%

Obstetric tactics

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

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

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

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

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

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

What complications can occur as a result of HELLP-syndrome in a mother and her baby

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

pulmonary edema;
acute renal failure;
hemorrhage in the brain;
formation of a hematoma in the liver;
liver rupture;
premature detachment of the placenta;
lethal outcome.

High blood pressure disrupts blood circulation in the placenta, resulting in the fetus not receiving the necessary oxygen. This leads to such complications for the baby:

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

Recovery after surgery

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

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

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

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

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

Preeclampsia and its complications during pregnancy - video

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

A severe complication of pregnancy, which is characterized by a triad of signs: hemolysis, damage to the hepatic parenchyma and thrombocytopenia. It is clinically manifested by rapidly growing symptoms - pain in the liver and abdomen, nausea, vomiting, edema, jaundice of the skin, increased bleeding, impaired consciousness up to coma. It is diagnosed on the basis of a general blood test, a study of enzymatic activity and the state of hemostasis. Treatment involves emergency delivery, the appointment of active plasma-substituting, hepatostabilizing and hepatoprotective therapy, drugs that normalize hemostasis.

ICD-10

O14.2 HELLP syndrome

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, is characterized by 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 the 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 is made up of fair-skinned pregnant women over 25 years old with severe somatic disorders. With each subsequent pregnancy, the likelihood of developing the disease increases, especially when it comes to bearing two or more fetuses.

Causes

To date, the etiology of the disorder has not been definitively determined. More than 30 theories of the occurrence of this acute obstetric pathology have been proposed by specialists in the field of obstetrics and gynecology. Most likely, it develops with a combination of a number of factors, aggravated by the course of preeclampsia. Some authors consider pregnancy as one of the variants of 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 a pair of prostacyclin/thromboxane is reduced. Sometimes the disease complicates the course of another autoimmune pathology - antiphospholipid syndrome.
Genetic anomalies. The basis for the development of the syndrome can be a congenital failure of the 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 intake 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 starting point in the development of HELLP syndrome is a decrease in the production of prostacyclin against the background of an autoimmune reaction resulting from the effect of antibodies on blood cells and endothelium. This leads to microangiopathic changes in the inner lining of the vessels and the release of placental thromboplastin, which enters the mother's bloodstream. In parallel with damage to the endothelium, vasospasm occurs, provoking ischemia of the placenta. The next stage in the pathogenesis of the HELLP syndrome is the mechanical and hypoxic destruction of erythrocytes, which pass through the spasmodic vascular bed and are actively attacked by antibodies.

Against the background of hemolysis, adhesion and aggregation of platelets increases, their overall level decreases, the blood thickens, multiple microthrombosis occurs, followed by fibrinolysis, and DIC develops. Violation of 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. Due to vasospasm, blood pressure rises. As other systems are involved in the pathological process, signs of multiple organ failure increase.

Classification

A unified systematization of the forms of the HELLP syndrome is not yet available. Some foreign authors suggest taking into account the data of laboratory studies when determining the variant of the pathological condition. In one of the existing classifications, three categories of laboratory parameters are distinguished, which correspond to hidden, suspected and obvious signs of intravascular coagulation. More accurate is the option based on determining the concentration of platelets. 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 grade. The content of blood platelets is from 50 to 100×10 9 /L. The course of the syndrome and the 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

Initial manifestations of the disease are nonspecific. A pregnant woman or a woman in labor complains of pain in the epigastrium, the area of the 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 at the injection sites and on the mucous membranes, areas of hemorrhages form, the skin becomes icteric. There is lethargy, confusion. In severe cases of the disease, convulsive seizures, the appearance of blood in the vomit, are possible. In the terminal stages, a coma develops.

Complications

The 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 DIC, every third patient has 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, fiber exfoliates, causing a hemorrhagic stroke. 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 woman or the woman in labor.

HELLP syndrome is dangerous not only for the mother, but also for the child. If the 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 have 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 the HELLP syndrome in a patient is the basis for urgent laboratory tests that verify damage to the hemostasis system and hepatic parenchyma. Additionally, control of the main vital parameters (respiratory rate, pulse temperature, blood pressure, which is increased in 85% of patients) is provided. The most valuable in the diagnostic plan are the following types of examinations:

General blood analysis. A decrease in the number of erythrocytes and their polychromasia, deformed or destroyed red blood cells are determined. One of the diagnostically reliable criteria is thrombocytopenia less than 100×10 9 /l. The number of leukocytes and lymphocytes is usually not changed, there is a slight decrease in ESR. The level of hemoglobin falls.
Liver tests. Violations of enzyme systems typical of liver damage are revealed: aminotransferase activity (AST, ALT) is increased by 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. Concentrations of proteins and haptoglobin 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. Prolongation of thrombin time, a decrease in APTT and fibrinogen concentration also indicate a violation of blood coagulation.

It should be noted that the typical laboratory signs of the HELLP syndrome may deviate from the normative values unevenly, in such cases they speak of the variants of the disease - the ELLP syndrome (there is no hemolysis of erythrocytes) and the HEL syndrome (the platelet count is not impaired). For a rapid assessment of the state of the liver, an ultrasound examination is performed.

Since kidney function is impaired in severe forms of the disease, 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 are considered a prognostically unfavorable factor. Taking into account the pathogenesis of the disease, an ECG, ultrasound of the kidneys, and an examination of the fundus are recommended. In the prenatal period, CTG, ultrasound of the uterus, Doppler ultrasound are performed to monitor the condition of the fetus, the hemodynamics of the fetus and mother.

HELLP syndrome must be differentiated from severe gestosis, fatty hepatosis of pregnant women, viral and drug-induced hepatitis, hereditary thrombocytopenic purpura, and hemolytic uremic syndrome. Differential diagnosis is also carried out with intrahepatic cholestasis, Dubin-Johnson syndromes and Budd-Chiari syndrome, systemic lupus erythematosus, cytomegalovirus infection, infectious mononucleosis and other pathological conditions.

Given the seriousness of the prognosis of the disease, its overdiagnosis has recently been noted. In complex clinical cases, a hepatologist, neuropathologist, ophthalmologist, infectious disease specialist and other specialists are involved in the diagnostic search.

Treatment of HELLP syndrome

Medical tactics in identifying a disease in a pregnant woman is aimed at terminating the pregnancy within 24 hours from the moment of diagnosis. In patients with a mature cervix, vaginal delivery is recommended, but more often an emergency caesarean section is performed under endotrachial anesthesia with the use of 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, and fibrinolysis inhibitors, the woman's condition is maximally stabilized, and, if possible, disturbed multiple organ disorders are compensated.

Complex drug therapy aimed at eliminating angiopathy, microthrombosis, hemolysis, affecting various links of pathogenesis, restoring the function of the liver, other organs and systems, is actively continued in the postoperative period. For the treatment of the syndrome, prevention or elimination of its possible consequences are recommended:

Infusion and blood replacement therapy. The introduction of blood plasma and its substitutes, thromboconcentrates, complex saline solutions makes it possible to replenish the destroyed formed elements and fluid deficiency in the intravascular bed. An additional effect of such therapy is the 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 the normalization of hemostasis. To improve the performance of the blood coagulation system, reduce the manifestations of hemolysis and prevent microthrombosis, low molecular weight heparins, other antiplatelet agents and anticoagulants, drugs with vasoactive action are used. Protease inhibitors are 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 the indications, nootropic and cerebroprotective drugs, vitamin-mineral complexes are prescribed. If manifestations of acute renal failure occur, depending on the severity of the disorders, hemodialysis is also performed.

Forecast 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 be registered early in the antenatal clinic and constant monitoring by an obstetrician-gynecologist.

If signs of preeclampsia are found, it is important to carefully follow the prescriptions of the attending physician, normalize the diet, and adhere to the sleep and rest regimen. A rapid deterioration in the condition of a pregnant woman with the onset of symptoms of severe eclampsia and preeclampsia is an indication for emergency hospitalization in an obstetric hospital.

Makatsaria A.D., Bitsadze V.O., Khizroeva D.Kh.

Obstetrics, gynecology and reproduction. 2014; N2: c.61-68

Summary:

HELLP-syndrome among pregnant women with preeclampsia occurs, according to the generalized data of world literature, in 20-20% of cases and is characterized by high maternal and perinatal mortality. HELLP-syndrome usually develops in the III trimester of pregnancy, as a rule, at a period of 35 weeks, it can also occur after childbirth against the background of the normal course of pregnancy. The pathophysiology of the syndrome remains unexplored. To date, it is believed that endothelial dysfunction is a key stage in the formation of HELLP syndrome. As a result of damage to the endothelium and activation of the inflammatory response, blood coagulation processes are activated, which leads to the development of coagulopathy, increased consumption of platelets, and the formation of platelet-fibrin microthrombi. Perhaps, the deepening of knowledge about the pathogenesis of HELLP syndrome, the development of ideas about the complication of pregnancy as an extreme manifestation of a systemic response to inflammation, leading to the development of multiorgan dysfunction, will allow us to develop effective methods for the prevention and intensive care of this threatening condition.

HELLP-SYNDROME

Keywords: HELLP syndrome, eclampsia, catastrophic antiphospholipid syndrome, hemolysis.

GBOU VPO "First Moscow State Medical University named after I.M. Sechenov” of the Ministry of Health of the Russian Federation, Moscow

Today, thanks to the advances in molecular medicine and a detailed study of the mechanisms of inflammation, the understanding of many diseases, the cause of which has long remained a mystery, has significantly expanded. More and more data appear in favor of the fact that diseases and syndromes such as thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome, catastrophic antiphospholipid syndrome (CAPS), HELLP syndrome, heparin-induced thrombocytopenia are various manifestations of a universal reaction of the body - systemic response to inflammation.

Despite the fact that these pathological processes may be based on various genetic and acquired anomalies (blood clotting factors, complement system, etc.), the development of clinical manifestations is based on a universal systemic inflammation reaction. The key mechanism of the pathogenesis of each of these pathological processes is the progressive damage to the endothelium, the development of an inflammatory response and the activation of coagulation processes with the development of thrombosis.

Due to the fact that these diseases are relatively rare and, due to the lack of experimental models, remain largely incomprehensible to researchers until today, treatment is predominantly imperial, and mortality, despite the success of theoretical medicine, is high. However, recent molecular and genetic studies have made it possible to significantly expand the understanding of the pathogenetic mechanisms of these diseases, without knowledge of which it is impossible to hope for an improvement in the diagnosis of methods for treating these pathologies.

In 1954, Pritchard and colleagues first described three cases of preeclampsia, in which intravascular hemolysis, thrombocytopenia, and liver dysfunction were observed. In 1976, the same author described 95 women with preeclampsia, 29% of whom had thrombocytopenia, and 2% had anemia. At the same time, Goodlin described 16 women with severe preeclampsia, accompanied by thrombocytopenia and anemia, and called this disease "the great imitator", since the manifestations of preeclampsia can be extremely diverse. The term HELLP syndrome (hemolysis, elevated liver enzymes, low platelets) was first introduced into clinical practice by Weinstein in 1982 as an extremely progressive form of gestosis, accompanied by the development of microangiopathic hemolysis, thrombocytopenia, and an increase in the concentration of liver enzymes.

HELLP-syndrome among pregnant women with preeclampsia occurs, according to the generalized data of world literature, in 2-20% of cases and is characterized by high maternal (from 3.4 to 24.2%) and perinatal (7.9%) mortality. HELLP-syndrome usually develops in the third trimester of pregnancy, usually at 35 weeks, and can also occur after childbirth against the background of the normal course of pregnancy. For example, according to Sibai et al. (1993), HELLP-syndrome can develop both before childbirth (in 30% of cases) and after childbirth (70%). The latter group of women has a higher risk of developing acute renal and respiratory failure. Signs of HELLP syndrome may appear within 7 days. after childbirth and most often appear within the first 48 hours after childbirth.

HELLP-syndrome is more often observed in multiparous with gestosis, over the age of 25, with a complicated obstetric history. There is evidence of a possible hereditary predisposition to the development of HELLP syndrome. HELLP syndrome occurs more frequently among whites and Chinese, much less frequently (almost 2.2 times) among the East Indian population.

Clinical picture in HELLP syndrome

In addition to the general manifestations of gestosis - edema, proteinuria, hypertension - HELLP syndrome is characterized by hemolysis, thrombocytopenia, and liver damage. These clinical manifestations lead to serious complications, such as the development of eclampsia, renal failure, intracranial hemorrhage, subcapsular hematoma, and the development of DIC.

The clinical picture of the HELLP syndrome is characterized by a rapid increase in symptoms and often manifests itself as a sharp deterioration in the condition of the pregnant woman and the fetus (see Table 1). Initial manifestations are nonspecific and include headache, fatigue, malaise, nausea, vomiting, pain in the abdomen and especially in the right hypochondrium. Early clinical symptoms of HELLP syndrome may include nausea and vomiting (86%), pain in the epigastric region and in the right hypochondrium (86%), severe edema (67%). The most characteristic manifestations of the disease are jaundice, vomiting with blood, hemorrhage at the injection sites, and progressive liver failure. Neurological symptoms include headache, seizures, symptoms of cranial nerve damage, and in severe cases, coma. Visual disturbances, retinal detachment, and vitreous hemorrhages may occur. One of the signs of developing HELLP syndrome may be hepatomegaly and signs of peritoneal irritation. Irritation of the phrenic nerve by the enlarged liver can cause pain to spread to the pericardium, pleura, and shoulder, as well as the gallbladder and esophagus.

Table 1. Symptoms of HELLP-syndrome.

Often, laboratory changes in HELLP syndrome appear long before the described complaints and clinical manifestations. One of the main and first symptoms of HELLP syndrome is hemolysis (microangiopathic hemolytic anemia), which is determined by the presence of wrinkled and deformed erythrocytes, erythrocyte fragments (schistocytes), and polychromasia in a peripheral blood smear. The cause of hemolysis is the destruction of erythrocytes during their passage through narrowed microvessels with damaged endothelium and fibrin deposits. Fragments of erythrocytes accumulate in spasmodic vessels with the release of substances that promote aggregation. The destruction of red blood cells leads to an increase in the content of lactate dehydrogenase and indirect bilirubin in the blood. The accumulation of indirect bilirubin is also promoted by hypoxia, which develops as a result of hemolysis of erythrocytes and limits the activity of hepatocyte enzymes. Excess indirect bilirubin causes staining of the skin and mucous membranes.

Violation of blood flow in intrahepatic vessels due to the deposition of fibrin in them and the development of hypoxia lead to degeneration of hepatocytes and the appearance of markers of the cytolytic syndrome (increased liver enzymes) and hepatocellular insufficiency syndrome (decrease in protein synthesis function, decrease in the synthesis of blood coagulation factors, leading to the development of bleeding ) . Ischemic liver damage is explained by a decrease in portal blood flow due to the deposition of fibrin in the hepatic sinuses and spasm of the hepatic artery, which is confirmed by Doppler study data. In the postpartum period, hepatic artery tone is restored, while portal blood flow, which normally provides 75% of hepatic blood flow due to fibrin deposits, is restored much more slowly.

Due to obstruction of blood flow in dystrophically altered hepatocytes, overstretching of the Glisson capsule occurs, which leads to the appearance of typical complaints of pain in the right hypochondrium, in the epigastrium. An increase in intrahepatic pressure can lead to the appearance of a subcapsular hematoma of the liver and to its rupture at the slightest mechanical impact (increased intra-abdominal pressure during childbirth through the natural birth canal - Christeller's manual, etc.). Spontaneous liver rupture is a rare but severe complication of HELLP syndrome. According to the world literature, liver rupture in HELLP syndrome occurs with a frequency of 1.8%, while the maternal mortality rate is 58-70%.

Thrombocytopenia in HELLP syndrome is caused by platelet depletion due to the formation of microthrombi in endothelial injury and consumption during DIC. A decrease in the half-life of platelets is characteristic. The detection of an increase in the level of platelet precursors in the peripheral blood indicates a re-irritation of the platelet germ.

Laboratory changes are maximally manifested in the postpartum period (within 24-48 hours after delivery), at the same time, the full clinical picture of the HELLP syndrome unfolds. Interestingly, in contrast to the HELLP syndrome, in severe preeclampsia, the regression of laboratory and clinical symptoms occurs during the first day of the postpartum period. In addition, in contrast to the severe form of preeclampsia, which is most common in primiparas, among patients with HELLP-syndrome, the percentage of multiparous (42%) is quite high.

Perhaps the appearance of only one or two typical signs of HELLP-syndrome. HELLP syndrome is called "partial" or ELLP syndrome (in the absence of signs of hemolysis). Women with "partial" HELLP syndrome have a better prognosis. Van Pampus et al. (1998) indicate the occurrence of severe complications (eclampsia, abruption of a normally located placenta, cerebral ischemia) in 10% of cases with ELLP syndrome and in 24% of cases with HELLP syndrome. However, other studies do not support differences in outcomes between ELLP and HELLP syndromes.

The classic triad of preeclampsia symptoms (edema, proteinuria, hypertension) in HELLP syndrome is detected only in 40-60% of cases. So, only in 75% of women with HELLP-syndrome, blood pressure exceeds 160/110 mm Hg. Art., and 15% have diastolic blood pressure
Maternal and perinatal complications of the HELLP syndrome are exceptionally high (see Table 2).

Table 2. Maternal complications in HELLP-syndrome, %.

According to the generalized data of Egerman et al. (1999), maternal mortality in HELLP-syndrome reaches 11%, although according to earlier data Sibai et al. - 37%. Perinatal complications are caused by the severity of the mother's condition, premature birth of the fetus (81.6%), intrauterine growth retardation of the fetus (31.6%). According to Eeltnic et al. (1993), who studied the level of perinatal mortality in 87 women with HELLP syndrome, perinatal fetal death develops in 10% of cases, and in another 10% of women the child dies in the first week of life. In children born to mothers with HELLP syndrome, characteristic symptoms are observed: thrombocytopenia - in 11-36%, leukopenia - in 12-14%, anemia - in 10%, DIC - in 11%, somatic pathology - in 58 %, 3-4 times more often observed respiratory distress syndrome (36%), instability of the cardiovascular system (51%). Intensive care of newborns should include the prevention and control of coagulopathy from the very first hours. Thrombocytopenia in newborns with HELLP syndrome occurs in 36% of cases, which can lead to the development of hemorrhages and damage to the nervous system.

According to Abramovici et al. (1999), who analyzed 269 cases of pregnancies complicated by HELLP syndrome, severe preeclampsia and eclampsia, with timely diagnosis and adequate treatment, the level of perinatal mortality in HELLP syndrome does not exceed that in severe preeclampsia and eclampsia.

Pathological anatomical picture in HELLP-syndrome

Post-mortem changes in HELLP syndrome include platelet-fibrin microthrombi and multiple petechial hemorrhages. Autopsy is characterized by polyserositis and ascites, bilateral exudative pleurisy, multiple petechial hemorrhages in the peritoneum and pancreatic tissue, subcapsular hematomas and liver ruptures.

The classic liver injury associated with HELLP syndrome is periportal or focal parenchymal necrosis. Immunofluorescent studies reveal microthrombi and fibrin deposits in the sinusoids. According to Barton et al. (1992), who studied 11 liver samples obtained by biopsy during caesarean section in women with HELLP syndrome, there is no correlation between the degree of histological changes in the liver and the severity of clinical and laboratory symptoms.

According to Minakami et al. (1988), who studied 41 liver samples of those who died from HELLP syndrome, histologically it is impossible to distinguish between acute fatty degeneration of the liver (AFLD) and HELLP syndrome. In both AIDP and HELLP syndrome, vacuolization and necrosis of hepatocytes are noted. However, if in AIDP these changes are located in the central zone, then in HELLP syndrome, periportal necrosis is more present. The authors conclude that the pathogenetic mechanisms of preeclampsia, HELLP-syndrome and AIDP are unity. OZHRP is a relatively rare pathology that develops in the third trimester of pregnancy. With this pathology, as with the HELLP syndrome, an emergency delivery is necessary, which can significantly improve the prognosis for the mother and child.

Fundamentals of the pathogenesis of HELLP syndrome

The etiology and pathogenesis of HELLP syndrome remain not fully understood. Currently, damage to the endothelium and the development of microangiopathy are considered to be the key link in the pathogenesis of HELLP syndrome. The characteristic features of the HELLP syndrome are the activation of coagulation with the deposition of fibrin in the lumen of the vessels, excessive activation of platelets, manifested in their accelerated consumption and the development of thrombocytopenia.

Today, there is more and more evidence about the role of systemic inflammation in the pathogenesis of preeclampsia. It is possible that the HELLP syndrome is based on excessive progressive activation of inflammation processes, endothelial dysfunction, which leads to the development of coagulopathy and multiorgan dysfunction. There is also no doubt that the complement system is involved in the pathogenesis of the HELLP syndrome. According to Barton et al. (1991), immune complexes in HELLP syndrome are found in the hepatic sinuses and even in endocardial needle biopsy. It is possible that the autoimmune mechanism of damage involving the complement system is due to an autoimmune reaction to a semi-allotransplant fetus. So, in the serum of patients with HELLP-syndrome, antiplatelet and antiendothelial autoantibodies are found. Activation of the complement system has a stimulating effect on leukocytes. At the same time, there is an increase in the synthesis of pro-inflammatory cytokines: 11-6, TNF-a, 11-1 (etc.), which contributes to the progression of the inflammatory response. An additional confirmation of the role of inflammation in the pathogenesis of the HELLP syndrome is the detection of neutrophilic infiltration of the liver tissue during immunological studies.

Thus, today it is believed that the key stage in the formation of HELLP syndrome is endothelial dysfunction. As a result of damage to the endothelium and activation of the inflammatory response, blood coagulation processes are activated, which leads to the development of coagulopathy, increased platelet consumption, and the formation of platelet-fibrin microthrombi. The destruction of platelets leads to a massive release of vasoconstrictive substances: thromboxane A2, serotonin. Increased platelet activation and endothelial dysfunction lead to imbalance of the thromboxane-prostacycline system involved in maintaining the balance of the hemostasis system. There is no doubt parallel with the development of HELLP-syndrome intravascular coagulation. Thus, DIC is observed in 38% of women with HELLP syndrome and causes almost all clinical manifestations and severe complications of HELLP syndrome - premature detachment of a normally located placenta, intrauterine fetal death, obstetric bleeding, subcansular hematoma of the liver, liver rupture, cerebral hemorrhage . Although changes are most often found in the liver and kidneys in HELLP syndrome, endothelial dysfunction can also develop in other organs, which is accompanied by the development of heart failure, acute respiratory distress syndrome, and cerebral ischemia.

Thus, gestosis in itself is a manifestation of multiple organ failure, and the addition of the HELLP syndrome indicates an extreme degree of activation of systemic inflammation and organ damage.

According to Sullivan et al. (1994), who studied 81 women who underwent HELLP syndrome, subsequent pregnancy in 23% of cases is complicated by the development of preeclampsia or eclampsia, and in 19% of cases there is a recurrence of HELLP syndrome. However, subsequent studies by Sibai et al. (1995) and Chames et al. (2003) indicate a lower risk of re-development of the HELLP syndrome (4-6%). Sibai et al. indicate a higher risk of preterm birth, IUGR, miscarriage, perinatal mortality in subsequent pregnancies in women who have experienced HELLP syndrome. A sufficiently high risk of recurrence of the HELLP syndrome and the development of complications in subsequent pregnancies indicate the possible presence of a certain hereditary predisposition in such women. For example, according to Kraus et al. (1998), in women who have experienced HELLP syndrome, an increased frequency of resistance to activated protein C and a factor V Leiden mutation are detected. Schlembach et al. (2003) found that factor V Leiden mutation is 2 times more common in women with HELLP syndrome compared to healthy pregnant women. In addition, the combination of HELLP syndrome and thrombophilia was associated with a higher risk of developing IUGR. Moesmer et al. (2005) described the development of HELLP syndrome in a woman with a homozygous mutation of the G20210A prothrombin gene. At the same time, a heterozygous mutation of the prothrombin gene was found in the child. It should be noted that the frequency of mutation of the prothrombin gene, especially homozygous, in the general population is not high. HELLP syndrome is also a fairly rare complication of pregnancy (0.2-0.3%). In addition, the relationship between thrombophilias and an increased risk of HELLP syndrome is not found in all studies. However, the presence of genetic thrombophilias, especially in combination with abnormal hemostasis in the fetus, can be a serious risk factor for the development of coagulopathy (particularly HELLP syndrome) during pregnancy. For example, according to Schlembach et al. (2003), thrombophilia in the fetus can contribute to the formation of placental microthrombi, impaired placental blood flow and the occurrence of IUGR.

Altamura et al. (2005) described a woman with HELLP syndrome complicated by stroke, who had a heterozygous mutation in MTHFR and the prothrombin gene. Pregnancy itself is a condition characterized by hypercoagulability and the development of subclinical systemic inflammation. Thus, according to Wiebers et al. (1985), the incidence of stroke in non-pregnant women aged 15 to 44 is 10.7/1000,000, while the risk of stroke increases 13 times during pregnancy. In the presence of hereditary preexisting anomalies of hemostasis (genetic thrombophilia, APS), pregnancy can serve as a trigger factor for excessive activation of systemic inflammation and the development of coagulopathy, which form the pathogenetic basis of a number of pathologies: HELLP syndrome, preeclampsia, eclampsia, DIC, IUGR.

On the one hand, HELLP-syndrome may be the first manifestation of a hereditary pathology of hemostasis, and on the other hand, genetic analysis for hereditary thrombophilia makes it possible to identify women at risk for the possibility of developing a complicated pregnancy, which require special attention of doctors and specific prevention.

The development of thrombotic microangiopathy, in addition to the HELLP syndrome, is also characteristic of TTP, HUS, and is also one of the manifestations of CAPS. This indicates the presence of a common mechanism of pathogenesis of these diseases. It is known that APS is associated with a high incidence of pregnancy pathologies: IUGR, intrauterine fetal death, premature birth, preeclampsia. In addition, a number of researchers have described cases of the occurrence of HELLP syndrome in women with APS, which once again confirms the importance of hemostasis pathology as a predisposing factor for the occurrence of HELLP syndrome. Koenig et al. (2005) described a woman with APS, whose pregnancy was complicated by the development of HELLP syndrome, and after operative delivery, a clinical picture of CAPS with infarcts of the liver, gastrointestinal tract, and bone marrow developed due to progressive microangiopathy. It should also be taken into account that HELLP syndrome may be the first manifestation of APS. Therefore, in women with HELLP syndrome, an analysis for antiphospholipid antibodies is necessary.

Diagnosis of HELLP syndrome

Diagnostic criteria for HELLP syndrome are:
1. Severe form of preeclampsia (preeclampsia, eclampsia).
2. Hemolysis (microangiopathic hemolytic anemia, deformed erythrocytes).
3. Elevated bilirubin >1.2 mg/dl;
4. Increased lactate dehydrogenase (LDH)> 600 IU / l.
5. An increase in liver enzymes - aminotransferases - aspartate aminotransferase (ACT) > 70 IU / l.
6. Thrombocytopenia (platelet count 7. Hemostasiogram:
– lengthening of the index r+k of the thromboelastogram;
– prolongation of APTT;
- prolongation of prothrombin time;
– increase in the content of D-dimer;
– an increase in the content of the thrombin-antithrombin III complex;
– decrease in the concentration of antithrombin III;
– increase in the level of prothrombin fragments;
– decrease in protein C activity (57%);
– circulation of lupus anticoagulant.
8. Determination of the level of daily proteinuria;
9. Ultrasound of the liver.

A characteristic sign of the HELLP syndrome is also a decrease in the concentration of haptoglobin less than 0.6 g/l.

Martin et al. (1991) analyzed 302 cases of HELLP syndrome and, depending on the severity of thrombocytopenia, identified three degrees of severity of this pregnancy complication: the first degree - thrombocytopenia 150-100x109 / ml, the second degree - 1.00-50x109 / ml, the third - less than 50x109 / ml .

Differential diagnosis HELLR-syndrome should be carried out, first of all, with liver diseases - acute fatty degeneration of the liver, intrahepatic cholestatic jaundice; HELLP syndrome should also be differentiated from liver diseases that can worsen during pregnancy, including Budd-Chiari syndrome (hepatic vein thrombosis), viral diseases, cholelithiasis, chronic autoimmune hepatitis, Wilson-Konovalov disease. The combination of hemolysis, increased activity of liver enzymes and thrombocytopenia can also be observed in obstetric sepsis, spontaneous liver ruptures in pregnant women, and systemic lupus erythematosus. In 1991, Goodlin described 11 cases of misdiagnosis of HELLP syndrome in women with acute cardiomyopathy, dissecting aortic aneurysm, cocaine addiction, glomerulonephritis, gangrenous cholecystitis, SLE, and pheochromocytoma. Therefore, when thrombocytopenia, microangiopathic anemia, and signs of cytolysis are detected, the diagnosis of HELLP syndrome can only be made with a thorough assessment of the clinical picture and the exclusion of other causes of these symptoms.

If HELLP syndrome is suspected the pregnant woman must be hospitalized in the intensive care unit (see Table 3).

Table 3 Required amount of research for suspected HELLP syndrome.

Principles of treatment of HELLP-syndrome

The main task of treating patients with preeclampsia is, first of all, the safety of the mother and the birth of a viable fetus, whose condition does not require long-term and intensive neonatal care. Initial treatment is hospitalization to assess the condition of the mother and fetus. Subsequent therapy should be individualized, depending on the condition and gestational age. The expected result of therapy in the majority of patients with mild disease should be the successful completion of pregnancy. The results of therapy in patients with severe disease will depend both on the condition of the mother and fetus at admission, and on the gestational age.

The main problem in the treatment of HELLP syndrome is the fluctuating course of the disease, the unpredictable occurrence of severe maternal complications, and high maternal and perinatal mortality. Since there are no reliable clinical and laboratory, clearly defined criteria for the prognosis and course of the disease, the outcome of the HELLP syndrome is unpredictable. High maternal morbidity and mortality is mainly due to the development of disseminated intravascular coagulation (DIC); the frequency of development of the acute form of DIC significantly increases with an increase in the interval between diagnosis and delivery.

With HELLP syndrome, delivery by caesarean section is carried out regardless of the duration of pregnancy.

Indications for emergency delivery are:
- progressive thrombocytopenia;
- signs of a sharp deterioration in the clinical course of preeclampsia;
- impaired consciousness and severe neurological symptoms;
- progressive deterioration of liver and kidney function;
– pregnancy 34 weeks or more;
- fetal distress.

Conservative management of pregnancy in these cases is associated with an increased risk of eclampsia, placental abruption, development of respiratory and renal failure, maternal and perinatal mortality. An analysis of recent studies has shown that aggressive tactics lead to a significant reduction in maternal and perinatal mortality. Childbirth through the natural birth canal is possible only with sufficient maturity of the cervix, a thorough assessment of the condition of the fetus and blood flow in the umbilical artery during a Doppler study. Conservative tactics is justified only in cases of fetal immaturity in a situation where there are no signs of disease progression, intrauterine fetal suffering and intensive monitoring is carried out in a specialized obstetric hospital by a qualified obstetrician-gynecologist in close and mandatory cooperation with an anesthesiologist and neonatologist.

The principles of therapy include the replenishment of BCC with the restoration of microcirculation with plasma substitutes: hydroxyethyl starch, albumin, fresh frozen plasma. A single-group donor erythrocyte mass is used to eliminate anemia when hemoglobin is less than 70 g/l. A transfusion of the platelet mass is carried out with a decrease in the level of platelets to 40 thousand or less. With the progression of multiple organ failure with signs of functional decompensation of the liver, kidneys, hemodiafiltration, hormonal therapy with corticosteroids, and antibiotic therapy are effective methods of treatment. Antihypertensive therapy is prescribed individually (see Table 4).

Table 4 Principles of HELLP-syndrome therapy.

Principles of therapy	Specific measures
1. Replenishment of BCC and restoration of microcirculation	Hydroxyethyl starch 6% and 10%; albumin 5%; fresh frozen donated plasma
2. Elimination of anemia	At Hb
3. Elimination of thrombocytopenia	With thrombocytopenia
4. Prevention and control of DIC	Transfusion of fresh frozen plasma
5. Hormone therapy	Corticosteroids
6. Efferent treatments	Plasmapheresis, hemodiafiltration (with progression of multiple organ failure)
7. Antibacterial therapy	Broad spectrum drugs
8. Antihypertensive therapy	Target BP Dihydralazine, labetalol, nifedipine; sodium nitroprusside (for BP >180/110 mmHg), magnesium (to prevent seizures)
9. Control of hemostasis	Antithrombin 111 (for the purpose of prevention - 1000-1500 IU / day, in the treatment of the initial dose - 1000-2000 IU / day, then 2000-3000 IU / day), dipyridamole, aspirin
10. Delivery	C-section

The fight against DIC in combination with detoxification therapy is carried out by performing therapeutic discrete plasmapheresis with the replacement of 100% of the BCC with donor fresh frozen plasma in an equivalent volume, and in case of hypoproteinemia - with transfusion. The use of plasmapheresis in the intensive care complex for HELLP syndrome can reduce maternal mortality in this complication from 75 to 3.4-24.2%.

Intravenous administration of high doses of glucocorticoids can not only reduce perinatal mortality due to the prevention of ARDS, but also reduce maternal mortality, which was confirmed in five randomized trials. Goodlin et al. (1978) and Clark et al. (1986) describe cases when the use of glucocorticoids (10 mg of dexamethasone IV every 12 hours) and the observance of complete rest by the pregnant woman made it possible to achieve a transient improvement in the clinical picture (decrease in blood pressure, increase in platelet count, improvement in liver function, increase in diuresis). Data from Magann et al. (1994), Yalcin et al. (1998), Isler et al. (2001) indicate that the use of glucocorticoids before and after childbirth helps to reduce the severity of the HELLP syndrome, the need for blood transfusion and allows you to prolong pregnancy by 24-48 hours, which is important for the prevention of neonatal respiratory distress syndrome. Isler (2001) showed greater efficacy of intravenous glucocorticoids compared to intramuscular.

It is assumed that the use of glucocorticoids can help restore endothelial functions, prevent intravascular destruction of erythrocytes and platelets and the progression of SIRS. However, following the improvement of the clinical picture within 24-48 hours of the use of glucocorticoids, the so-called rebound phenomenon may occur, which manifests itself as a deterioration in the condition of the pregnant woman. Thus, the introduction of glucocorticoids does not completely prevent the development of the pathological process, but only briefly improves the clinical picture, creating conditions for a more successful delivery.

In most patients with HELLP syndrome, it is recommended to use 10 mg of dexamethasone IV twice with a break of 6 hours, followed by an additional, twice, 6 mg of dexamethasone IV every 6 hours. In severe HELLP syndrome (thrombocytopenia
In the postpartum period, some clinicians recommend the administration of corticosteroids (4x intravenous administration of dexamethasone at 12-hour intervals - 10, 10, 5, 5 mg) immediately after delivery and transfusion of fresh frozen donor plasma. According to Martin et al. (1994), the use of glucocorticoids in the postpartum period can reduce the risk of complications and maternal mortality.

In the postpartum period, it is necessary to continue monitoring the woman until the complete disappearance of clinical and laboratory symptoms. This is due to the fact that, unlike preeclampsia and eclampsia, the symptoms of which usually disappear quickly after delivery, with the HELLP syndrome, the peak of hemolysis is observed 24-48 hours after delivery, which often requires repeated red blood cell transfusion. In the postpartum period, magnesium therapy should be continued for 24 hours. The only exception is women with kidney failure. With continued hemolysis and a decrease in the number of platelets for more than 72 hours after delivery, plasmapheresis is indicated.

In conclusion, it should be noted that the success of HELLP syndrome intensive therapy largely depends on timely diagnosis both before delivery and in the postpartum period. Despite close attention to the problem, the etiology and pathogenesis of HELLP syndrome remain largely a mystery. Perhaps, the deepening of knowledge about the pathogenesis of HELLP syndrome, the development of ideas about the complication of pregnancy as an extreme manifestation of a systemic response to inflammation, leading to the development of multiorgan dysfunction, will allow us to develop effective methods for the prevention and intensive care of this life-threatening condition.

Literature/References:

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HELLP-syndrome

Makatsariya A.D., Bitsadze V.O., Khizroeva D.Kh.

First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation

Abstract: the pathophysiology of HELLP syndrome is not well defined. Nowadays endothelial dysfunction if considered the key moment of the development of HELLP-syndrome. Endothelial cell dysfunction results in hypertension, proteinuria, and increased platelet activation and aggregation. Furthermore, activation of the coagulation cascade causes consumption of platelets due to adhesion onto a damaged and activated endothelium, in addition to microangiopathic hemolysis caused by shearing of erythrocytes as they traverse through capillaries laden with platelet-fibrin deposits. Multiorgan microvascular injury and hepatic necrosis causing liver dysfunction contribute to the development of HELLP.

Key words: HELLP-syndrome, catastrophic antiphospholipid syndrome, eclampsia, hemolysis.

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 arose after childbirth. George Pritchard was the first to describe this pathology. It must be said that this rare syndrome appears only in seven percent of women, but 75% of cases end in death.

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

H - destruction of erythrocytes.
EL - an increase in the level of liver enzymes.
LP - a decrease in the number of red blood cells, which serve for blood clotting.

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

Etiology

To date, the exact causes of this syndrome have not yet been clarified. However, scientists do not cease to put forward various theories of its origin. Today, there are already more than thirty theories, but none can point to the fact that affects the appearance of pathology. Experts noticed one pattern - such a deviation appears against the background of a late manifestation.

The pregnant woman suffers from edema, which begins on the arms, legs, then goes to the face, then to the whole body. The level of protein in the urine increases, and blood pressure also rises. This condition is extremely unfavorable for the fetus, because antibodies aggressive 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 tissues is violated.

As mentioned earlier, HELP syndrome occurs for 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, congenital pathology;
change in the number and purpose of lymphocytes;
formation in the blood vessels of the liver;
long-term use of medications without medical supervision.

By monitoring the pathology, certain factors can be distinguished, which 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 the HELP syndrome shows, 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 of the same name HELP is divided into two classes.

Symptoms

The first signs shown are nonspecific, so it is impossible to diagnose the disease by them.

A pregnant woman has symptoms such as:

nausea;
often vomiting;
dizziness;
pain in the side;
causeless anxiety;
fast fatiguability;
pain in the upper abdomen;
change in skin color to yellow;
the appearance of shortness of breath even with small loads;
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 urination process is disturbed, convulsions occur, body temperature rises. In some situations, a woman may fall into a coma. A doctor can make an accurate diagnosis of HELP syndrome only on the basis of laboratory results.

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

Diagnostics

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

urinalysis - with its help you can find out the level and presence of protein, in addition, the functioning of the kidney is diagnosed;
blood sampling 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 state of the placenta, peritoneum, liver and kidneys;
computed tomography, so as not to make a false diagnosis, which has similar signs;
cardiotocography - determines the viability of the fetus and evaluates its heartbeat.

In addition to these studies, a visual examination of the patient and the collection of anamnesis are carried out. The presence of signs such as yellow skin, bruising from injections will help to more accurately establish the diagnosis.

Often doctors resort to the help of their colleagues working in other areas, for example, a resuscitator, gastroenterologist, hepatologist.

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

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

According to the results of the diagnosis, the tactics of treatment are determined.

Treatment

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

However, there is a chance to save the child, because such a condition in pregnant women manifests itself already in the later stages, so the woman is stimulated 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 gestational age is shorter, then the woman is prescribed glucocorticosteroids: they will help the lungs of the fetus open up. But if there are signs such as heavy bleeding, high blood pressure, cerebral hemorrhage, then an urgent caesarean section is necessary, and it does not matter at what stage of pregnancy the woman is. The therapy performed stabilizes the condition of the woman, and helps to restore the health of the crumbs after surgery.

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

After that, the doctor needs:

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 has a caesarean section, she may be given a procedure such as plasmapheresis - plasma is removed from the blood, but only a clearly marked volume is used.

This is carried out with a special sterile, moreover, disposable apparatus that separates the plasma. This is a non-hazardous procedure that does not cause any discomfort to a woman. The duration of the event takes approximately two hours. This is followed by a blood transfusion.

Also, in the process of preparing for the operation and immediately after it, the woman is prescribed medication to reduce blood pressure, liver failure, and kidney failure.

This will help only complex therapy, which will include drugs such as:

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

After the operation, the 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, the recurrence of the disease is high in all subsequent pregnancies.

Possible Complications

The occurrence of complications from such a pathology is a fairly common occurrence. Unfortunately, deaths are not 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 profuse bleeding of any location. In severe cases, hemorrhages may occur in the brain, and these are malfunctions of the central nervous system.

Violations in the kidneys and liver are also terrible, because the consequences are such that poisoning of the body occurs. Some cases of pathology end in a coma, and it is not easy to get a woman out of this state.

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

Such a disease causes the following symptoms in a woman:

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

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

When the placenta is separated by one third, 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 observe the following rules of prevention:

consciously plan pregnancy, avoid unwanted conception;
carrying out therapeutic actions in the detection of any diseases;
do more sports, observe a clear daily routine;
after establishing the fact of pregnancy, a visit to the doctor according to the schedule;
regular health checks, i.e. testing;
timely therapy of late toxicosis;
healthy eating;
the use of the required amount of fluid per day;
refuse hard physical work, avoid;
observance of the work/rest regime;
be sure to tell your doctor about taking medications for chronic diseases.

Self-medication is unacceptable.