HELLP syndrome — a heavy complication of pregnancy of which the triad of signs is characteristic: , damage of a hepatic parenchyma and thrombocytopenia. It is clinically shown by quickly accruing symptomatology — pains in a liver and in a stomach, nausea, vomiting, hypostases, yellowness of the skin raised by bleeding, violation of consciousness up to a coma. It is diagnosed on the basis of the general blood test, a research of fermental activity and a condition of a hemostasis. Treatment assumes the emergency rodorazresheniye, purpose of active plazmozameshchayushchy, gepatostabiliziruyushchy and hepatoprotective therapy, the medicines normalizing a hemostasis.
Though in recent years HELLP syndrome is observed infrequently, it in 4-12% of cases complicates a current of heavy gestoz and in the absence of adequate treatment differs in high rates of maternal and child mortality. The syndrome as a separate pathological form was for the first time described in 1954. The name of frustration is formed by the first letters of the terms defining key displays of a disease: H-hemolysis (), EL – elevated liver enzymes (increase in activity of hepatic enzymes), LP – low level platelet (thrombocytopenia). HELLP syndrome usually arises in the 3rd trimester of pregnancy on 33-35 week. It develops in 30% of cases for 1-3 days after the delivery. By results of observations, the risk group is made by white-skinned pregnant women 25 years with the expressed somatic frustration are more senior. With each subsequent pregnancy the probability of development of a disease increases, especially if the speech about incubation of two and more fruits.
Today the frustration etiology is finally not defined. Experts in the sphere of obstetrics and gynecology more than 30 theories of developing of this sharp obstetric pathology are offered. Most likely, it develops at a combination of a number of the factors aggravated by a current of a gestoz. Some authors considers pregnancy as one of allotransplantation options, and HELLP syndrome as autoimmune process. Among the most frequent causes of illness call:
- Immune and autoimmune violations. In blood of patients the depression of B-and T-lymphocytes is noted, antibodies to platelets and an endoteliya of vessels are defined. The ratio in couple prostatsiklin/tromboksan is reduced. Sometimes the disease complicates the course of other autoimmune pathology — an anti-phospholipidic syndrome.
- Genetic anomalies. Congenital insolvency of fermental systems of a liver can become a basis for development of a syndrome that increases sensitivity of hepatocytes to action of the damaging factors arising at the autoimmune answer. At a number of pregnant women congenital violations of the curtailing system are also noted.
- Uncontrollable reception of some medicines. The probability of development of pathology increases at the use of the pharmacological medicines having hepatotoxic effect. First of all it is about the tetratsiklena and chloramphenicol which damaging effect increases at immaturity of fermental systems.
Reduction of development of a prostatsiklin against the background of the autoimmune reaction which arose owing to impact of antibodies on cellular elements of blood and endoteliya becomes the starting moment in development of HELLP syndrome. It leads to mikroangiopatichesky changes of an internal cover of vessels and release of a placentary tromboplastin who comes to mother's blood-groove. In parallel with damage the endoteliya arises the angiospasm provoking placenta ischemia. The following stage of pathogenesis of a syndrome HELLP is mechanical and hypoxemic destruction of erythrocytes which pass through the spazmirovanny vascular course and are exposed to the active attack of antibodies.
Against the background of a gemoliz adhesion and aggregation of platelets amplifies, their general level decreases, blood is condensed, there is multiple microthrombosis with the subsequent fibrinoliz, the DVS-syndrome develops. Violation of perfusion in a liver leads to formation of a gepatoz with a parenchyma necrosis, formation of subkapsulyarny hematomas and increase in level of enzymes in blood. Because of an angiospasm arterial pressure increases. In process of involvement in pathological process of other systems signs of polyorgan insufficiency accrue.
Uniform systematization of the HELLP syndrome forms is absent so far. Some foreign authors suggest to consider data of laboratory researches when determining option of a pathological state. In one of the existing classifications mark out three categories of laboratory indicators which correspond to the hidden, suspected and strong indications of intra vascular coagulation. More exact is the option based on definition of concentration of platelets. By this criterion distinguish three classes of a syndrome:
- 1st class. Level of thrombocytopenia makes less 50×109/. The clinic is characterized by a heavy current and the serious forecast.
- 2nd class. The maintenance of platelets of blood makes from 50 to 100×109/. The current of a syndrome and the forecast are more favorable.
- 3rd class. Moderate displays of thrombocytopenia are noted (from 100 to 150×109/). The first clinical signs are observed.
Initial displays of a disease are not specific. The pregnant woman or the woman in labor complains of morbidity to epigastriya, areas of the right podreberye and an abdominal cavity, a headache, dizziness, feeling of weight in the head, painful feelings in muscles of a neck and a humeral belt. Weakness and bystry fatigue increases, sight worsens, there is nausea and vomiting, puffiness. The clinical symptomatology very quickly progresses. In process of aggravation of a state in places of injections and on mucous sites of gemorragiya are formed, skin becomes icteric. Block, confusion of consciousness is observed. At the heavy course of a disease convulsive attacks, emergence of blood in emetic masses are possible. In terminal stages coma develops.
Polyorgan violations from decompensations of the main vital signs of an organism are characteristic of a syndrome of HELLP. Almost in half of cases the disease is complicated by the DVS-syndrome, every third patient has OPN signs, at every tenth — hypostasis of a brain or lungs. At some patients ekssudativny pleurisy and a pulmonary distress syndrome develops. In the postnatal period perhaps profuzny uterine bleeding with hemorrhagic shock. In rare instances at women with HELLP syndrome cellulose exfoliates, there is a hemorrhagic stroke. At 1,8% of patients subkapsulyarny hematomas of a liver which rupture usually leads to massive intra belly bleeding and death of the pregnant woman or woman in labor come to light.
The syndrome of HELLP constitutes danger not only to mother, but also to the child. If pathology develops at the pregnant woman, the probability of premature birth or an otsloyka of a placenta with koagulopatichesky bleeding increases. In 7,4-34,0% of cases the fruit perishes vnutriutrobno. Practically at a third of newborns the thrombocytopenia leading to hemorrhages in brain fabric and to the subsequent neurologic frustration is noted. A part of children is born in a condition of asphyxia or with respiratory a distress syndrome. The intestines necrosis revealed at 6,2% of babies becomes a serious, though infrequent complication of a disease.
Suspicion on development in the patient of a syndrome of HELLP is the basis for the emergency implementation of the laboratory analyses verifying defeat of system of a hemostasis and hepatic parenchyma. Control of the key vital parameters (frequency of breath, temperature of pulse, arterial pressure which is increased at 85% of patients) is in addition provided. In the diagnostic plan the following types of inspections are the most valuable:
- General blood test. The decrease in quantity of erythrocytes and their polikhromaziya deformed or the destroyed red blood cells is defined. One of diagnostic reliable criteria thrombocytopenia less 100×109/ is considered. The quantity of leukocytes and lymphocytes is usually not changed, some decrease in SOE is noted. Hemoglobin level falls.
- Hepatic tests. The violations of fermental systems typical for damage of a liver come to light: aminotransferazny activity (NUCLEAR HEATING PLANT, ALT) is increased at 12-15 times (to 500 Pieces/l). By 3 times activity of alkaline phosphatase increases more. Bilirubin level in blood exceeds 20 µmol/l. Concentration of proteins and a gaptoglobin are reduced.
- Assessment of system of a hemostasis. Laboratory signs of a koagulopatiya of consumption are characteristic – the maintenance of the curtailing factors synthesized in a liver with vitamin K participation decreases. Anti-thrombin III level is reduced. Fibrillation violation is also demonstrated by lengthening of trombinovy time, decrease in AChTV and concentration of fibrinogen.
It should be noted that typical laboratory signs of a syndrome of HELLP can deviate standard indicators unevenly, in such cases speak about disease options — an ELLP syndrome (is absent erythrocytes) and a HEL syndrome (the maintenance of platelets is not broken). For express assessment of a condition of a liver conduct its ultrasonography. As at severe forms of a disease function of kidneys, reduction of daily amount of urine, emergence of a proteinuria and increase in content in blood of nitrogenous substances is broken (urea, creatinine) it is considered predictively an adverse factor. Taking into account pathogenesis of a disease ECGs, ultrasonography of kidneys, a research of an eye bottom are recommended. In the prenatal period for monitoring of a condition of a fruit, haemo dynamics of a fruit and mother carry out KTG, ultrasonography of a uterus, a dopplerometriya.
Taking into account gravity of the forecast of a disease its hyper diagnostics is noted recently. HELLP syndrome needs to be differentiated from a heavy current of gestoz, a fatty gepatoz of pregnant, viral and medicinal hepatitises, hereditary trombotsitopenichesky purples, a hemolytic uraemic syndrome, an intra hepatic holestaz, Dabin-Johnson's syndrome, Badda-Kiari's syndrome, a system red volchanka, a Cytomegaloviral infection, an infectious mononukleoz and other pathological states. In difficult clinical cases involve the hepatologist, the intensivist, the neuropathologist, the oculist, the infectiologist, the immunologist, the therapist, the rheumatologist, the surgeon, the nephrologist in diagnostic search.
Treatment of HELLP syndrome
Medical tactics at detection of a disease at the pregnant woman is directed to termination of pregnancy during 24 h from the diagnosis moment. The rodorazresheniye in natural patrimonial ways is recommended to patients with a mature neck of a uterus, however more often in the emergency order Cesarean section under an endotrakhialny anesthesia with use of negepatotoksichny anesthetics and the prolonged IVL is carried out. At a stage of intense preoperative training due to introduction of freshly frozen plasma, kristalloidny solutions, glucocorticoids, inhibitors of a fibrinoliz the condition of the woman is as much as possible stabilized, the broken polyorgan violations are whenever possible compensated.
The complex medicamentous therapy directed to elimination of angiopatiya, microthromboses, a gemoliz, impact on various links of pathogenesis, restoration of function of a liver, other bodies and systems actively continues in the postoperative period. For treatment of a syndrome, prevention or elimination of its possible consequences are recommended:
- Infusional and krovozameshchayushchy therapy. Introduction of plasma of blood and its substitutes, trombokontsentrat, complex salt solutions allows to fill the destroyed uniform elements and shortage of liquid in the intra vascular course. The additional effect of such therapy puts improvement of rheological indicators and stabilization of haemo dynamics.
- Gepatostabiliziruyushchy and gepatoprotekorny medicines. To stabilize hepatic , appoint parenteral introduction of glucocorticoids. Use of gepatoprotektor is directed to improvement of work of hepatocytes, protection them from toxic metabolites, stimulation of restoration of the destroyed cellular structures.
- Means for normalization of a hemostasis. For improvement of indicators of the curtailing system of blood, reduction of manifestations of a gemoliz and prevention of microthrombosis low-molecular heparins, other dezagregant and anticoagulants, medicines with vazoaktivny action apply. Purpose of inhibitors of proteases is effective.
Taking into account haemo dynamics indicators to patients with a syndrome of HELLP carry out the individualized hypotensive therapy added with spazmolitika. For prevention of possible infectious complications use antibiotics except for aminoglycosides which possess gepato-and nefrotoksichny action. According to indications appoint nootropic and tserebroprotektorny medicines, vitamin and mineral complexes. At emergence of displays of a sharp renal failure depending on expressiveness of frustration carry out a plasma exchange and a hemodialysis.
Forecast and prevention
Forecast of HELLP syndrome always serious. In the past the lethality at a disease reached 75%. Now thanks to timely diagnostics and pathogenetic methods of therapy maternal mortality managed to be reduced to 25%. In the preventive purposes early registration in antenatal clinic and constant observation of the obstetrician-gynecologist is recommended to povtornorodyashchy women with chronic somatic diseases. At detection of signs of a gestoz careful performance of appointments of the attending physician, normalization of a diet, observance of the mode of a dream and rest is important. Bystry deterioration in a condition of the pregnant woman with emergence of symptomatology of a heavy eklampsiya and preeklampsiya is the indication to the emergency hospitalization in an obstetric hospital.