Hemolytic disease of the newborn


Hemolytic disease of the newborn (HDN) - a pathological condition of the newborn, accompanied by a massive breakdown of red blood cells, is one of the main causes of jaundice in newborns.

Hemolytic disease of newborns is diagnosed in 0.6% of newborns. Hemolytic disease of newborns is manifested in 3 main forms: anemic, icteric, edematous.


Hemolytic disease of the newborn (morbus haemoliticus neonatorum) - hemolytic anemia of the newborn, due to the incompatibility of the blood of the mother and the fetus according to the Rh factor, blood group and other blood factors. The disease is observed in children from the moment of birth or is detected in the first hours and days of life.

Hemolytic disease of the newborn, or fetal erythroblastosis, is one of the most serious diseases of children in the neonatal period. Appearing in the antenatal period, this disease can be one of the causes of spontaneous abortions and stillbirths. According to the WHO (1970), hemolytic disease of newborns is diagnosed in 0.5% of newborns, the mortality from it is 0.3 per 1000 children born alive.

Etiology, causes of hemolytic disease of the newborn.

The cause of hemolytic disease of the newborn became known only in the late 40s of the 20th century. in connection with the development of the doctrine of the Rh factor. This factor was discovered by Landsteiner and Wiener in 1940 in monkeys Macacus rhesus. Later, the same researchers found that Rh factor is present in red blood cells of 85% of people.

Further studies have shown that hemolytic disease of the newborn may be due to the incompatibility of the blood of the mother and fetus, both in the Rh factor and in the blood group. In rare cases, the disease results from the incompatibility of the blood of the mother and the fetus due to other blood factors (M, N, M5, N3, Rell, Kidd, Luis, etc.).

Rh factor is in the stroma of the erythrocytes. He has no connection with gender, age, and membership in ABO and MN systems. There are six main antigens of the Rh system, inherited by three pairs of genes and designated either C, C, D, d, E, E (according to Fisher), or rh ', hr', Rh0hr0, rh ", hr" (according to Winner). In the occurrence of hemolytic disease of the newborn, the most important is the D-antigen, which is absent in the mother and present in the fetus as a result of its inheritance from the father.

Hemolytic disease of the newborn, due to incompatibility of the ABO system, is more common in children with blood groups A (II) or B (III). The mothers of these children have 0 (I) blood type, which contains agglutinins α and β. The latter can block fetal red blood cells.

It was found that mothers whose children were born with manifestations of hemolytic disease, in most cases, even before the onset of this pregnancy, were sensitized to the erythrocyte antigens of this fetus due to previous blood transfusions, as well as pregnancies of the Rh-positive fetus.

Currently, there are three types of Rh antibodies that are formed in a sensitized body of people with Rh-negative blood: 1) complete antibodies, or agglutinins, 2) incomplete or blocking, 3) hidden.

Complete antibodies are antibodies capable of causing normal agglutination of erythrocytes specific for a given serum through a normal contact, this reaction does not depend on the salt or colloidal state of the medium. Incomplete antibodies can cause agglutination of erythrocytes only in a medium containing high-molecular substances (serum, albumin, gelatin). Hidden Rh antibodies are found in human serum with Rh negative blood in very high concentrations.

In the occurrence of hemolytic disease of the newborn, the most important role is played by incomplete Rh antibodies, which are able to easily penetrate the placenta into the fetus because of the small size of the molecule.

Pathogenesis. Hemolytic disease of the newborn

The normal course of pregnancy involves the synthesis of antibodies by a woman to genetically alien antigens of a paternal origin that come to her. It has been established that in the placenta and amniotic fluid maternal antibodies are bound by fetal antigens. With prior sensitization, with a pathological course of pregnancy, the barrier functions of the placenta are reduced, and maternal antibodies can flow to the fetus. This is most intense during childbirth. Therefore, hemolytic disease of newborns usually begins after birth.

In the pathogenesis of hemolytic disease, hemolysis of erythrocytes in the fetus or newborn child is of primary importance due to damage to the red cell membrane by maternal antibodies. This leads to premature extravascular hemolysis. With the breakdown of hemoglobin, bilirubin is formed (35 mg of bilirubin is formed from each gram of hemoglobin).

Intensive hemolysis of erythrocytes and enzymatic immaturity of the liver of the fetus and the newborn child lead to the accumulation in the blood of free (indirect) bilirubin, which has toxic properties. It is insoluble in water, is not excreted in the urine, but easily penetrates into tissues rich in lipids: the brain, adrenal glands, and liver, disturbing the processes of cellular respiration, oxidative phosphorylation, and transport of some electrolytes.

Severe complication of hemolytic disease is nuclear jaundice (kernicterus), caused by the toxic effect of indirect bilirubin on the nuclei of the base of the brain (subthalamic, hypocampus, striatal body, cerebellum, cranial nerves). Prematurity, acidosis, hypoalbuminemia, infectious diseases, as well as a high level of indirect bilirubin in the blood (more than 342 μmol / l) contribute to this complication. It is known that when the serum bilirubin level is 342-428 µmol / l, nuclear jaundice occurs in 30% of children.

In the pathogenesis of hemolytic disease of the newborn, a role is played by impaired function of the liver, lungs, and cardiovascular system.

Symptoms Flow. The clinical picture of hemolytic disease of the newborn.

Three forms of hemolytic disease of the newborn are clinically distinguished: swollen, icteric, and anemic.

Edematous form is the most severe. For it, pronounced edema with fluid accumulation in the cavities (pleural, abdominal), pallor of the skin and mucous membranes, a significant increase in the size of the liver and spleen are hacharacteristic. Some newborns have small bruises and petechiae.

Large changes are observed in the composition of peripheral blood. In such patients, the amount of hemoglobin is reduced to 30-60 g / l, the number of erythrocytes often does not exceed 1x10 12 / l, anisocytosis, poikilocytosis, polychromasy, norm and erythroblastosis are expressed, the total number of leukocytes is increased, neutrophilia is marked with a sharp shift to the left. Anemia in such children is so severe that in combination with hypoproteinemia and damage to the capillary wall leads to the development of heart failure, which is considered the main cause of death before or after the birth of the child.

The icteric form is the most common clinical form of hemolytic disease of the newborn. The first symptom of the disease is jaundice, which occurs on the 1-2 day of life. The intensity and hue of jaundice gradually change: first orange, then bronze, then lemon, and finally the color of unripe lemon. There is icteric staining of the mucous membranes, sclera. The size of the liver and spleen increases. In the lower abdomen there is pasty tissue. Children become lethargic, adynamic, poorly suck, they have reduced reflexes of newborns.

In the study of peripheral blood revealed anemia of varying severity, pseudoleukocytosis, which occurs due to an increase in young nucleated cells of the red row, perceived in Goryaev’s cell as leukocytes. The number of reticulocytes increases significantly.

For the icteric form of hemolytic disease of the newborn, an increase in the level of indirect bilirubin in the blood is characteristic. Already in cord blood, its level may be higher than 60 μmol / l, and later it reaches 265-342 μmol / l and more. There is usually no clear connection between the degree of yellowness of the skin, the severity of anemia and the severity of hyperbilirubinemia, but it is believed that the yellowness of the palms indicates a bilirubin level of 257 µmol / L and above.

Severe complications of the icteric form of hemolytic disease of the newborn are damage to the nervous system and the development of nuclear jaundice. When these complications occur, the child first develops lethargy, decreased muscle tone, the absence or inhibition of the Moro reflex, regurgitation, vomiting, and pathological yawning. Then classical signs of nuclear jaundice appear: muscular hypertension, stiff neck, forced posture with opisthotonus, stiff limbs, fists clenched with brushes, a sharp “brain” cry, hyperesthesia, bulging of a spring, facial muscle twitching, convulsions, a symptom of the “setting sun” , nystagmus, a symptom of Grefe, apnea periodically occurs.

Another relatively common complication is bile thickening syndrome. His signs are discolored stools, saturated urine color, liver enlargement. In the study of blood detect an increase in the level of direct bilirubin.

Anemic form is observed in 10-15% of patients with hemolytic disease of newborns. Early and persistent symptoms should be considered a general pronounced lethargy and pallor of the skin and mucous membranes. Pallor is clearly detected by the 5th – 8th day after birth, since at first it is masked by a small jaundice. An increase in the size of the liver and spleen.

In the peripheral blood in this form, the hemoglobin content is reduced to 60-100 g / l, the number of erythrocytes is within 2.5x10 12 / l-3.5x10 12 / l, normoblastosis, reticulocytosis is observed. Bilirubin levels are normal or moderately elevated.

The diagnosis of hemolytic disease of the newborn is based on anamnesis (mother sensitization due to previous blood transfusions, birth of children in the jaundice family, their death in the neonatal period, mother's instructions on her previously late miscarriages, stillbirths), and on the assessment of clinical symptoms and data laboratory research. The latter in the diagnosis of the disease are of leading importance.

First of all, the determination of the blood group and Rh-accessories of the mother and child is carried out, the content of reticulocytes in the peripheral blood and the level of bilirubin in the venous blood of the child are examined.

In the case of Rh incompatibility, the titer of Rh-antibody in the blood and milk of the mother is determined; a direct Coombs test is performed with the child's red blood cells and indirectly with the mother's blood serum. If the AVO system is incompatible in the mother’s blood and milk, the titer of a- or p-agglutinins in salt and protein media is determined. Immune antibodies in the protein medium have a titer four times higher than in salt. These antibodies belong to class G immunoglobulins and cross the placenta, causing the development of hemolytic disease of the newborn. Coombs' direct reaction to ABO-incompatibility is usually negative.

If clinical and laboratory data clearly indicate hemolysis, and the mother’s and child’s blood are compatible with the Rh factor and ABO system, then it is advisable to put the Coombs reaction, test for individual compatibility of the mother’s blood and the child’s red blood cells, look for antibodies to antigens that rarely cause hemolytic disease newborns: s, d, e, kell, diffy, kidd.

For antenatal diagnosis, the determination of bilirubin in the amniotic fluid with a gestational age of 32-38 weeks is of prognostic importance: with optical spectrophotometric density of amniotic fluid (with a filter of 450 nm) of 0.15-0.22 units. develops a mild form of hemolytic disease of the newborn, above 0.35 units. - severe form. Edematous form of hemolytic disease of the newborn in the antenatal period can be diagnosed using ultrasound.

The detection of women sensitized to Rh antigens is facilitated by the determination of rhesus antibody titer in the blood of pregnant women. However, it is not always the degree of increase in titer of Rh antibodies in the blood of a pregnant woman corresponds to the severity of hemolytic disease. Prognostically unfavorable consider prancing rhesus antibody titer of a pregnant woman.

Diagnostics. Differential diagnosis of hemolytic disease of the newborn.

Hemolytic disease of the newborn has to be differentiated from a number of diseases and physiological conditions. The first step is to establish the hemolytic nature of the disease and to exclude hyperbilirubinemia of hepatic and mechanical origin.

Among the causes of the appearance of jaundice of the second group in newborns, congenital diseases of an infectious nature are most important: viral hepatitis, syphilis, tuberculosis, listeriosis, toxoplasmosis, cytomegalovirus infection, and sepsis, acquired not only in utero, but after birth.

Common signs of jaundice in this group are as follows: the absence of signs of hemolysis (anemia, signs of irritation of the red blood row, an increase in the level of indirect bilirubin, an increase in the spleen) and an increase in the level of direct bilirubin.

It should also be remembered that in newborns, obstructive jaundice may occur, which usually appears due to an abnormal development of the biliary tract - agenesis, atresia, stenosis and cysts of the intrahepatic bile ducts. In these cases, jaundice appears usually by the end of the 1st week, although it may appear in the first days of life. It is progressively enhanced, and the skin acquires a dark green, and in some cases a brownish tint. Feces may be slightly colored. With anomalies of biliary tract development, the amount of bilirubin in the serum is very high, it can reach 510-680 μmol / l due to an increase in direct bilirubin. In severe and advanced cases, indirect bilirubin may increase due to the impossibility of conjugating it due to an overflow of liver cells with bilirubin of bile. Urine is dark and stains diapers yellow. The amount of cholesterol and alkaline phosphatase is usually increased. The liver and spleen are enlarged and with increased jaundice compacted. Gradually, children develop dystrophy, there are signs of hypovitaminosis K, D and A. Biliary cirrhosis develops, from which children die before reaching 1 year of age.

With a high level of indirect bilirubin in the blood and in the absence of other signs of increased hemolysis of red blood cells, the conjugation nature of jaundice is suspected. In such cases, it is advisable to investigate the activity of lactate dehydrogenase and its first fraction, hydroxybutyrate dehydrogenase, in the blood serum of a child. In hemolytic disease of the newborn, the level of these enzymes is sharply increased, and in conjugation jaundice corresponds to the age norm.

We must not forget about the existence of a rather rare disease known as Kriegler-Nayar syndrome (Krigler and Najar). This is non-hemolytic hyperbilirubinemia, accompanied by the development of nuclear jaundice. The disease is inherited in an autosomal recessive manner. Boys are sick more often than girls.

The basis of the Crigler-Nayar syndrome is a sharp violation of the formation of bilirubing diglucoronide (direct bilirubin) due to the complete absence of UDP-glucoronyl-transferase conjugating bilirubin. The main symptom of the disease is jaundice, which appears on the first day after birth and grows intensively, being held throughout the life of the child. Jaundice is associated with a sharp increase in blood indirect bilirubin, the amount of which very quickly reaches 340-850 μmol / l. Against the background of a sharp increase in the blood of indirect bilirubin, symptoms of nuclear jaundice develop. Anemia is not observed. The number of young forms of erythrocytes is not increased. The amount of urobilin in the urine is within normal limits. Bile lacks direct, conjugated bilirubin. Damage to the central nervous system leads to the death of a child in the first months of life. Children rarely live to 3 years.

Hereditary hemolytic anemias are diagnosed on the basis of (specific morphological signs of erythrocytes, measurements of their diameter, osmotic stability, studies of the activity of erythrocyte enzymes (primarily glucose-6-phosphate dehydrogenase, etc.), hemoglobin types.

Treatment of hemolytic disease of the newborn.

Treatment of hemolytic disease of newborns with a high level of indirect bilirubin can be conservative or operative (operation of interchangeable blood transfusion).

For newborns with hemolytic disease, adequate nutrition is very important.

Conservative treatment of hemolytic disease of the newborn includes the following measures:

  1. мероприятия, направленные на уменьшение гемолиза за счет стабилизации мембраны эритроцитов (внутривенные вли­вания 5% раствора глюкозы, назначение АТФ, эревита),
  2. терапию, способствующую ускорению метаболизма и экскреции билирубина из организма (прием фенобарбитала из расчета до 10 мг/кг в сутки, разделенный на три приема, внутрь),
  3. administration of substances that adsorb bilirubin in the intestine and accelerate its excretion with feces (agar-agar 0.1 g three times a day orally, 12.5% ​​solution of xylitol or magnesium sulfate orally 1 teaspoon three times a day or allohol by ' /2 crushed dragees are also taken three times a day inside),
  4. the use of tools and measures to reduce the toxicity of indirect bilirubin (phototherapy), recently there have been reports of effectiveness in combating the toxic effects of indirect bilirubin of small doses of ultraviolet irradiation.

It is useful to carry out infusion therapy. The volume of infusion therapy is as follows: on the first day - 50 ml / kg and then add 20 ml / kg per day, bringing to 150 ml / kg by the 7th day.

The composition of the infusion solution: 5% glucose solution with the addition of 1 ml of 10% calcium solution for every 100 ml, 1 mmol of sodium and chlorine from the second day of life, 1 mmol of potassium from the third day. Infusion rate - 3-5 drops in 1 min. The addition of 5% albumin solution is indicated only in children with infectious diseases, prematurely, when hypoproteinemia is detected (below 50 g / l). Infusion of hemodez and reopoliglukina with hemolytic disease of the newborn is not shown.

Replacement blood transfusion is carried out for certain indications. The absolute indication for replacement blood transfusion is hyperbilirubinemia above 342 µmol / L, as well as the rate of increase of bilirubin above 6 µmol / L at 1 hour, its level in cord blood above 60 µmol / L.

Indications for replacement blood transfusion in the first days of life are anemia (hemoglobin less than 150 g / l), normoblastosis and proven incompatibility of the blood of the mother and the child in a group or rhesus factor.

In the case of Rh-conflict, blood of the same group as that of the child is used for replacement blood transfusions, no more than 2–3 days of conservation of Rh-negative, in the amount of 150–180 ml / kg (when the level of indirect bilirubin is more than 400 μmol / l - in the amount of 250-300 ml / kg). In an ABO conflict, blood of group 0 (I) is transfused with a low titer of a- and ß-agglutinins, but in an amount of 250-400 ml, while, as a rule, the next day, a second replacement transfusion should be performed in the same volume. If a child is simultaneously incompatible with resuz- and ABO-antigens, then the child must be transfused with blood 0 (I) of the group.

When performing a replacement blood transfusion, a catheter is inserted into the umbilical vein for a length of not more than 7 cm. Blood should be warmed to a temperature of at least 28 ° C. Suction is carried out before the operation. Begin the procedure with the removal of 40-50 ml of baby’s blood, the amount of injected blood should be 50 ml more than that taken out. The operation is carried out slowly (3-4 ml in 1 min), alternating removal and the introduction of 20 ml of blood. The duration of the entire operation is at least 2 hours. It should be remembered that for every 100 ml of injected blood, 1 ml of 10% calcium gluconate solution must be injected. This is done to prevent citrate shock. 1-3 h after the replacement of blood transfusion should determine the level of glucose in the blood.

Complications of replaceable blood transfusion include: acute heart failure with the rapid introduction of large amounts of blood, cardiac rhythm disturbances, transfusion complications with an incorrect donor selection, electrolyte and metabolic disorders (hyperkalemia, hypocalcemia, acidosis, hypoglycemia), hemorrhagic syndrome, thrombosis and embolism, infectious complications (hepatitis, etc.), necrotic enterocolitis.

After the replacement of blood transfusion prescribed conservative therapy. The indication for re-replacement blood transfusion is the rate of increase in indirect bilirubin (replacement blood transfusion is indicated at a bilirubin growth rate of more than 6 µmol / l per hour).

To perform a replacement blood transfusion, you must have the following set of tools: sterile polyethylene catheters No. 8, 10, bell-shaped probe, scissors, two surgical tweezers, needle holder, silk, four-six syringes with a capacity of 20 ml and two-three syringes with a capacity of 5 ml, two glasses on 100-200 ml.

The technique of catheterization of the umbilical vein is as follows: after processing the operative field, the end of the umbilical cord is cut across at a distance of 3 cm from the umbilical ring, the catheter is inserted by careful rotational movements, guiding it after passing the umbilical ring up along the abdominal wall, towards the liver. If the catheter is inserted correctly, blood is secreted through it.

Prevention of hemolytic disease of the newborn.

The basic principles for the prevention of hemolytic disease of the newborn are as follows. First, given the great importance of prior sensitization in the pathogenesis of hemolytic disease of the newborn, each girl should be treated like a future mother, and therefore the girls need to carry out blood transfusion only for life reasons. Secondly, an important place in the prevention of hemolytic disease of the newborn is given to work to explain to women the harm of abortion. To prevent the birth of a child with hemolytic disease of the newborn, the introduction of anti-O-globulin in an amount of 250-300 μg, which promotes the rapid elimination of the red blood cells of the child, is recommended for all women with Rh-negative blood factor on the first day after abortion (or after delivery) mothers, preventing the synthesis of Rh antibodies by the mother. Thirdly, pregnant women with a high titer of antiresus antibodies are hospitalized for 12-14 days in the antenatal wards for periods of 8, 16, 24, 32 weeks, where they are given non-specific treatment: intravenous fluids of glucose with ascorbic acid, cocarboxylase, are prescribed rutin, vitamin E, calcium gluconate, oxygen therapy, with the development of threatened abortion prescribe progesterone, endonasal electrophoresis of vitamins B1, C. For 7-10 days before delivery, the administration of phenobarbital is shown in a dose of 100 mg three times a day. Fourth, with the increase in titer of antiresus antibodies in a pregnant woman, delivery is carried out ahead of schedule at the 37-39th week by cesarean section surgery.

Consequences and prognosis of hemolytic disease of the newborn.

Hemolytic disease of newborns: the consequences can be dangerous, up to the death of the child, the liver and kidney functions in the child can be disturbed. It is necessary to begin treatment immediately.

The prognosis of hemolytic disease of the newborn depends on the form of the disease and the adequacy of the preventive and therapeutic measures taken. Patients with edematous form are not viable. The prognosis for icteric form is favorable, provided that adequate treatment is provided, the prognostically unfavorable development of bilirubin encephalopathy and nuclear jaundice, since in the group of such patients the percentage of disability is very high. The anemic form of hemolytic disease of the newborn is prognostically favorable, in patients with this form, self-healing is observed.

The modern level of development of medicine, the correct diagnostic and therapeutic tactics allow to avoid the pronounced effects of hemolytic disease of the newborn.

Symptoms of hemolytic disease of the newborn

Clinical symptoms depend on the form of the disease.

  • Edematous form (or fetal dropsy) is rare.
    • It is considered the most severe form among others.
    • As a rule, it begins to develop in utero.
    • Often there are miscarriages in early pregnancy.
    • Sometimes the fetus dies late in life or is born in a very serious condition with widespread edema, severe anemia (decrease in hemoglobin (blood dye that carries oxygen) and red blood cells per unit volume of blood), oxygen starvation, and heart failure.
    • The skin of such a newborn is pale, waxy in color. Face rounded. The muscle tone is sharply reduced, the reflexes are depressed.
    • Significantly enlarged liver and spleen (hepatosplenomegaly). The belly is large, barrel-shaped.
    • Common tissue edema is characteristic, sometimes with effusion (accumulation of fluid that has come out of small vessels) into the abdominal cavity, cavities around the heart (pericardial) and lungs (pleural). This is due to the increased capillary permeability (the thinnest vessels in the body) and a decrease in total protein in the blood (hypoproteinemia).
  • Anemic form - this is the most favorable form of the flow.
    • Clinical symptoms appear in the first days of a child’s life.
    • Gradually, anemia, pallor of the skin and mucous membranes, an enlarged liver and spleen in size is progressing.
    • The general condition suffers slightly.
  • The icteric form is the most common form. Its main symptoms are:
    • jaundice (yellow staining of body tissues due to excessive accumulation of bilirubin in the blood (bile pigment) and its metabolic products),
    • anemia (decrease in hemoglobin (blood dye that carries oxygen) and red blood cells per unit volume of blood),
    • hepatosplenomegaly (enlarged liver and spleen in size).
  • Jaundice develops in the first 24 hours after the birth of a child, less often on the second day, has a progressive course.
    • The skin of such a patient has a yellow color with an orange tint.
    • Visible mucous membranes and sclera turn yellow.
    • The sooner jaundice appears, the more severe the disease is.
    • As the level of bilirubin in the blood increases, children become lethargic, drowsy, their reflexes and muscle tone decrease.
    • For 3-4 days, the level of indirect bilirubin (bile pigment formed as a result of the breakdown of hemoglobin and not having time to pass through the liver) reaches a critical value (more than 300 µmol / l).
    • Symptoms of nuclear jaundice appear (damage to the subcortical nuclei of the brain by indirect bilirubin):
      • motive concern
      • stiff muscles of the neck (a sharp increase in muscle tone),
      • opisthotonus (convulsive posture with a sharp back arch, with head tilted back (resembles an arc with support only on the back of the head and heels), stretching the legs, flexing the arms, hands, feet and fingers),
      • the symptom of the “setting sun” (the movement of the eyeballs is directed downwards, while the iris covers the lower eyelid). All this is accompanied by a squeak and a strong cry (“brain” piercing cry).
    • By the end of the week, against the background of massive breakdown of red blood cells, bile excretion in the intestine decreases (bile thickening syndrome) and signs of cholestasis (stagnation of bile) appear: the skin becomes greenish-dirty, the feces become discolored, the urine darkens, and the level of direct bilirubin increases that went through the liver and got harmless).

The cause of hemolytic disease of the newborn is the incompatibility of the blood of the mother and the fetus, most often by the Rh factor, less commonly by group antigens (ABO systems) and only in a small percentage of cases by other antigens.

  • Rhesus conflict occurs when a developing fetus has Rh-positive blood in a Rh-negative woman.
  • The immune conflict in the AVO system develops in the O (I) -group of the mother’s blood and A (II) or B (III) in the fetus.
  • A child is born sick only if the mother has previously been sensitized (has already increased sensitivity to blood components that she has encountered before).
  • The Rh-negative woman can be sensitized as a result of transfusion of Rh-positive blood even in early childhood, with miscarriage, especially with induced abortion.
  • The most common cause of sensitization (increased sensitivity of the body to the effects of any environmental factor or internal environment) is childbirth. Therefore, the first child is in a much more favorable position than subsequent children.
  • With the development of conflict in the ABO system, the number of previous pregnancies is irrelevant, since in normal life sensitization (increased sensitivity to foreign agents for the body) to antigens A and B occurs very often (for example, with food, with vaccinations, some infections).
  • A significant role in the development of hemolytic disease is played by the placenta (a special organ that communicates between the mother and baby during pregnancy). In case of violation of its barrier function, the transition of the red blood cells of the fetus into the mother’s bloodstream and mother’s antibodies to the fetus is facilitated.
  • Together with erythrocytes, foreign proteins enter the mother's body (Rh factor, antigens A and B).
    • They cause the formation of Rh antibodies or immune antibodies (anti-A or anti-B), which cross the placenta into the bloodstream of the fetus.
    • Antigens and antibodies unite on the surface of erythrocytes, form complexes that destroy them (hemolysis of fetus and newborn erythrocytes).
  • As a result of the pathological breakdown of red blood cells in the blood of the fetus, the level of indirect (unconjugated) bilirubin increases, and anemia develops.
  • Unconjugated bilirubin has a toxic effect on brain cells, causing significant changes in them up to necrosis.
  • At a certain concentration (over 340 µmol / l in full-term and over 200 µmol / l in very premature babies), it can penetrate the blood-brain barrier (the physiological barrier between the circulatory system and the central nervous system) and damage the subcortical nuclei of the brain and the cortex, which leads to the development of nuclear jaundice.
  • This process is exacerbated by reducing the level of albumin (blood protein), glucose, with the use of certain drugs, such as steroid hormones, antibiotics, salicylates, sulfonamides.
  • As a result of toxic damage to the liver cells, direct bilirubin (neutralized by the liver) appears in the blood.
  • In the bile ducts, cholestasis (stagnation of bile) occurs, disruption of bile in the intestine is disturbed.
  • With anemia (decrease in hemoglobin (blood dye that carries oxygen) and red blood cells in a unit of blood volume, new foci of hematopoiesis appear due to hemolysis of red blood cells.
  • Erythroblasts (young forms of red blood cells) appear in the blood.

A pediatrician will help in the treatment of the disease.

Complications and consequences

In severe cases of this disease, the prognosis is poor. Often occurs:

  • perinatal (from the 28th week of pregnancy to 7 days after birth) fetal death,
  • disability,
  • cerebral palsy is a symptom complex of movement disorders, accompanied by a change in muscle tone (usually a rise in tone),
  • hearing loss (hearing loss),
  • blindness,
  • delayed psychomotor development,
  • reactive hepatitis (inflammation of the liver) on the background of stagnation of bile,
  • psychovegetative syndrome - a mental disorder (anxiety, depression) against the background of this disease.


Red blood cells of the fetus may differ in their properties from the mother's erythrocytes.

  • If such red blood cells penetrate the placenta (the main organ that communicates with the mother and the fetus), they become foreign agents (antigens) and antibodies are produced in response to them (blood proteins, which are produced by the introduction of other substances into the body, including bacteria, viruses, toxins).
  • The penetration of these antibodies into the fetus can cause:
    • hemolysis (erythrocyte breakdown),
    • anemia (decrease in hemoglobin (blood dye that carries oxygen) and red blood cells per unit volume of blood),
    • extremely dangerous jaundice (yellow staining of body tissues due to excessive accumulation of bilirubin in the blood (bile pigment) and its metabolic products).

System rhesus factor and rhesus conflict

The antigens of the Rhesus factor system include D-C-c-E-e-antigens. The most important of these is the D-antigen, better known as the Rh factor (Rh).

  • In 85% of people, red blood cells carry the D-antigen - Rh positive (Rh +).
  • 15% of the population does not have a D-antigen on erythrocytes - Rhesus is negative (Rh-).
The reason hemolytic disease of the newborn in the Rhesus factor system is Rh-conflict: the incompatibility of the mother’s blood Rh (-) with the blood of the fetus Rh (+).

In the case of pregnancy of the Rh-negative mother from the Rh-positive father, there is a 50% probability of conceiving a Rh-positive baby. The Rh-positive erythrocytes of the fetus, entering the blood of the Rh-negative mother, are taken by the maternal immune system as foreign and cause the production of anti-Rh antibodies. If the pregnancy is first, then the amount (titer) of these antibodies is small and the Rhesus-conflict does not occur. But the mother’s immune system is already informed (sensitized) and when she meets again with the Rh antigen (Rh-positive fetus erythrocyte) she will respond with massive production of anti-Rh antibodies that will penetrate the fetus’s blood, will contact the fetus erythrocytes and destroy it as carrying the erythrocyte.

The destruction of fetal red blood cells by the mother’s antibodies with the release of hemoglobin is called hemolysis. The clinical manifestations of this pathological process are called hemolytic disease of the newborn.

Forms of hemolytic disease of the newborn

1. Fetal death.
Death occurs due to severe hemolytic anemia and edema of the fetal tissues (fetal dropsy).

2. Edematous.
Cytotoxins damage the tissues of the fetus, provoke general congenital edema of the fetus. As a result, the stillbirth or death of the child in the first hours after birth.

  • - skin is very pale, with jaundiced tint,
  • — отмечается отёк кожи, подкожной клетчатки и внутренних органов,
  • — резко выражена анемия,
  • — анемия и отёки вызывают сердечно-сосудистую недостаточность, от которой и погибает ребёнок.

3. Анемическая.
Частота: 10-20%.
Congenital anemia is most pronounced by the end of the first and the beginning of the second week of life.

  • - sharp pallor of baby’s skin (“marble pallor”, “whiteness of lily”,
  • - yellowness is insignificant or absent,
  • - the liver and spleen are enlarged,
  • - systolic heart murmurs,
  • - fever,
  • - in the blood: increasing anemia, reticulocytosis (up to 50% and above), ESR increased, leukocytosis, and sometimes thrombocytopenia (Evans syndrome),
  • - Coombs test (detects antibodies on red blood cells): positive (80-90%).

4. Jaundiced.
The frequency is 90%.
Jaundice is due to the high production of toxic unconjugated (indirect) bilirubin - a breakdown product of hemoglobin from destroyed red blood cells.
With significant hemolysis and high serum bilirubin concentration, there is a high risk of damage to the central nervous system due to bilirubin deposition in brain cells (nuclear jaundice) with the development of bilirubin encephalopathy.

With congenital jaundice:

  • - yellowness of the skin is observed already at birth,
  • - moderate enlargement of the liver and spleen.

For postnatal jaundice:

  • - characteristic skin color, sclera appears on the first-second day after birth and on the fourth day reaches a maximum,
  • - in the urine: urobilinogen (a product of bilirubin decomposition) is elevated, the urine is light,
  • - feces: dark due to the high content of bilirubin and stercobilin in it.
  • - in the blood: bilirubin increased due to the unconjugated fraction, clinical signs of hemolytic anemia.

Without effective treatment and growth of bilirubin, by the end of the first week of life, the child’s condition worsens, and neurological signs of nuclear jaundice appear:

  • - baby sluggish,
  • - bad breastfeeding, belching,
  • - there is shortness of breath, an abnormal heart rhythm, attacks of frequent pathological yawning.

  • - anxiety, hypertonia,
  • - throwing back the head,
  • - protrusion of fontanel,
  • - convulsions
  • - oculomotor disorders: eyes wide open, eyeballs float,
  • - hyperthermia.

At 3-4 weeks of life, a period of imaginary well-being and smoothing of neurological symptoms can occur. Later residual effects of nuclear jaundice appear:

  • - cerebral palsy,
  • - deafness
  • - paresis,
  • - delayed speech and psychomotor development.

The severity of hemolytic disease of the newborn (HDN)

  • - the number of anti-rhesus antibodies that penetrated the baby’s blood,
  • - permeability of the placenta,
  • - the duration of the action of antibodies on the fetus,
  • - the reaction of the fetus itself to the damaging effects of antibodies.

Factors that aggravate the risk of developing hemolytic disease of the newborn

1. Abortions (miscarriages), abdominal injuries during pregnancy, Rh-negative mother.
2. The second and subsequent pregnancies, the birth of the Rh-positive fetus Rh-negative mother.
3. Blood transfusion in the history of the mother, as well as transplantation of tissues (organs).
4. Cases of HDN in previous children.

What is the AB0 system

Group affiliation of human blood depends on the inheritance of two erythrocyte antigens - A and B.

0 (i) blood type - FIRST

Erythrocyte 0 (I) blood group does not carry antigens A and B on its surface. But in the serum of the first blood group there are A and B antibodies from birth.

A (II) blood type - SECOND

Erythrocyte A (II) blood group carries the A-antigen, in the plasma from birth there are B-antibodies.

B (III) blood type - THIRD

Erythrocyte B (III) blood group carries the B-antigen, in the plasma from birth there are A-antibodies.

AB (IV) blood type - FOURTH

Erythrocyte AB (IV) blood group carries the A and B antigen. Antibodies in the blood plasma is not.

Hemolytic disease of newborns in the AB0 system occurs when the blood of the mother and the child is incompatible in groups. More often:
Mother - blood type 0 (I)
The fetus is blood group A (II) or B (III).

The prevalence of conflict in the system AB0: 5-6 out of 1000 newborns.

A- and B-antibodies do not penetrate the placental barrier and, during the normal course of pregnancy, do not come into contact with the erythrocytes of the fetus.

If, nevertheless, such penetration has occurred, then the hemolytic disease in a newborn child proceeds much more easily than in the case of Rhesus conflict and has no fatal consequences.

Hemolytic disease of the newborn and other erythrocyte antigens.

There are other erythrocyte antigens that can cause HDN.

- From the Kell antigen group: K-antigen.
- From the group of Duffy antigens: Fy-antigens.
- From the Kidd antigen group: Jk antigens.

Symptoms of hemolytic disease of the newborn with incompatibility with these systems are, as a rule, even less severe than with a conflict over the AB0 system.

Modern technologies allow the identification of typical and atypical antibodies in the mother's plasma, which can provoke hemolytic disease in newborns, already in early pregnancy.

general information

Erythrocytes are red cells that are formed elements of human blood. They perform a very important function: they deliver oxygen from the lungs to the tissues and reverse the transport of carbon dioxide.

On the surface of erythrocytes, there are agglutinogens (protein antigens) of two types A and B, and the blood plasma contains antibodies to them - agglutinins α and ß - anti-A and anti-B, respectively. Various combinations of these elements serve as the basis for identifying four groups of the AB0 system:

  • 0 (I) - there are no both proteins, there are antibodies to them,
  • A (II) - there is protein A and antibodies to B,
  • B (III) - there is protein B and antibodies to A,
  • AB (IV) - there are both proteins and no antibodies.

On the erythrocyte membrane are other antigens. The most significant of these is the antigen D. In its presence, the blood is considered to have a positive Rh factor (Rh +), and in the absence it is considered negative (Rh-).

Blood type according to the AB0 system and the Rh factor is of great importance during pregnancy: the conflict between the blood of the mother and the child leads to agglutination (gluing) and subsequent destruction of the red cells, that is, to the hemolytic disease of the newborn. It is found in 0.6% of children and without adequate therapy leads to serious consequences.

The cause of hemolytic disease of newborns is the conflict between the blood of the child and the mother. It occurs under the following conditions:

  • a woman with a Rh-negative (Rh-) blood develops a Rh-positive (Rh +) fetus,
  • in the future mother, the blood belongs to the 0 (I) group, and in the child - to A (II) or B (III),
  • there is a conflict over other antigens.

In most cases, HDN develops due to Rh-conflict. It is believed that the incompatibility of the AB0 system is more common, but due to the mild course of the pathology, it is not always diagnosed.

Rhesus-conflict provokes hemolytic disease of the fetus (newborn) only under the condition of prior sensitization (increased sensitivity) of the organism of matter. Sensitizing factors:

  • Rh + blood transfusion to a woman with Rh - regardless of the age at which it was performed,
  • previous pregnancies, including those that were interrupted after 5-6 weeks, the risk of developing HDN increases with each subsequent birth, especially if they were complicated by placental abruption and surgical interventions.

In hemolytic disease of the newborn with incompatibility in the blood group, sensitization of the organism occurs in everyday life - with the use of certain products, during vaccination, as a result of infections.

Another factor that increases the risk of pathology is a violation of the barrier functions of the placenta, which occurs as a result of the presence of chronic diseases in a pregnant woman, malnutrition, bad habits, and so on.

The pathogenesis of hemolytic disease of the newborn is related to the fact that the female immune system perceives the elements of the blood (erythrocytes) of the fetus as foreign agents and produces antibodies for their destruction.

In the case of Rh conflict, the Rh-positive red blood cells of the fetus enter the mother’s blood with Rh-. In response, her body produces anti-Rh antibodies. They pass through the placenta, enter the baby’s blood, bind to receptors on the surface of its red blood cells and destroy them. At the same time, the amount of hemoglobin in the fetal blood significantly decreases and the level of unconjugated (indirect) bilirubin increases. So develop anemia and hyperbilirubinemia (hemolytic jaundice of newborns).

Indirect bilirubin is a bile pigment that has a toxic effect on all organs - the kidneys, liver, lungs, heart, and so on. At high concentrations, it is able to penetrate the barrier between the circulatory and nervous systems and damage brain cells, causing bilirubin encephalopathy (nuclear jaundice). The risk of brain damage in hemolytic disease of the newborn increases in the case of:

  • reduction of albumin - a protein that has the ability to bind and neutralize bilirubin in the blood,
  • hypoglycemia - glucose deficiency,
  • hypoxia - lack of oxygen,
  • acidosis - increasing the acidity of the blood.

Indirect bilirubin damages liver cells. As a result, the concentration of conjugated (direct, neutralized) bilirubin increases in the blood. Insufficient development of the bile duct in a child leads to its poor elimination, cholestasis (stagnation of bile) and hepatitis.

Due to severe anemia in hemolytic disease of the newborn, there may be foci of extramedullary (extra-cerebral) hematopoiesis in the spleen and liver. As a result, these organs increase, and erythroblasts appear in the blood - immature erythrocytes.

Products of hemolysis of red blood cells accumulate in the tissues of organs, metabolic processes are disturbed, and there is a shortage of many minerals - copper, cobalt, zinc, iron and others.

The pathogenesis of HDN with blood group incompatibility is characterized by a similar mechanism. The difference is that proteins A and B mature later than D. Therefore, the conflict is a danger to the baby near the end of pregnancy. In premature babies, red blood cell breakdown does not occur.

Hemolytic disease of newborns occurs in one of three forms:

  • icteric - 88% of cases
  • anemic - 10%,
  • edematous - 2%.

Symptoms of icteric form:

  • jaundice - discoloration of the skin and mucous membranes as a result of accumulation of bilirubin pigment,
  • decrease in hemoglobin (anemia),
  • enlarged spleen and liver (hepatosplenomegaly),
  • lethargy, decreased reflexes and muscle tone.

In the event of a conflict on rhesus, jaundice occurs immediately after birth, and according to the AB0 system, for 2-3 days. The skin tone gradually changes from orange to pale lemon.

If the indicator of indirect bilirubin in the blood exceeds 300 µmol / l, nuclear hemolytic jaundice in newborns may develop for 3-4 days, which is accompanied by damage to the subcortical nuclei of the brain. Nuclear jaundice is characterized by four stages:

  • Intoxication. It is characterized by loss of appetite, monotonous cry, motor weakness, vomiting.
  • The defeat of the nuclei. Symptoms - tension of the occipital muscles, sharp cry, swelling of the fontanel, tremor, opisthotonus (posture with arching back), the disappearance of some reflexes, bradycardia.
  • Imaginary well-being (improvement of the clinical picture).
  • Complications of hemolytic disease of the newborn. Appear at the end of 1 - the beginning of 5 months of life. Among them are paralysis, paresis, deafness, cerebral palsy, developmental delay, and so on.

At 7-8 days of hemolytic jaundice in newborns may appear signs of cholestasis:

  • stool discoloration,
  • greenish dirty skin tone
  • dark urine
  • increase in blood levels of direct bilirubin.

In anemic form, the clinical manifestations of hemolytic disease of the newborn include:

  • anemia,
  • pallor,
  • hepatosplenomegaly,
  • slight increase or normal bilirubin level.

Anemic form is characterized by the most mild course - the general well-being of the child almost does not suffer.

The edematous variant (intrauterine dropsy) is the most severe form of HDN. Signs:

  • pallor and severe swelling of the skin,
  • big belly,
  • a marked increase in the liver and spleen,
  • muscle flaccidity
  • muffling heart tones,
  • respiratory disorders
  • severe anemia.

Edematous hemolytic disease of the newborn leads to miscarriage, stillbirth and death of children.

Treatment of the disease

In children after hemolytic jaundice, abnormalities in the neuropsychiatric status can be observed. In severe forms, it is cerebral childhood paralysis, accompanied by a change in muscle tone, involuntary movements that have the character of athetosis. Speech and hearing may be impaired. In milder forms of damage to the central nervous system, strabismus, a symptom of the "setting sun", a moderate delay in the development of static functions and the psyche, and a decrease in hearing are observed. In addition, in the group of children who have had HBN, the overall incidence is higher, more often inadequate responses to prophylactic vaccinations and manifestations of exudative-catarrhal and lympho-hypoplastic diathesis.

In the case of a congenital edematous form, it is necessary to immediately press the umbilical cord and urgently (within an hour) to start a replaced blood transfusion or red blood cell mass, if the children are not jaundiced.

  1. Currently, the most widely used, effective and practically safe method of conservative treatment of hyperbilirubinemia is phototherapy.
  2. Of the other methods by which treatment of hemolytic disease of the newborn is carried out, drugs that adsorb indirect bilirubin (NB) in the intestine are shown. It is believed that cleansing enema in the first 2 hours of life reliably reduces the severity of the maximum rise in blood NB level. After 12 hours of life, this treatment has no effect.
  3. Inside appoint in 3 receptions "Cholestyramine" (1.5 g / kg per day) and agar-agar (0.3 g / kg per day), and only on the first day. This reduces the likelihood of developing high bilirubinemia, increases the effectiveness of phototherapy.
  4. "Phenobarbital", "Zixorin", appointed after birth for treatment, contribute to the activation of bilirubin formation and reduce the flow of bile.
  5. Immunoglobulins are injected intravenously in high doses, this reduces the need for replacement transfusions and reduces the duration of phototherapy and hospitalization, the need for blood transfusions in late anemia after suffering from HDN.

Replacement blood transfusion in newborns

In severe cases, surgical treatment of hemolytic disease - replacement transfusion, hemosorption, plasma exchange. Replacement transfusion allows you to remove unconjugated bilirubin and AT from the blood of the child and compensate for the deficiency of red blood cells. For interchangeable transfusions, Rhnegative blood of the same group as the baby’s blood is usually used. At the present time, Rh is not transfused, but Rh is a negative red cell mass mixed with fresh frozen plasma. If HDN is caused by group incompatibility, then the erythrocyte mass of the 0 (1) group is used, and the plasma is either AB (IV) group or one-group.

The absolute indications for replacement transfusion on the first day of life in full-term newborns are as follows:

  • The concentration of unconjugated bilirubin in cord blood is more than 60 μmol / l.
  • The hourly increase in the concentration of unconjugated bilirubin is more than 6–10 µmol / l.
  • The concentration of unconjugated bilirubin in peripheral blood is more than 340 µmol / l.
  • Severe anemia (Hb less than 100 g / l).

Often (if the hourly increase in bilirubin concentration exceeds 610 µmol / l) during the period of treatment of the baby, the replacement transfusion must be repeated (12 hours after the first procedure). From the 3rd day of life of a child with hemolytic disease, only the absolute value of the concentration of bilirubin is important, since hourly growth slows down due to the destruction of AT. When interpreting the results of laboratory tests in each case, they must be compared with clinical data. Deep prematurity, the pathological course of pregnancy, the coincidence of mother and child groups in Rh conflict are risk factors for the development of bilirubin encephalopathy. In this case, it is necessary to carry out a replacement transfusion at a lower concentration of unconjugated bilirubin.

Conservative treatment of hemolytic disease of the newborn

After surgical treatment or with a milder course of the disease, conservative methods of reducing hyperbilirubinemia are used: infusion of protein preparations, glucose, which significantly reduces the likelihood of unconjugated bilirubin passing through BBB. For severe forms, prednisone can be administered intravenously for 4–7 days. Phototherapy is widely used to cure hemolytic disease. After the introduction of this method, the need for a replacement blood transfusion decreased by 40%.

Currently, instead of special lamps located above the child’s bed, for treatment they use a system consisting of a light source in the form of a high-intensity tungsten halogen lamp with a built-in reflector and a photo-optical cable that passes light from the lamp to the fiber-optic cushion. The latter is a mattress with optical fiber woven into it. A protective coating is put on the pillow that is in direct contact with the skin of the newborn. Coating reduces the risk of contamination and the spread of infection. The pillow is attached to the body of the child with a special vest. In phototherapy, photo-oxidation of unconjugated bilirubin in the skin occurs, with the formation of biliverdin and other water-soluble isomers, excreted in the urine and feces.

Для лечения гемолитической болезни применяют также индукторы микросомальных ферментов печени (например, фенобарбитал). Введение витаминов Е, В1, В2, AT6, C, cocarboxylase improves liver function and stabilizes metabolic processes. In order to inhibit the absorption of bilirubin in the intestine, cleansing enemas and activated carbon are prescribed in the first 12 hours of life. The syndrome of thickening bile arrest the appointment of inside choleretic funds [magnesium sulfate, allohol, drotaverina (for example, but-shpy)]. In severe anemia, red blood cell transfusions or washed red blood cells are produced.

Previously it was believed that the treatment of hemolytic disease of the newborn will be as effective as possible if the baby is applied to the breast in 1–2 weeks. after birth, since in the milk there are protivozarusnye AT. At present, it has been proven that early attachment to the breast does not lead to a more severe course of the disease, since ATs contained in milk are destroyed by hydrochloric acid and GIT enzymes and do not have a hemolytic effect.

What is hemolytic disease and how is it dangerous?

Hemolytic disease is a rather serious condition of a newborn, in which a massive erythrocyte breakdown in the baby’s blood is called hemolysis. Scientists explain this phenomenon by the difference in the composition of the blood of the mother and child.

There are several varieties of this disease, but the most dangerous condition is caused by incompatibility of blood by the Rh factor. This problem almost in one hundred percent of cases occurs in women with negative rhesus. If the illness develops due to the difference in the blood types of the mother and the baby (according to the AB0 system), its course is less complicated.

More often, hemolytic disease of newborns is manifested by a change in skin color - it becomes yellow. However, this is only one of the symptoms of the disease. The most dangerous manifestations can be determined only by the results of laboratory tests, ultrasound, Doppler, reflex disorders.

A mild form of the disease can pass without consequences, but it also requires the intervention of a specialist. If hemolytic jaundice of newborns in moderate and severe form is not treated, the child may die. Today, there is a fully developed mechanism for the prevention and treatment of this dangerous condition, and therefore the disease scenario is in most cases favorable.

The causes of the disease in newborns

Why does pathology arise? Consider its main reasons. All people have a specific blood group. There are four of them - 0, A, B and AB (in the domestic medicine the notation I, II, III, IV is used). The group is assigned on the basis of the blood composition in which the antigens are present.

In addition to antigens, in the blood of most of the Caucasoid population of the planet (about 85%) there are special red blood cell proteins (antigens D) that determine the Rh factor. If a patient does not find this protein, his blood belongs to the Rh-negative group.

The composition of blood in a newborn may differ from the parent (according to genetic probability). If the mother and the fetus have a different group or Rh factor, there are prerequisites for an immunological conflict.

What is this contradiction? A woman's body perceives the blood cells of the fetus as alien to him and begins to fight with them, producing antibodies. These particles enter the bloodstream of the baby, penetrating through the placenta.

The described process can begin as early as the 8th week of pregnancy, when the Rh factor and blood type are formed in the fetus. However, more often, massive penetration of antigens through the placenta occurs at the time of delivery. As a result of this, the process of erythrocyte breakdown in the baby begins in the blood - hemolysis.

Such a breakdown of the red blood cells causes the bile pigment, bilirubin, which causes damage to vital organs - the liver, spleen, and bone marrow - to accumulate in the children's body tissues. This component of bile is especially dangerous in that it can penetrate the hematoencephalic barrier and disrupt the functioning of the brain.

In addition, hemolysis significantly reduces the level of hemoglobin in the blood, and the baby has anemia. Anemia is a rather dangerous condition for the newborn, as it contributes to oxygen starvation of tissues and organs.

Blood mismatch in the ABO system (that is, in the group) usually does not lead to serious consequences. However, if during pregnancy a woman had acute respiratory viral infections, influenza or other infectious diseases, this increases the permeability of the placenta, which leads to the development of dangerous forms of the disease.

According to statistics, the disease often occurs in babies who do not match the Rh factor with maternal blood. However, some experts believe that the immunological conflict in the ABO system is not a rare phenomenon, only its symptoms can be blurred and the diagnosis is often not made.

Classification and symptoms of hemolytic disease of the newborn

As we mentioned, hemolytic disease has several varieties. More precisely, there are four of them.

Icteric hemolytic disease

Consider these types in more detail:

  1. The icteric form of hemolytic disease is particularly common in newborns. This is an average type of disease. It is characterized by the appearance of initial symptoms only on the following day after birth. A child is born with normal skin color and no visible pathologies. Then the skin of the baby acquire a yellowish tint, which gradually becomes brighter. The child may be depressed reflexes, increases the liver, spleen.
  2. Nuclear jaundice or bilirubin encephalopathy is a dangerous brain intoxication. The disease occurs when late treatment of the icteric type of the disease. Nuclear jaundice takes place in two stages. The starting phase is characterized by a relaxed posture of the infant, weak reactions to stimuli. The skin becomes bluish, there are cramps, the eyes of the baby are wide open (we recommend reading: why do cramps occur in children?). The next stage is spastic. The child is screaming, his muscles are tense, his breathing is straining. This disease can lead to cerebral palsy, deafness, speech disorders.
  3. Anemic form - the most harmless. In this condition, the child has hemoglobin in the blood, the baby is sluggish, weak, and sucks the breast poorly. This type of illness is noted in every 10 sick newborns and has a favorable scenario.
  4. Edematous species is the most dangerous case of the disease. A child is born with characteristic edema in all cavities of the body - the heart bag, the pleural region, the abdominal cavity. The skin has a yellow tint, with a pronounced pallor. The liver and spleen are enlarged, the blood test shows deep anemia (we recommend reading: the reasons why the spleen is enlarged in children). Such symptoms can cause the development of heart failure and can be fatal in the womb or immediately after birth.

Diagnostic methods

Diagnosis of the disease is carried out at the stage of fetal development and after birth. Consider the methods of natal and postnatal diagnosis.

If the mother has Rh negative blood, even during the pregnancy, the doctor collects data to make a picture of possible pathologies. Considered a lot of information: the incompatibility of the blood of parents, abortions, miscarriages, previous births of the mother.

At least three times during a woman’s pregnancy, a titre of antimicrobial antibodies is detected. Anxiety symptoms — discontinuous values, their stable growth, and also a decrease in the level shortly before delivery — may indicate the penetration of antibodies through the placenta.

If there is a risk of an immune conflict, the doctor prescribes a study of amniotic fluid (determined by bilirubin, the level of protein, iron, glucose, etc.). Be sure to take into account the results of ultrasound and Doppler - placental thickening, polyhydramnios, blood flow velocity in the cerebral artery, etc.

If a pregnant woman has Rh blood negative, most likely doctors will insist on the analysis of amniotic fluid

Postnatal diagnosis is based on examination of the infant after birth. These are the presence of jaundice, the control of bilirubin in dynamics, erythroblastosis, the level of hemoglobin in the blood, etc. All indications are considered in combination. Immunological conflict in the ABO system, despite the favorable prognosis, also requires the attention of the doctor.

Differential diagnosis is carried out with conditions such as hereditary hemolytic jaundice, sepsis, hemorrhages, which can cause anemia. Cytamegalovirus infections and toxoplasmosis are also eliminated.

Newborn jaundice may be exclusively physiological. Its occurrence is due to insufficient maturity of liver enzymes and replacement of hemoglobin cells. As soon as the enzymes begin to be produced in the right amount, the baby’s skin color becomes normal. The described condition does not require treatment.

Features of treatment

Treatment of icteric and other types of hemolytic disease is based on the severity of its course. It is important to quickly rid the body of toxins - erythrocyte breakdown products. Bilirubin reduction therapy is also required to stop the process of hemolysis (see also: a table with the norm of bilirubin in newborns by day).

In difficult cases, blood transfusion is shown, which is done to the fetus in utero or after birth. Other ways to eliminate symptoms are hemosorption (passing blood through special filters) and plasmapheresis (removing plasma containing toxins from a limited blood volume). However, the replacement blood transfusion and other interventions have clear indications:

  • if indirect bilirubin in cord blood exceeds 60 µmol / l or grows at a rate of more than 10 similar units per hour,
  • hemoglobin level in a baby is critical - less than 100 g / l,
  • jaundice appeared immediately after birth or in the first 12 hours.

It should be remembered that blood transfusions often carry complications, most of which are associated with a violation of the procedure technique. Only fresh blood stored for not more than 2 days and low transfusion rate is used. In addition, it is important that the erythrocyte mass is close to body temperature in order to avoid cardiac arrest.

Conservative treatment is indicated for babies with milder symptoms. Typically, this is:

  • in / in the introduction of glucose, protein,
  • use of liver enzyme activators,
  • the appointment of absorbents that help to bind and excrete toxins,
  • the use of vitamins and drugs that stimulate the liver and accelerate metabolic processes in the body of the baby.

All children with signs of yellowing skin phototherapy prescribed. This procedure involves exposing the skin to the crumbs of fluorescent light (white or blue). Such activities excrete indirect bilirubin from the body, transforming it into water-soluble substances.

Also, phototherapy is carried out in order to prevent the occurrence of hyperbilirubinemia, if fetal hypoxia has occurred, and thermoregulation disorders. Often the procedure is prescribed to premature babies.

To begin lactation with hemolytic disease is possible only after the permission of the attending physician. As a rule, attachment to the breast is carried out only three weeks after birth. During this period, antibodies are completely removed from mother's milk, but for now the baby is fed with a mixture or donor milk.

With HDN, it is not possible to immediately apply a baby to the breast, as a rule, lactation can be started at 3-4 weeks after birth

Vaccinations, which are usually done at the hospital, with jaundice can be postponed. In particular, BCG is done a little later.

Consequences of hemolytic disease for a child

The consequences of hemolytic disease of the newborn can be absent altogether, and can be quite significant. It all depends on the form of the disease, as well as on the timeliness and adequacy of therapy. When diagnosing a mild form of hemolytic disease, by the end of the second week, all indicators of the child’s health return to normal. Subsequently, the baby will grow well and develop according to his age.

If the level of bilirubin exceeds the critical value of 340 µmol / l, in the future there may be negative consequences. The development of the nuclear form of the disease is considered short-term, when the baby’s brain suffers from toxins. This form can lead to various mental disorders, the development of cerebral palsy, and hearing loss.

The development of the nuclear form can be judged by the rigidity of the muscles on the back of the head, protrusion of the spring, muscle twitching, signs of suffocation. There are also other symptoms of this condition that are known to neonatologists.

Babies who have undergone severe HDN are registered by a neurologist, ophthalmologist and pediatrician

High rates of bilirubin may affect later. According to statistics, every third child with similar symptoms is diagnosed neuropsychic disorders. In this regard, kids with a severe form of hemolytic disease after stabilization of the condition are registered by a neurologist, ophthalmologist and pediatrician.

Some of the children need a long rehabilitation period, otherwise a couple of months are enough for final recovery. However, the observation of specialists is shown by both.

Preventive actions

Preventive measures include special control of the state of a pregnant woman at risk.

The following events are held:

  1. History taking - pre-pregnancy blood transfusions, miscarriages, stillborn children, abortions. This information will help assess the likely level of antigens in the patient's blood. The most vulnerable of them are those who have given birth or with a history of abortions, since in these cases the body is already ready to "fight back" and the likelihood of immune conflict is high.
  2. In critical cases, the doctor recommends injecting anti-Rh immunoglobulin to suppress the production of antibodies. Such an injection will be the prevention of problems with the new pregnancy.
  3. Systematic monitoring of the blood of a pregnant woman for the presence of Rh antibodies. If their concentration increases, the patient is sent for preventive treatment.
  4. Often, the doctor prescribes the stimulation of labor after 36 weeks of pregnancy. Premature delivery causes a high risk of hemolytic disease of the newborn, since it is in the last month of the infant that the permeability of the placenta increases and the exchange of blood cells of the mother and the baby is activated.

Hemolytic disease of the newborn: causes

The causes of hemolytic disease of the newborn can be immunological conflicts that develop in the presence of:

  • incompatibility of maternal and fetal blood by the Rh factor. This usually happens if the mother has Rh-negative blood and the fetus inherits the papin positive Rh factor. This condition in medicine is called Rhesus conflict. It usually occurs during repeated pregnancies, or if a woman has previously been transfused with blood or red blood cells with a non-Rh factor. The accumulation of antibodies goes from pregnancy to pregnancy (even if it was an abortion or miscarriage), and the risks increase if not provided with adequate therapy.
  • the incompatibility of the blood of the mother and the fetus according to the blood group, if the mother has the first blood group, and the fetus has any other. It is not as difficult as with Rh incompatibility, it can occur during the first pregnancy. Special antibodies of the mother, directed to the erythrocytes of the fetus, penetrate into the child’s blood.
  • rare incompatibility variants for different antigens on the surface of the red blood cells of the fetus, not associated with blood group or Rh factor.

How will manifest?

During pregnancy, the special manifestations do not occur, symptoms may appear, similar to gestosis. But for the fetus hemolytic disease of the newborn can manifest itself in variants:

  • intrauterine death after 20 weeks or more due to the attack of my mother's antibodies on fetal tissue
  • anemic form
  • icteric form
  • edematous form of the disease.

Hemolytic disease of newborns in the blood group does not fundamentally differ in clinical course from that in the Rhesus factor, but the manifestations will not be so bright and heavy, and the forecasts will be more positive.

In an edematous form of the disease, the liver and spleen, all glands and the heart are sharply enlarged, the amount of protein in the blood is reduced, the skin and subcutaneous tissue swell strongly, in the abdomen and chest, the heart also accumulates fluid, which increases more times.

It is the edematous form that is most difficult due to severe disruption of all organs and systems, severe tissue hypoxia due to very low red blood cell count and hemoglobin, damage to the nervous system, which often leads to the death of the baby soon after birth.

The icteric variant is easier, the baby is born on time and with normal weight, the skin is also of normal color, but after a few hours the baby begins to turn yellow suddenly, and congenital jaundice may be more rare.

Spleen and liver, heart and lymph nodes increase, the level of toxic bilirubin in the blood increases dramatically.

This level of bilirubin damages the cells of the liver and heart, kidney, but it is especially dangerous for the brain, manifesting itself as encephalopathy and nuclear jaundice.

Мозг буквально пропитывается билирубином и прокрашивается в желтый цвет.

Дети вялые и плохо кушают, сильно срыгивают и не набирает вес, у них снижены рефлексы, возможны судороги и рвоты.

The level of bilirubin above 340 micromol will be critical, if the child is not full term, the level of bilirubin damaging the brain will be even lower for him.

The consequences of this condition can be dangerous - from the death of a baby to severe neurological disorders and developmental delays in the future.

The anemic form is the easiest, with a child’s pallor, enlarged liver and spleen and poor sucking, weight gain and physical and mental retardation.

It is worth remembering that the more in the mother’s blood there are antibodies to the red blood cells of the fetus, the more serious the prognosis for the child will be. The diagnosis is sometimes made even at the stage of pregnancy, and they prepare in advance for helping the baby immediately after birth.