Hemolytic disease of the newborn - is non-physiological development of hemolytic anemia isoimmune type arising as a result of the incompatibility of erythrocyte antigens of the fetus and the mother's blood, which contains a high concentration of antibodies to these antigens.Level diagnosing such diseases as "hemolytic disease of the newborn" is 0.6%, and the rate of perinatal mortality according to global statistics do not exceed 2.5%.
Causes of hemolytic disease of the newborn
based etiopathogenetic mechanism of hemolytic disease of the newborn immune put the development of the conflict that occurs when the condition of "antigen-negative" mother and "antigen-positive" unborn child.In order to develop the classical hemolytic disease of the fetus, a prerequisite is a preliminary sensitization of the mother's body, that is, of the facts subsequent pregnancy.In a situation where hemolytic disease of the blood group incompatibility develops, the fact sensitization mother
pathogenetic mechanisms of development of hemolytic disease of the newborn starts in the moment of impact erythrocyte antigen-positive blood cells in the body of antigen-negative pregnant women.What matters most is the amount of fetal blood that gets into the mother's blood.There is a whole spectrum is not modifiable risk factors that contribute to the development of hemolytic disease of the newborn as previous abortions and miscarriages, the fact of having an ectopic pregnancy in history, the use of invasive diagnostic techniques and the threat of termination of pregnancy in the past.
basis of the disease is the intracellular erythrocyte hemolysis that occurs in the second trimester of pregnancy and after delivery sharply progressive.After entering erythrocyte antigen-positive cells by the fetus fetoplacental barrier in the mother mechanisms are activated or enhanced generation antirhesus antigruppovyh antibodies.Group IgG antibody produced freely come back into the bloodstream of the fetus, and a result of their exposure to the antigen the fetus develops active intracellular erythrocytic hemolysis.
During the first pregnancy erythrocyte hemolysis does not occur due to insufficient amount of red blood cells of the fetus, as well as by immunosuppressive mechanisms of the mother's body, but the process is started sensitizing the mother having a value in the development of hemolysis in subsequent pregnancies.
percentage of the possible development of hemolytic disease of the newborn child of the ABO system is 10%, but the complexity of this disease lies in the fact that erythrocyte hemolysis already formed during the first pregnancy.Despite this, this form of the disease is characterized by a favorable flow and minimal risk of complications.
to edematous form of hemolytic disease of the newborn is characterized by the development of erythrocyte hemolysis at the stage of fetal development from 20 weeks of pregnancy.As a result of progressive anemia in the fetus develops total hypoxic defeat of all structures and tissues, accompanied by severe metabolic disturbances and damage to the vascular walls.As a result, damage to vessel walls, the conditions for release of excess water from the blood plasma albumin in fetal interstitial interstitium while reducing protein production in the liver parenchyma.
Long lasting hypoproteinemia inevitably leads to the formation of diffuse edema syndrome in utero, and severe heart failure.Due to the fact that the resulting bilirubin resulting erythrocyte hemolysis enters the body through the mother fetoplacental barrier newborn baby does not develop jaundice skin.
icteric current version of hemolytic disease of the newborn develops when the erythrocyte hemolysis developed in the last weeks of pregnancy or immediately before delivery.Due to the fact that as a result of intensive destruction of a large number of erythrocytic cells is released high concentrations of bilirubin.The skin of a newborn baby becoming icteric coloration as a result of excessive accumulation of bilirubin in their structures.More severe complications arise in a situation where indirect bilirubin accumulates in the neurons of the brain, as this situation develops irreversible bilirubin encephalopathy.As a result, excessive excretion of conjugated bilirubin biliary ways, conditions are created for the stagnation of bile, and the development of signs of cholestatic syndrome.
Anemic version of hemolytic disease of the newborn is the most favorable, as in the pathogenesis of development holds the minimum amount of flow of antibodies in the blood of a fruit just before the birth, so erythrocyte hemolysis is a low-intensity in nature.
Signs and symptoms of hemolytic disease of the newborn
most unfavorable course is accompanied by swollen version of hemolytic disease of the newborn, and the anemic current version can all asymptomatic.Despite the fact that each of the clinical forms of this disease is different development of specific pathognomonic symptoms, there are a number of clinical manifestations characteristic of any current version of hemolytic disease of the newborn.These clinical symptoms should be referred pronounced pallor of the mucous membranes and hepatosplenomegaly.
the edematous form of hemolytic disease of the newborn has been a rapid progression of symptoms of edema, which manifests itself in the accumulation of large amounts of fluid in natural cavities in the form of exudative pleurisy, pericarditis, ascites.Due to prolonged hypoxia affecting the fetus, the conditions for the development of DIC, hemodynamic and respiratory disorders.In a situation where a child is born viable, visually marked a sharp diffuse puffy, pasty skin.An objective examination of the newborn revealed a dramatic expansion of cardiac dullness, muffled heart sounds, no vesicular breathing over a large area of the lung fields.
most common clinical variant of hemolytic disease of the newborn is jaundiced, pathognomonic symptom is discoloration, not only the skin of a newborn baby, and vernix, amniotic fluid and umbilical cord.Most often, the above changes are observed immediately after delivery or within the first days of life.Given the severity of the clinical and laboratory changes are divided icteric form of hemolytic disease of the newborn on the severity.
Mild jaundice syndrome is set when ikterichnost skin occurs no earlier than two days after birth, and the rate of bilirubin in the blood withdrawn from the umbilical cord does not exceed the 51 mmol / l.In this state, there is no significant increase in spleen and liver parameters, and in some situations, their dimensions remain normal.
Jaundice moderate severity is different early onset (immediately after delivery) and a significant concentration of bilirubin in the umbilical cord blood in excess of 68 mmol / l.Moderate jaundice is always accompanied by a significant increase in the size of the liver and spleen.
diagnosis of hemolytic disease of the newborn, accompanied by severe jaundice syndrome, is the product of the placenta ultrasound, amniocentesis to determine bilirubin absorbance.In a situation where a severe form of hemolytic disease can not be timely medical correction, develop severe complications such as kernicterus.
Symptoms indicating the development of kernicterus, intoxication syndrome is a weakness, abnormal yawning, lack of appetite, vomiting, muscle hypotonia.Severe intoxication defeat structures of central nervous system characterized by the development of the situation of forced child in bed with opisthotonos, the advent of "brainstorming" crying bulging fontanelle large, the disappearance of inborn reflexes, increased convulsive and pathological oculomotor symptoms.
Unfortunately, isolated anemic form of hemolytic disease of the newborn is the category of the rarest.With this clinical variant of the child is not observed serious health problems and are more diagnosis is made after a study of the placenta, which is characterized by large size due to the development of edema.
In situations where the mother's blood is produced by a large number of critical antibodies with further entering into the bloodstream of the fetus, fetal death occurs at any stage of pregnancy.In general, women have suffered rhesus conflict, say heavy during pregnancy because of toxicity and injury patterns hepatobiliary system.
for timely diagnosis of hemolytic disease of the newborn is essential comprehensive survey of women during pregnancy, including an immunoassay, ultrasound Doppler fetoplacental blood flow, amniocentesis with subsequent amniocentesis, cordocentesis followed by analysis of the blood of the fruit.
immunoassay allows not only to determine whether existing antibodies in the mother's blood, but also to analyze their concentration, as well as the dynamics of the growth of the title.When conducting ultrasound necessarily measured volume and the area of the placenta, it is rendered possible local thickening hydramnion detected and determined in the early stages of fluid accumulation in the abdomen of the fetus, which is defined as a significant increase in the size of the stomach.Amniotic fluid are studied in order to determine the increase of the optical density of bilirubin, which takes place at the intracellular erythrocyte hemolysis fruit blood.
forms of hemolytic disease of the newborn
based division of hemolytic disease of the blood of the fetus by categories laid species pathogenic form of the disease variants.The main category of patients with hemolytic disease among newborns, in whom there is incompatibility with the mother erythrocyte antigens, in particular Rh affiliation.The second category of patients are aged newborn patients who develop the clinical picture of hemolytic anemia due to blood group incompatibility with the mother.Hematology allocated another specific form of hemolytic disease of the fetus, the occurrence of which is caused by the incompatibility of rare hematological factors (Lutheran, Kell).
Depending on the specificity of the clinical course of the disease and the prevalence of various clinical and laboratory syndrome hemolytic disease of children is divided into three basic forms:
1. Otechny option is a combination of the main symptom of anemia with massive edema syndrome.
2. When icteric form of hemolytic disease of the newborn to the fore jaundice syndrome in combination with severe anemic syndrome.
3. Anemic form is considered the most favorable in regard to the effect on the health of the child and also the most difficult to diagnose.
In a situation where the child's time in the treatment measures are taken, there is uncomplicated variant of the flow of hemolytic disease and accession complications of the underlying disease is observed a complicated shape, are extremely severe progressive course and a high risk of death.
treatment of hemolytic disease of the newborn
situation existing in rhesus crucial early the diagnosis of hemolytic disease in the early antenatal period with an accompanying assessment of severity and prognosis for the viability of the child.Possible effective therapeutic measures are divided on the basis of invasiveness.
noninvasive method of correcting haemolysis is plasmapheresis and immunoglobulinoterapiya pregnant woman.Despite the fact that plasmapheresis has good immunocorrective and detoxication effect, there is a range of absolute contraindications to its use as a pregnant woman existing severe heart disease, severe anemia, hypoproteinemia, immunodeficiency and allergic reactions to the colloidal preparations.
The aim is to reduce the use of immunoglobulinoterapii production of antibodies, which used intravenous immunoglobulin estimated dose of 0.4 g per 1 kg of body weight of a pregnant woman.This dose is distributed for 5 days, which is one course.The frequency of the exchange rate of immunoglobulin therapy every three weeks for the entire pregnancy.This method applies only in the case of moderate course of the disease.
greater effectiveness against signs of leveling hemolytic disease of the fetus invasive techniques as cordocentesis and antenatal blood transfusions that are pathogenetically appropriate in regard to the removal of intrauterine hemolysis.For use cordocentesis should take place in the form of strict indications burdened obstetric history (available credible evidence of fetal death from hemolytic disease of the fetus), the high antibody titer greater than 1:32 ratio, the presence of ultrasound signs of hemolytic disease, high optical density of bilirubin in the amniotic fluid.The optimal period for cordocentesis is the period 24-35 weeks of pregnancy.
Fetal blood transfusions used only in case of critical hemoglobin and hematocrit, which are used only Rh-negative red blood cells are first washed blood.This procedure is performed only once.
Therapy hemolytic disease after giving birth is to use techniques aimed at eliminating hyperbilirubinemia, anemia, and the use of drug symptomatic therapy.Newborn children with hemolytic disease, subject to artificial feeding, as in human breast milk is concentrated a large number of antibodies that can exacerbate the underlying disease.
To correct giperbilirubinemicheskogo syndrome are currently used both conservative and surgical methods.Under the conservative therapy is meant phototherapy and parenteral immunoglobulinoterapii.The therapeutic effect of phototherapy is the formation of a water-soluble isomer indirect bilirubin in the skin and subcutaneous fat layer under the influence of photoirradiation skin.Synthesized lyumirubin later eliminated from the body with urine and bile.To achieve a good result in the form of elimination of yellowness of the skin of a newborn baby and reduce bilirubin, should carefully monitor the implementation of all technical components of phototherapy, that is, to be an adequate loading dose, a child must be in the incubator with the imposed remedies on the periorbital region and genitals.The duration of a continuous course of phototherapy on average five days, but there is an absolute criterion for termination of exposure of a newborn baby, are to reduce bilirubin less than 170 mmol / l.In order to avoid complications from the use of phototherapy, should abandon this type of treatment if the child has signs of cholestatic syndrome.