Hemolytic disease of newborns. Exchange blood transfusion Classification of hemolytic disease of newborns

Hemolytic disease Newborns (HDN) is a very common disease. This pathology is registered in approximately 0.6% of children born. Despite the development various methods treatment, the mortality rate from this disease reaches 2.5%. Unfortunately, a large number of scientifically unsubstantiated “myths” are widespread about this pathology. For a thorough understanding of the processes occurring during hemolytic disease, knowledge of normal and pathological physiology, as well as, of course, obstetrics is necessary.

What is hemolytic disease of the newborn?

TTH is a consequence of a conflict between the immune systems of mother and child. The disease develops due to the incompatibility of the blood of a pregnant woman with antigens on the surface of the fetal red blood cells (primarily this). Simply put, they contain proteins that are recognized by the mother’s body as foreign. That is why in the body of a pregnant woman the processes of activation begin immune system. What's going on? So, in response to the ingress of an unfamiliar protein, the biosynthesis of specific molecules occurs that can contact the antigen and “neutralize” it. These molecules are called antibodies, and the combination of antibody and antigen is called immune complexes.

However, in order to get a little closer to a true understanding of the definition of HDN, it is necessary to understand the human blood system. It has long been known that blood contains different types cells. Largest number cellular composition represented by erythrocytes. At the current level of development of medicine, at least 100 different systems of antigenic proteins present on the erythrocyte membrane are known. The most well studied are the following: rhesus, Kell, Duffy. But, unfortunately, there is a very common misconception that hemolytic disease of the fetus develops only according to group or Rh antigens.

The lack of accumulated knowledge about erythrocyte membrane proteins does not mean that incompatibility with this particular antigen in a pregnant woman is excluded. This is the debunking of the first and, perhaps, the most basic myth about the causes of of this disease.

Factors causing immune conflict:

Video: about the concepts of blood group, Rh factor and Rh conflict

Probability of conflict if the mother is Rh-negative and the father is Rh-positive

Very often, a woman who is Rh negative worries about her future offspring, even without being pregnant. She is afraid of the possibility of developing a Rhesus conflict. Some are even afraid to marry an Rh-positive man.

But is this justified? And what is the likelihood of developing an immunological conflict in such a couple?

Fortunately, the Rh sign is encoded by the so-called allelic genes. What does it mean? The fact is that the information located in the same areas of paired chromosomes can be different:

• The allele of one gene contains a dominant trait, which is the leading one and is manifested in the organism (in our case, the Rh factor is positive, let’s denote it with a capital letter R);
• A recessive trait that does not manifest itself and is suppressed by a dominant trait (in this case, the absence of the Rh antigen, let’s denote it with a small letter r).

What does this information tell us?

The bottom line is that a person who is Rh positive can contain either two dominant traits (RR) or both dominant and recessive (Rr) on their chromosomes.

Moreover, a mother who is Rh negative contains only two recessive traits (rr). As you know, during inheritance, each parent can give only one trait to their child.

Table 1. The probability of inheritance of a Rh-positive trait in a fetus if the father is a carrier of a dominant and recessive trait (Rr)

Table 2. Probability of inheriting a Rh-positive trait in a fetus if the father is a carrier of only dominant traits (RR)

Thus, in 50% of cases, there may not be an immune conflict at all if the father is a carrier of the recessive trait of the Rh factor.

So, we can draw a simple and obvious conclusion: the judgment that an Rh-negative mother and an Rh-positive father must necessarily have immunological incompatibility is fundamentally wrong. This is the “exposure” of the second myth about the causes of the development of hemolytic disease of the fetus.

In addition, even if the child still has a positive Rh factor, this does not mean that the development of tension-type headache is inevitable. Don't forget about the protective properties. During a physiological pregnancy, the placenta practically does not allow antibodies to pass from mother to child. Proof of this is the fact that hemolytic disease occurs only in the fetus of every 20th Rh-negative woman.

Forecast for women with a combination of negative Rh and first blood group

Having learned about the identity of their blood, women with a similar combination of group and Rhesus fall into panic. But how justified are these fears?

At first glance, it may seem that the combination of “two evils” will create a high risk of developing TTH. However, ordinary logic does not work here. It's the other way around: the combination of these factors, oddly enough, improves the prognosis. And there is an explanation for this. In the blood of a woman with the first blood group there are already antibodies that recognize a foreign protein on red blood cells of a different group. This is how nature intended, these antibodies are called agglutinins alpha and beta, all representatives of the first group have them. And when hit large quantity fetal red blood cells into the mother's bloodstream, they are destroyed by existing agglutinins. Thus, antibodies to the Rh factor system simply do not have time to form, because agglutinins are ahead of them.

Women with the first group and negative Rh have a small titer of antibodies against the Rh system, and therefore hemolytic disease develops much less frequently.

Which women are at risk?

Let us not repeat that negative Rh or first blood group is already a certain risk. However, It is important to know about the existence of other predisposing factors:

1. Blood transfusion in an Rh-negative woman during her life

This is especially true for those who have had various allergic reactions. Often in the literature one can find the judgment that those women who received a blood type transfusion without taking into account the Rh factor are at risk. But is this possible in our time? This possibility is practically excluded, since Rhesus status is checked at several stages:

• During blood collection from a donor;
• At the transfusion station;
• The hospital laboratory where blood transfusions are performed;
• A transfusiologist who conducts a three-time compatibility test between the blood of the donor and the recipient (the person receiving the transfusion).

The question arises: Where then is it possible for a woman to become sensitized (presence of hypersensitivity and antibodies) to Rh-positive erythrocytes?

The answer was given quite recently, when scientists found out that there is a group of so-called “dangerous donors” whose blood contains red blood cells with a weakly expressed Rh-positive antigen. It is for this reason that their group is defined by laboratories as Rh negative. However, when such blood is transfused, the recipient’s body may begin to produce specific antibodies in a small volume, but even their quantity is sufficient for the immune system to “remember” this antigen. Therefore, in women with a similar situation, even in the case of their first pregnancy, an immune conflict may arise between her body and the child.

2. Repeated pregnancy

It is believed that in During the first pregnancy, the risk of developing an immune conflict is minimal. And the second and subsequent pregnancies already occur with the formation of antibodies and immunological incompatibility. And indeed it is. But many people forget that the first pregnancy should be considered the fact of the development of the fertilized egg in the mother’s body to any term.

Therefore, women who have had:

1. Spontaneous abortions;
2. Frozen pregnancy;
3. Medical and surgical termination of pregnancy, vacuum aspiration of the fetal egg;
4. Ectopic pregnancy (tubal, ovarian, abdominal).

Moreover, primigravidas with the following pathologies are also at increased risk:

• Chorionic detachment, placenta during this pregnancy;
• Formation of a retroplacental hematoma;
• Bleeding with low placenta previa;
• Women who have had invasive diagnostic methods (needling) amniotic sac with collection of amniotic fluid, taking blood from the fetal umbilical cord, biopsy of a chorion section, examination of a section of the placenta after 16 weeks of pregnancy).

Obviously, the first pregnancy does not always mean the absence of complications and the development of an immune conflict. This fact dispels the myth that only the second and subsequent pregnancies are potentially dangerous.

What is the difference in hemolytic disease of the fetus and newborn?

There are no fundamental differences in these concepts. Simply hemolytic disease in the fetus occurs in the prenatal period. HDN means leakage pathological process after the birth of the child. Thus, the difference lies only in the conditions under which the baby is staying: in utero or after birth.

But there is one more difference in the mechanism of this pathology: during pregnancy, maternal antibodies continue to enter the fetus’s body, which lead to a deterioration in the condition of the fetus, while after childbirth this process stops. That is why women who have given birth to a baby with hemolytic disease are strictly prohibited from feeding their baby breast milk. This is necessary in order to prevent the entry of antibodies into the baby’s body and not to aggravate the course of the disease.

How does the disease progress?

There is a classification that well reflects the main forms of hemolytic disease:

1. Anemic– the main symptom is a decrease in fetus, which is associated with the destruction of red blood cells () in the baby’s body. Such a child has all the signs:

2. Edema form. The predominant symptom is the presence of edema. A distinctive feature is the deposition of excess fluid in all tissues:

• In subcutaneous tissue;
• In the chest and abdominal cavity;
• In the pericardial sac;
• In the placenta (during the prenatal period)
• Hemorrhagic skin rashes are also possible;
• Sometimes there is a dysfunction of blood clotting;
• The child is pale, lethargic, weak.

3. Jaundice form characterized by, which is formed as a result of the destruction of red blood cells. This disease causes toxic damage to all organs and tissues:

• The most severe option is the deposition of bilirubin in the liver and brain of the fetus. This condition is called “kernicterus”;
• A yellowish coloration of the skin and sclera of the eyes is characteristic, which is a consequence of hemolytic jaundice;
• It is the most frequent form(in 90% of cases);
• Possible development diabetes mellitus with damage to the pancreas.

4. Combined (the most severe) - is a combination of all previous symptoms. It is for this reason that this type of hemolytic disease has the highest mortality rate.

How to determine the severity of the disease?

In order to correctly assess the child’s condition, and most importantly, prescribe effective treatment, it is necessary to use reliable criteria when assessing severity.

Diagnostic methods

Already during pregnancy, it is possible to determine not only the presence of this disease, but even the severity.

The most common methods are:

1. Determination of the titer of Rh or group antibodies. It is believed that a titer of 1:2 or 1:4 is not dangerous. But this approach is not justified in all situations. Here lies another myth that “the higher the titer, the worse the prognosis.”

The antibody titer does not always reflect the real severity of the disease. In other words, this indicator is very relative. Therefore, it is necessary to assess the condition of the fetus using several research methods.

2. Ultrasound diagnostics is a very informative method. The most characteristic signs:

• Placenta enlargement;
• The presence of fluid in the tissues: tissue, chest, abdominal cavity, swelling of the soft tissues of the fetal head;
• Increased blood flow speed in uterine arteries, in the vessels of the brain;
• Presence of suspension in amniotic fluid;
• Premature aging of the placenta.

3. Increased density of amniotic fluid.

4. Upon registration - signs and disturbances of heart rhythm.

5. In rare cases, cord blood testing is performed(determine the level of hemoglobin and bilirubin). This method is dangerous due to premature termination of pregnancy and fetal death.

6. After the baby is born, there are more simple methods diagnostics:

• Taking blood to determine: hemoglobin, bilirubin, blood group, Rh factor.
• Examination of the child (in severe cases, jaundice and swelling are evident).
• Determination of antibodies in the child's blood.

Treatment of tension-type headache

Treatment for this disease can begin now. during pregnancy, to prevent deterioration in the condition of the fetus:

1. Introduction of enterosorbents into the mother’s body, for example “Polysorb”. This drugs helps reduce antibody titer.
2. Drip administration of solutions of glucose and vitamin E. These substances strengthen the cell membranes of red blood cells.
3. Injections of hemostatic drugs: “Ditsinon” (“Etamzilat”). They are needed to increase blood clotting ability.
4. In severe cases, intrauterine delivery may be required. However, this procedure is very dangerous and is fraught with adverse consequences: fetal death, premature birth and etc.

Methods of treating a child after childbirth:

For severe disease, the following treatment methods are used:

1. Blood transfusion. It is important to remember that only “fresh” blood is used for blood transfusion, the date of collection of which does not exceed three days. This procedure is dangerous, but it can save the baby's life.
2. Blood purification using hemodialysis and plasmapheresis machines. These methods help remove toxic substances from the blood (bilirubin, antibodies, products of red blood cell destruction).

Prevention of the development of immune conflict during pregnancy

Women at risk for developing immunological incompatibility You must adhere to the following rules, there are only two of them:

• Try not to have an abortion; to do this, you need to consult a gynecologist to prescribe reliable methods of contraception.
• Even if the first pregnancy went well, without complications, then after birth, within 72 hours it is necessary to administer anti-Rhesus immunoglobulin (“KamROU”, “HyperROU”, etc.). The completion of all subsequent pregnancies should be accompanied by the administration of this serum.

Hemolytic disease of the newborn is a serious and very dangerous disease. However, you should not unconditionally believe all the “myths” about this pathology, even though some of them are already firmly entrenched among most people. A competent approach and strict scientific validity are the key to a successful pregnancy. In addition, it is necessary to pay due attention to prevention issues in order to avoid potential problems as much as possible.

HDN– pathological condition, which occurs as a result of incompatibility between the blood of mother and fetus for certain antigens, in which hemolysis of fetal red blood cells occurs under the influence of maternal isoantibodies that penetrate the placental barrier.

Etiology and pathogenesis. The conflict most often develops over the erythrocyte antigens Rh-Hr and AB0.

Causes of isoimmunization:

a) iatrogenic - associated with the introduction of Rh-positive blood into a woman’s body during blood transfusions in the past or during autohemotherapy;

b) fetal-maternal transplacental transfer of fetal red blood cells into the maternal bloodstream during pregnancy and childbirth.

When the antigen first enters the mother’s bloodstream, class M immunoglobulins are produced, the molecules of which are large in size and do not penetrate the placental barrier to the fetus. The entry of an antigen into the mother's bloodstream during repeated pregnancy causes a rapid secondary immune response in the form of the formation of small-sized class G immunoglobulins, which, freely penetrating the placental barrier, are deposited on the membrane of the erythrocytes of the Rh-positive fetus, leading to their hemolysis and accelerated destruction in the organs reticuloendothelial system. Massive destruction of red blood cells leads to the development of anemia in the fetus, the appearance of which causes an increase in the concentration of erythropoietin in the bloodstream of the fetus. Erythropoietin stimulates hematopoiesis, as a result of which foci of extramedullary hematopoiesis appear, mainly in the liver and spleen of the fetus, which increase significantly. Extramedullary hematopoiesis is characterized by incomplete development of red blood cells and the appearance of erythroblasts in the circulation.

Clinical picture. There are anemic, icteric and edematous forms of the disease.

1. Anemic form. Appears from the first hours of life. The main symptoms are pallor skin, low level hemoglobin and red blood cells, petechial rashes, physical inactivity, enlarged liver and spleen. Anemia develops not so much due to hemolysis, but as a result of inhibition of function bone marrow and delays in the release of immature and mature forms of red blood cells.

2. Jaundice form. The most important symptoms are jaundice, anemia, enlarged liver and spleen. In severe cases, symptoms of central nervous system damage are observed. At the birth of a child, attention is often drawn to icteric staining of the amniotic fluid, vernix lubrication, and skin. Anemia is often normochromic or hyperchromic and usually does not reach a pronounced degree. The appearance and intensification of jaundice is caused by an increase in the level of indirect bilirubin in the blood. As jaundice increases, the child’s condition worsens, symptoms appear indicating damage to the central nervous system: convulsive twitching, nystagmus, hypertonicity, etc. Symptoms of “kernicterus” develop (with bilirubin equal to 307.8 - 342.0 µmol/l).

3. Edema form. Pronounced symptoms of the disease are general edema (anasarca, ascites), large liver and spleen, significant anemia, less pronounced jaundice (due to the large amount of fluid in the body), hemodynamic disorders (hypervolemia, increased venous pressure, congestion in the pulmonary circulation, cardiac -pulmonary failure). Often observed hemorrhagic syndrome.

TTH according to the ABO system, as a rule, does not have specific manifestations at the time of birth of the child.

Severity of HDN according to Rh factor:

A) light form(I degree of severity) is characterized by some pallor of the skin, a slight decrease in hemoglobin concentration (up to 150 g/l) and a moderate increase in the level of bilirubin in the umbilical cord blood (up to 85.5 μmol/l), slight pastiness of the subcutaneous fatty tissue;

b) moderate form (II degree of severity) is characterized by pale skin, decreased hemoglobin levels (150 - PO g/l), increased bilirubin content in umbilical cord blood (85.6 - 136.8 µmol/l), pasty subcutaneous tissue, enlarged liver and spleen;

c) severe form ( III degree severity) is characterized by sharp pallor of the skin, a significant decrease in hemoglobin levels (less than 110 g/l), a significant increase in the content of bilirubin in the umbilical cord blood (136.9 µmol/l or more), generalized edema.

The most severe complication of HDN is bilirubin encephalopathy- associated with toxic damage to neurons from indirect bilirubin.

Factors that increase the risk of CNS damage: prematurity, asphyxia, hypothermia, acidosis, hypoproteinemia, malnutrition.

There are 4 phases of the course of bilirubi encephalopathy:

1) bilirubin intoxication - lethargy, hypo-, adynamia, hypo-, areflexia;

2) signs of kernicterus - spasticity, convulsions, “brain scream”, “setting sun” symptom;

3) imaginary well-being - from the 2nd week, a decrease in spasticity and seizures;

4) formation clinical picture neurological complications- children's cerebral paralysis, athetosis, paralysis, paresis, deafness.

Diagnostics.

1. Immunological analysis - identification of Rh-sensitized women.

2. CTG, ultrasound – identifying signs of chronic hypoxia.

3. Study of amniotic fluid.

4. Immediately after the birth of a child from a woman with an Rh-negative blood type, the child’s blood type, hemoglobin and bilirubin levels are determined, and a Coombs test is performed.

Treatment.

1. Nonspecific desensitization of all pregnant women with Rh “-” blood. It is carried out for 10-12 days at a period of 10-12, 22-24, 32-34 weeks. Intravenous administration of 20 ml of 40% glucose with 2 ml of 5% solution is used ascorbic acid, 2 ml of 1% sigetin and 100 mg of cocarboxylase. Rutin, theonicol, calcium gluconate, iron supplements, tocopherol, and antihistamines are prescribed internally.

2. Transplantation of a flap of the husband’s skin to pregnant women.

3. With severe sensitization in complex therapy include prednisolone 5 mg daily from 28 weeks of pregnancy.

4. Pregnant women with a complicated medical history undergo plasmapheresis and hemosorption.

5. Children with a mild form of HDN are born in satisfactory condition and resuscitation measures dont need. Their condition should be monitored dynamically. Such children should begin phototherapy from the first hours of life.

6. Children with a moderate form of HDN according to the Rh factor can be born in a state of asphyxia. After providing the necessary resuscitation measures, they should be transferred to the intensive care unit, where they urgently need to establish an adequate infusion of 10% glucose solution and phototherapy. Treatment is carried out under constant monitoring for the level of Hb, Ht in the peripheral blood, the level of bilirubin (every 6 hours on the 1st day).

If conservative therapy is ineffective, a replacement blood transfusion is necessary.

7. Children with severe forms of hyperventilation require resuscitation measures in the delivery room. After completion of primary resuscitation, it is necessary to stabilize hemodynamics, perform umbilical vein catheterization and perform partial PCO. To correct severe anemia in the edematous-anemic form of HDN, a partial replacement of the child’s blood is immediately carried out with Rh-negative donor red blood cells (without the introduction of plasma) of group 0 (1) at the rate of 60 - 70 ml/kg. At the end of the operation, the child should be given an infusion of fresh frozen plasma at a rate of 10-15 ml-kg and 10% glucose solution, at a constant rate. Depending on the severity of symptoms of respiratory and cardiovascular failure, adequate syndromic therapy should be carried out.

Children with TTH due to A0 and rare blood factors require the same care and treatment as children with TTH due to the Rh factor.

Indications for PCP:

1. Emergency PCP for severe HDN (partial PCP technique is used).

2. Early PCD (in the first 24 hours of life):

a) the level of total bilirubin in umbilical cord blood is above 77.5 µmol/l;

b) umbilical cord blood Hb level is below 110 g/l; Ht below 35%;

c) an hourly increase in bilirubin above 8.5 µmol/l - rapidly progressing anemia (a drop in the Hb blood level below 120 g/l in the first day of life, even with a relatively low increase in bilirubin). In this case, the ZPK technique is used with the replacement of 2 bcc.

3. Later ZPK:

a) an increase in the concentration of total bilirubin in the blood serum of more than 256 µmol/l on the 2nd day of life and more than 340 µmol/l in full-term infants and more than 256 - 340 µmol/l in premature infants on the next day;

b) Clinical signs bilirubin intoxication serves as an indication for performing PCP at any level of bilirubin. In this case, the ZPK technique is used with the replacement of 2 bcc.

PDA operation technique:

1. Before starting the operation, hand treatment is carried out according to generally accepted methods, sterile gowns and gloves are put on.

2. The PCA is carried out through a sterile polyethylene inserted into the umbilical cord vein to a depth equal to the distance from the umbilical ring to the xiphoid process plus 1 cm upward towards the liver.

3. Before starting the procedure, it is necessary to rinse the catheter with a weak solution of heparin: no more than 5-10 units in 1 ml, i.e. 0.1 ml of standard solution (5000 units in 1 ml) should be diluted in 50 - 100 ml of sterile isotonic sodium chloride solution.

4. A catheter filled with isotonic sodium chloride solution or a weak heparin solution is inserted into the umbilical cord vein, since when the child cries, negative pressure may arise in the vein, and air may enter it from an unfilled catheter.

5. The first portions of blood obtained from the catheter are collected in several test tubes: to determine the blood type, bilirubin level and compatibility (no more than 5-10 ml of the child’s blood for all).

6. The child’s blood is slowly removed in fractional portions of 10-20 ml and replaced with the donor’s blood or red blood cells in the same quantity. In the latter case, for every 2 volumes of injected red blood cells, 1 volume of plasma is injected.

7. After every 100 ml of blood is administered, to prevent hypocalcemia, it is necessary to add 2 ml of 10% calcium gluconate solution or 2 ml of 10% calcium chloride solution, diluted with 2 ml of 10% glucose solution.

8. After replacing 2 BCCs, the operation can be completed. The average duration of the operation is 1.5 - 2.5 hours. Faster or slower operation may have a negative impact on the general condition of the child.

9. The operation is completed intravenous administration antibiotic wide range actions. The catheter is removed.

Complications of PCD: heart failure (with rapid administration, volume overload); cardiac arrhythmias (decreased calcium and increased potassium); infection; thromboembolism; perforation of the umbilical vein and intestines; portal hypertension; anaphylactic shock; ARF, hematuria; DIC syndrome (decrease in platelet count, deficiency of procoagulants); excessive heparinization; hypokalemia, acidosis, hyperkalemia, hypocalcemia; graft versus host disease; hypothermia.

Hemolytic disease in newborns (HDN) is a pathology that occurs during fetal development or during the first hours after birth. The cause of this pathology is incompatibility between the blood of the fetus and its mother, when the woman begins to produce antibodies to the child’s red blood cells (immunological conflict). The development of the disease occurs due to the fact that antibodies from female body enter the child's body. In this way, active destruction of the baby’s red blood cells occurs... in addition, HDN is almost the first on the list of reasons that cause kernicterus in babies and anemia.

It is worth noting that in last years Cases of hemolytic disease have increased significantly - approximately one case per 250-300 births. Usually, this pathology occurs due to Rh conflict between woman and child. If we talk about blood group incompatibility, then there are several times fewer such cases. Incompatibility with other erythrocyte antigens is generally considered rare, because such cases are isolated.

If hemolytic disease develops according to the Rh factor, then in 3-6% of cases it proceeds quite mildly, but at the same time it is very difficult to diagnose. There are cases when a hemolytic disease of this type was detected in a newborn already in advanced stage when treatment does not bring tangible results.

When a newborn begins to develop hemolysis or destruction of red blood cells, the level of bilirubin in his blood very quickly increases and provokes the development of anemia. When the level of bilirubin is too high and exceeds a critical level, it begins to release toxins that affect the brain and many other organs of the child. In addition, anemia begins to progress very quickly and the body begins to do everything possible to compensate for the lack of oxygen. Thus, the liver begins to increase in size, followed by the spleen.

Clinical forms of hemolytic anemia in a newborn baby

1. Edematous form of HDN. This form is the most severe and begins to develop in utero. As a result of the destruction of red blood cells, the child develops a severe form of anemia, metabolism is disrupted, tissues swell and protein levels decrease. If HDN begins to develop on early pregnancy, it could end in miscarriage. If the child still survives, he will be born very pale, with pronounced swelling.
2. Jaundice form of HDN. This form can be found most often. The main symptoms are the early development of jaundice, anemia and a significant enlargement of the liver and spleen. Jaundice may appear immediately after birth or after about 1-2 days, which is not typical for physiological jaundice. The earlier it appeared, the more severe the HDN will be. Signs of the disease include greenish skin color, dark urine and colorless feces.
3. Anemic form of HDN. This form is the most gentle and easiest. It appears within seven days after the birth of the child. It is not always possible to immediately notice the appearance of pale skin, and therefore HDN can be diagnosed at 2-3 weeks of the baby’s life. Externally, the child remains the same, but the liver and spleen begin to increase in size. Bilirubin levels will be elevated, but only slightly. This form of the disease can be easily cured without harmful consequences for the baby’s health.

Diagnosis and treatment of hemolytic disease of newborns

Antenatal diagnosis is carried out during pregnancy in women who are at risk. If a woman is Rh negative, she must be tested three times for the presence of antibodies in her blood during pregnancy. It is very important to take into account the results over time, because they can show a high risk of a child’s disease. In order to finally make sure of the diagnosis, you need to examine the amniotic fluid for the presence of levels of bilirubin, iron, glucose and protein. In addition, changes in fetal development, which can be detected by ultrasound, may cause suspicion.

Postnatal diagnosis is carried out after the birth of the child and consists entirely of studying clinical symptoms illnesses in a child. In this case, it is necessary to study absolutely all the data, both in complex and in dynamics.

How to treat hemolytic disease in a newborn baby

Indications for blood transfusion if the baby is premature

• The level of indirect bilirubin exceeds the critical value;
• bilirubin level increases every hour by approximately 6-10 µmol/l;
• a severe form of anemia is observed.

In addition, vitamins B2, B6, C can be used to treat hemolytic disease. Activated carbon, prednisone, cocarboxylase or phenobarbital. It is worth noting that previously it was believed that if a child had a more hemolytic disease, then he should not be put to the breast. Today it has been proven that the antibodies that are in a woman’s milk do not enter the baby’s blood and are completely destroyed under the influence of hydrochloric acid in the baby’s stomach. Therefore, there is no need to be afraid of anything and put your baby to your breast as often as possible. This will help him get stronger faster and begin to fight the disease on his own.

Prevention of hemolytic disease in a newborn baby

Jaundice is a visual manifestation of hyperbilirubinemia. Bilirubin, one of the end products of the catabolism of the heme protoporphyrin ring, accumulates in large quantities in the body and causes a yellow coloration of the skin and mucous membranes. The breakdown of 1 g of hemoglobin produces 34 mg of bilirubin. In adults it appears when the bilirubin level is more than 25 µmol/l, in full-term newborns - 85 µmol/l, and in premature infants - more than 120 µmol/l.

A transient increase in the concentration of bilirubin in the blood in the first 3-4 days after birth is observed in almost all newborns. In approximately half of full-term and most premature infants, this is accompanied by the development of icteric syndrome. An important task of a medical worker during the period of monitoring the health of a newborn child is to distinguish between physiological characteristics and pathological disorders of bilirubin metabolism.

Physiological jaundice

Clinical criteria:

appears 24-36 hours after birth;

increases during the first 3-4 days of life;

begins to fade from the end of the first week of life;

disappears in the second or third week of life;

the general condition of the child is satisfactory;

the size of the liver and spleen is not enlarged;

normal color of stool and urine.

Laboratory criteria:

concentration of bilirubin in umbilical cord blood (moment of birth) -< 51 мкмоль;

the hemoglobin concentration in the blood is normal;

maximum concentration of total bilirubin on days 3-4 in peripheral or venous blood: ≤240 µmol/L in full-term infants and ≤ 150 µmol/L in premature infants;

total blood bilirubin increases due to the indirect fraction;

the relative proportion of the direct fraction is less than 10%.

Pathological hyperbilirubinemia

Present at birth or appear on the first day or second

week of life;

Combined with signs of hemolysis (anemia, high reticulocytosis, nuclear erythroid forms in the blood smear, excess spherocytes), pallor, hepatosplenomegaly;

Lasts more than 1 week. in full-term and 2 weeks. - in premature babies;

They occur in waves (the yellowness of the skin and mucous membranes increases in intensity after a period of its decrease or disappearance);

The rate of increase (increase) of unconjugated bilirubin (NB, indirect bilirubin) is >9 µmol/l/h or 137 µmol/l/day.

The level of NB in ​​umbilical cord blood serum is >60 µmol/L or 85 µmol/L in the first 12 hours of life, 171 µmol/L on the 2nd day of life, the maximum NB values ​​on any day of life exceed 221 µmol/L

The maximum level of bilirubin diglucuronide (BDG, direct biliru-

bin) - >25 µmol/l

Deterioration of the child’s general condition against the background of a progressive increase in jaundice,

Dark urine or discolored stools

Physiological jaundice is a diagnosis excluding pathological jaundice.

There are four main mechanisms for the development of pathological hyperbilirubinemia:

1. Hyperproduction of bilirubin due to hemolysis;

2. Impaired conjugation of bilirubin in hepatocytes;

3. Impaired excretion of bilirubin into the intestines;

4. Combined violation of conjugation and excretion.

In this regard, from a practical point of view, it is advisable to distinguish four types of jaundice:

1) hemolytic;

2) conjugation;

3) mechanical;

4) hepatic.

Hemolytic disease of newborns (HDN) is an isoimmune hemolytic anemia that occurs in cases of incompatibility between the blood of mother and fetus for erythrocyte antigens, while the antigens are localized to the mother and fetus, and antibodies to them are produced in the mother's body. HDN in Russia is diagnosed in approximately 0.6% of all newborns.

Classification GBN provides for the establishment of:

Type of conflict (Rh-, AB0-, other antigenic systems);

Clinical form (intrauterine death of the fetus with maceration, edematous, icteric, anemic);

Degrees of severity for icteric and anemic forms (mild, moderate and severe);

Complications (bilirubin encephalopathy - kernicterus, other neurological disorders; hemorrhagic or edematous syndrome, damage to the liver, heart, kidneys, adrenal glands, "bile thickening" syndrome, metabolic disorders - hypoglycemia, etc.);

Concomitant diseases and underlying conditions (prematurity, intrauterine infections, asphyxia, etc.)

Etiology. A conflict may arise if the mother is antigen-negative and the fetus is antigen-positive. There are 14 known main erythrocyte group systems, combining more than 100 antigens, as well as numerous private ones and common ones with other tissues erythrocyte antigens. HDN usually causes incompatibility of the fetus and mother for Rh or ABO antigens. It has been established that the Rh antigen system consists of 6 main antigens (the synthesis of which is determined by 2 pairs of genes located on the first chromosome), designated either C, c; D,d; Ε, e (Fisher's terminology), or Rh", hr", Rho, hr0, Rh", hr" (Winner's terminology). Rh-positive red blood cells contain D-factor (Rho factor, in Winner's terminology), while so-called Rh-negative red blood cells do not. Incompatibility of ABO antigens, leading to HDN, usually occurs with the mother's blood group 0 (1) and the child's blood group A (II). If HDN develops due to double incompatibility of the child and mother, i.e. the mother is O (I) Rh(-), and the child is A (II) Rh(+) or B (III) Rh (+), then, as a rule, it is caused by A- or B-antigens. Rh-HDN is usually caused by sensitization of the Rh-negative mother to the Rh-O antigen prior to pregnancy. Sensitizing factors are, first of all, previous pregnancies (including ectopic ones and those ending in abortions), and therefore Rhesus HDN, as a rule, develops in children not born from the first pregnancy. In case of ABO conflict, this pattern was not noted, and ABO-THB can occur already during the first pregnancy, but when the barrier functions of the placenta are impaired due to the presence of somatic pathology in the mother, gestosis, leading to intrauterine hypoxia of the fetus.

Pathogenesis.

Previous abortions, miscarriages, ectopic pregnancy, childbirth, etc. predispose the antigen-positive erythrocytes of the fetus to enter the bloodstream of the antigen-negative mother. In this case, the mother’s body produces anti-Rhesus or group antibodies. Incomplete anti-red blood cell antibodies, belonging to class G immunoglobulins, damage the erythrocyte membrane, leading to an increase in its permeability and metabolic disorders in the erythrocyte. These red blood cells, changed under the influence of antibodies, are actively captured by macrophages of the liver, spleen, bone marrow and die prematurely; in severe forms of the disease, hemolysis can also be intravascular. The resulting large amount of NB entering the blood cannot be eliminated by the liver, and hyperbilirubinemia develops. If hemolysis is not too intense with a small amount of incoming maternal antibodies, the liver quite actively removes NB, then in the child the clinical picture of HDN is dominated by anemia with the absence or minimal severity of jaundice. It is believed that if anti-erythrocyte alloimmune antibodies penetrate to the fetus for a long time and actively during pregnancy before the onset of labor, then intrauterine maceration of the fetus or an edematous form of HDN develops. In most cases, the placenta prevents the penetration of alloimmune antibodies to the fetus. At the time of birth, the barrier properties of the placenta are sharply disrupted, and maternal isoantibodies enter the fetus, which, as a rule, causes the absence of jaundice at birth and its appearance in the first hours and days of life. Anti-erythrocyte antibodies can be passed to the baby through mother's milk, which increases the severity of HDN.

Features of pathogenesis in the edematous form of HDN. Hemolysis begins from 18-22 weeks. pregnancy, is intense and leads to severe fetal anemia. As a result, severe fetal hypoxia develops, which causes deep metabolic disorders and damage to the vascular wall, a decrease in albumin synthesis occurs, albumin and water move from the fetal blood to the tissue interstitium, which forms a general edematous syndrome.

Features of pathogenesis in the icteric form of HDN. Hemolysis begins shortly before birth, the level of bilirubin quickly and significantly increases, which leads to its accumulation in the lipid substances of tissues, in particular in the nuclei of the brain, an increase in the load on liver glucuronyltransferase and an increase in the excretion of conjugated (direct) bilirubin, which leads to impaired bile excretion .

Features of the pathogenesis of the anemic form of HDN. The anemic form of HDN develops when small amounts of maternal antibodies enter the fetal bloodstream shortly before birth. At the same time, hemolysis is not intense, and the newborn’s liver quite actively removes bilirubin.

Although hyperbilirubinemia with NB leads to damage to a variety of organs and systems (brain, liver, kidneys, lungs, heart, etc.), damage to the nuclei of the base of the brain is of leading clinical importance. The staining of the basal ganglia, globus pallidus, caudal nuclei, putamen of the lenticular nucleus is maximally expressed; less often the hippocampal gyrus, cerebellar tonsils, some nuclei of the thalamus optic, olives, dentate nucleus, etc. can be changed; this condition, according to the proposal of G. Schmorl (1904), was called “kernicterus”.

Clinical picture.

Edema form- the most severe manifestation of Rh-HDN. Typical is a burdened medical history of the mother - the birth of previous children in a family with HDN, miscarriages, stillbirths, prematurity, transfusions of Rh-incompatible blood, repeated abortions. During ultrasound examination of the fetus, the Buddha pose is characteristic - head up, lower limbs due to the barrel-shaped enlargement of the abdomen, they are bent in knee joints, located unusually far from the body; "halo" around the cranial vault. Due to edema, the weight of the placenta is significantly increased. Normally, the weight of the placenta is 1/6-1/7 of the body weight of the fetus, but in the edematous form this ratio reaches 1:3 and even 1:1. The placental villi are enlarged, but their capillaries are morphologically immature and abnormal. Polyhydroamnion is characteristic. As a rule, mothers suffer from severe gestosis in the form of preeclampsia and eclampsia. Already at birth, the child has: severe pallor (rarely with an icteric tint) and general swelling, especially pronounced on the external genitalia, legs, head, face; a sharply increased barrel-shaped belly; significant hepato- and splenomegaly (a consequence of erythroid metaplasia in organs and severe fibrosis in the liver); expansion of the boundaries of relative cardiac dullness, muffling of heart sounds. Ascites is usually significant even in the absence of general fetal edema. The absence of jaundice at birth is associated with the release of fetal NP through the placenta. Very often, immediately after birth, respiratory disorders develop due to hypoplastic lungs or hyaline membrane disease. The cause of pulmonary hypoplasia is seen in an elevated diaphragm with hepatosplenomegaly and ascites. Hemorrhagic syndrome (hemorrhages in the brain, lungs, gastrointestinal tract) is common in children with the edematous form of tension-type headache. A minority of these children have decompensated DIC syndrome, but all have very low levels of procoagulants in the blood plasma, the synthesis of which is carried out in the liver. Characteristic: hypoproteinemia (serum protein level falls below 40-45 g/l), increased levels of BDG in the umbilical cord blood (and not just NB), severe anemia (hemoglobin concentration less than 100 g/l), normoblastosis and erythroblastosis of varying severity, thrombocytopenia. Anemia in such children can be so severe that, in combination with hypoproteinemia and damage to the vascular wall, it can lead to heart failure. Children with congenital edematous form of HDN who survive after active treatment (about half of these children die in the first days of life) often develop severe neonatal infections, liver cirrhosis, and encephalopathy.

Jaundice form- the most common form of HDN. At birth, the amniotic fluid, umbilical cord membranes, and primordial lubricant may be jaundiced. Characteristic is the early development of jaundice, which is noticed either at birth or within 24-36 hours of a newborn’s life. The earlier the jaundice appeared, the more severe the course of HDN is usually. Jaundice is predominantly warm yellow. The intensity and shade of the jaundiced color gradually change: first an orange tint, then bronze, then lemon and, finally, the color of an unripe lemon. Also characterized by an enlarged liver and spleen, icteric staining of the sclera and mucous membranes, and pasty abdomen is often observed. As the level of BN in the blood increases, children become lethargic, adynamic, suck poorly, their physiological reflexes for newborns decrease, and other signs of bilirubin intoxication appear. Blood tests reveal varying degrees of anemia, pseudoleukocytosis due to an increase in the number of normoblasts and erythroblasts, often thrombocytopenia, less often leukemoid reaction. The number of reticulocytes is also significantly increased (more than 5%).

If treatment is not started in a timely manner or is inadequate, the icteric form of HDN may be complicated by bilirubin encephalopathy and bile thickening syndrome. Bile thickening syndrome is diagnosed when jaundice acquires a greenish tint, the liver increases in size compared to previous examinations, and the intensity of urine color increases.

Bilirubin encephalopathy(BE) is rarely clinically detected in the first 36 hours of life, and usually its first manifestations are diagnosed on the 3-6th day of life. The first signs of EB are manifestations of bilirubin intoxication - lethargy, decreased muscle tone and appetite up to the point of refusal to eat, a monotonous, unemotional cry, rapid depletion of physiological reflexes, regurgitation, vomiting. Then the classic signs of kernicterus appear - spasticity, stiff neck, forced body position with opisthotonus, stiff limbs and clenched hands; periodic excitement and a sharp “cerebral” high-frequency cry, bulging of a large fontanelle, twitching of facial muscles or complete amymia, large-scale tremors of the hands, convulsions; "setting sun" symptom; disappearance of the Moro reflex and the visible reaction to a strong sound, the sucking reflex; nystagmus, Graefe's symptom; respiratory arrest, bradycardia, lethargy. The outcome of BE will be athetosis, choreoathetosis, paralysis, paresis; deafness; cerebral palsy; impaired mental function; dysarthria, etc.

Risk factors for bilirubin encephalopathy are hypoxia, severe asphyxia (especially complicated by severe hypercapnia), prematurity, hypo- or hyperglycemia, acidosis, hemorrhages in the brain and its membranes, convulsions, neuroinfections, hypothermia, fasting, hypoalbuminemia, certain medications (sulfonamides, alcohol, furosemide , diphenin, diazepam, indomethacin and salicylates, methicillin, oxacillin, cephalothin, cefoperazone).

Anemic form diagnosed in 10-20% of patients. Children are pale, somewhat lethargic, suck poorly and gain weight. They have an increase in the size of the liver and spleen, in the peripheral blood - anemia of varying severity in combination with normoblastosis, reticulocytosis, spherocytosis (with ABO conflict). Sometimes hypogenerator anemia is observed, i.e. there is no reticulocytosis and normoblastosis, which is explained by inhibition of bone marrow function and delayed release of immature and mature forms of red blood cells from it. NB levels are usually normal or moderately elevated. Signs of anemia appear at the end of the first or even the second week of life.

Diagnostics.

The studies required to diagnose tension-type headache are presented in Table 3.

Table 3.

Examination of the pregnant woman and fetus in case of suspected

hemolytic disease of the fetus.

All women with Rh-negative blood are tested at least three times for the titer of anti-Rhesus antibodies. The first study is carried out upon registration at the antenatal clinic. It is optimal to further conduct a repeat study at 18-20 weeks, and in the third trimester of pregnancy, carry it out every 4 weeks. The mother's Rh antibody test does not accurately predict the future severity of HDN in the child, and determining the level of bilirubin in the amniotic fluid is of great value. If the titer of Rh antibodies is 1:16-1:32 or greater, then at 6-28 weeks. amniocentesis is performed and the concentration of bilirubin-like substances in the amniotic fluid is determined. If the optical density with a 450 mm filter is more than 0.18, intrauterine blood transfusion is usually necessary. It is not performed on fetuses older than 32 weeks. gestation. Another method for diagnosing the congenital edematous form of HDN is ultrasound examination, which reveals fetal edema. It develops when the hemoglobin level is 70-100 g/l.

Since the prognosis for HDN depends on the content of hemoglobin and the concentration of bilirubin in the blood serum, it is first necessary to determine these indicators to develop further medical tactics, and then conduct an examination to identify the causes of anemia and hyperbilirubinemia.

Examination plan for suspected TTH:

1. Determination of the blood group and Rhesus status of the mother and child.

2. Analysis of the child’s peripheral blood with evaluation of the blood smear.

3. Blood test with reticulocyte count.

4. Dynamic determination of bilirubin concentration in blood serum

see the child.

5. Immunological studies.

Immunological studies. In all children of Rh-negative mothers, the blood type and Rh affiliation, and serum bilirubin level are determined in the umbilical cord blood. In case of Rh incompatibility, the titer of Rh antibodies in the mother’s blood and milk is determined, and a direct Coombs reaction (preferably an aggregate agglutination test according to L.I. Idelson) is performed with the child’s red blood cells and an indirect Coombs reaction with the mother’s blood serum, and the dynamics of Rh is analyzed. antibodies in the mother's blood during pregnancy and the outcome of previous pregnancies. In case of ABO incompatibility, the titer of allohemagglutinins (to the erythrocyte antigen present in the child and absent in the mother) is determined in the blood and milk of the mother, in protein (colloid) and salt media, in order to distinguish natural agglutinins (have a large molecular weight and belong to class M immunoglobulins, do not penetrate the placenta) from immune ones (have a small molecular weight, belong to class G immunoglobulins, easily penetrate the placenta, and after birth - with milk, i.e. are responsible for the development of HDN). In the presence of immune antibodies, the titer of allohemagglutinins in the protein medium is two steps or more (i.e., 4 times or more) higher than in the saline medium. A direct Coombs test for ABO conflict in a child is usually weakly positive, i.e. slight agglutination appears after 4-8 minutes, whereas with Rh-conflict, pronounced agglutination is noticeable after 1 minute. If there is a conflict between the child and the mother regarding other rare erythrocyte antigenic factors (according to various authors, the frequency of such conflict ranges from 2 to 20% of all cases of HDN), the direct Coombs test is usually positive in the child and the indirect test in the mother, and incompatibility of the child’s erythrocytes and mother's serum in an individual compatibility test.

Changes in the child’s peripheral blood: anemia, hyperreticulocytosis, when viewing a blood smear - an excessive number of spherocytes (+++, +++++), pseudoleukocytosis due to an increased amount of nuclear forms of the erythroid series in the blood.

The plan for further laboratory examination of the child includes regular determinations of the level of glycemia (at least 4 times a day in the first 3-4 days of life), NB (at least 2-3 times a day until the level of NB in ​​the blood begins to decrease), plasma hemoglobin (in the first day and further as indicated), platelet count, transaminase activity (at least once) and other studies depending on the characteristics of the clinical picture.

Table 4.

Examinations for suspected HDN.

Diagnostic criteria for tension-type headache:

Clinical criteria:

*Dynamics of jaundice

Appears in the first 24 hours after birth (usually the first 12 hours);

Increases during the first 3-5 days of life;

Begins to fade from the end of the first to the beginning of the second week of life;

Disappears by the end of the third week of life.

*Features of the clinical picture

The skin with an AB0 conflict is usually bright yellow; with an Rh conflict it may have a lemon tint (jaundice on a pale background),

The general condition of the child depends on the severity of hemolysis and the degree of hyperbilirubinemia (from satisfactory to severe)

In the first hours and days of life, as a rule, there is an increase in the size of the liver and spleen;

Usually - normal coloring of stool and urine; against the background of phototherapy, there may be a green coloration of the stool and short-term darkening of the urine.

Laboratory criteria:

Concentration of bilirubin in umbilical cord blood (moment of birth) - in mild forms of immunological conflict for Rh and in all cases of A0 incompatibility -<=51 мкмоль/л; при тяжелых формах иммунологического конфликта по Rh и редким факторам – существенно выше 51 мкмоль/л;

The concentration of hemoglobin in umbilical cord blood in mild cases is at the lower limit of normal, in severe cases it is significantly reduced;

The hourly increase in bilirubin in the first day of life is more than 5.1 µmol/l/hour, in severe cases – more than 8.5 µmol/l/hour;

The maximum concentration of total bilirubin on days 3-4 in peripheral or venous blood: >> 256 µmol/L in full-term infants, >> 171 µmol/L in premature infants;

Total blood bilirubin increases mainly due to the indirect fraction,

The relative proportion of the direct fraction is less than 20%;

decrease in hemoglobin level, red blood cell count and increase in reticulocyte count in clinical blood tests during the 1st week of life.

Based on clinical and laboratory data, three degrees of severity are distinguished:

a) A mild form of hemolytic disease (1st degree of severity) is characterized by some pallor of the skin, a slight decrease in the concentration of hemoglobin in the umbilical cord blood (up to 150 g/l), a moderate increase in bilirubin in the umbilical cord blood (up to 85.5 µmol/l), hourly an increase in bilirubin to 4-5 µmol/l, a moderate enlargement of the liver and spleen of less than 2.5 and 1 cm, respectively, a slight pastiness of the subcutaneous fat.

b) Moderate form (2nd degree of severity) is characterized by pallor of the skin, a decrease in cord blood hemoglobin in the range of 150-110 g/l, an increase in bilirubin in the range of 85.6-136.8 µmol/l, an hourly increase in bilirubin up to 6- 10 µmol/l, pasty subcutaneous fat, enlarged liver by 2.5 - 3.0 cm and spleen by 1.0 - 1.5 cm.

c) Severe form (3rd degree of severity) is characterized by severe pallor of the skin, a significant decrease in hemoglobin (less than 110 g/l), a significant increase in bilirubin in the umbilical cord blood (136.9 μmol/l or more), generalized edema, the presence of symptoms bilirubin damage to the brain of any severity and during all periods of the disease, respiratory and cardiac dysfunction in the absence of data indicating concomitant pneumo- or cardiopathy.

Differential diagnosis of tension-type headache carried out with hereditary hemolytic anemias (spherocytosis, elliptocytosis, stomatocytosis, deficiencies of certain erythrocyte enzymes, anomalies of hemoglobin synthesis), which are characterized by a delayed (after 24 hours of life) appearance of the above clinical and laboratory signs, as well as changes in the shape and size of erythrocytes during a morphological examination of a smear blood, a violation of their osmotic stability in dynamics, a change in the activity of erythrocyte enzymes and the type of hemoglobin.

Examples of diagnosis formulation.

Hemolytic disease due to Rh conflict, edematous-icteric form, severe, complicated by bile thickening syndrome.

Hemolytic disease due to conflict according to the ABO system, icteric form, moderate severity, uncomplicated.

Modern principles of prevention and treatment.

Treatment of hemolytic disease of the fetus is carried out with Rh isoimmunization during the period of fetal development in order to correct anemia in the fetus, prevent massive hemolysis, and maintain pregnancy until the fetus reaches viability. Plasmapheresis and cordocentesis are used with intrauterine transfusion of red blood cells ("washed" red blood cells of blood group 0(II), Rh-negative are used).

Management tactics for tension headaches.

An important condition for the prevention and treatment of hyperbilirubinemia in newborns is the creation of optimal conditions for the early neonatal adaptation of the child. In all cases of illness in a newborn, care must be taken to maintain optimal body temperature, provide his body with a sufficient amount of fluid and nutrients, and prevent metabolic disorders such as hypoglycemia, hypoalbuminemia, hypoxemia and acidosis.

In cases where there are clinical signs of a severe form of hemolytic disease at the time of birth of a child in a woman with Rh-negative blood (severe pallor of the skin, icteric staining of the skin of the abdomen and umbilical cord, swelling of the soft tissues, enlargement of the liver and spleen), emergency PCD surgery is indicated without waiting for laboratory tests. data. (In this case, the partial PCD technique is used, in which 45-90 ml/kg of the child’s blood is replaced with a similar volume of donor red blood cells of group 0(1), Rh-negative)

In other cases, the management tactics for such children depend on the results of the initial laboratory examination and dynamic observation.

In order to prevent PCD in newborns with isoimmune HDN for any of the blood factors (Coombs test - positive), who have an hourly increase in bilirubin of more than 6.8 μmol/l/hour, despite phototherapy, it is advisable to prescribe standard immunoglobulins for intravenous administration. Human immunoglobulin preparations are administered intravenously to newborns with HDN slowly (over 2 hours) at a dose of 0.5-1.0 g/kg (on average 800 mg/kg) in the first hours after birth. If necessary, repeated administration is carried out 12 hours from the previous one.

The management tactics for children with tension-type headache over 24 hours of age depend on the absolute values ​​of bilirubin or the dynamics of these indicators. It is necessary to assess the intensity of jaundice with a description of the number of skin areas stained with bilirubin.

It should be remembered that there is a relative correspondence between the visual assessment of jaundice and the concentration of bilirubin: the larger the surface of the skin is yellow, the higher the level of total bilirubin in the blood: Staining of the 3rd zone in premature infants and the 4th zone in full-term newborns require urgent determination concentrations of total blood bilirubin for further management of children.

Scale of indications for exchange blood transfusion (N.P. Shabalov, I.A. Leshkevich).

The ordinate is the concentration of bilirubin in the blood serum (in µmol/l); on the x-axis - the child’s age in hours; dotted line - bilirubin concentrations at which PCA is necessary in children with no risk factors for bilirubin encephalopathy; solid lines - bilirubin concentrations at which BPC is necessary in children with the presence of risk factors for bilirubin encephalopathy (with ABO and Rh conflict, respectively)

Doctors at maternity hospitals detect yellowness of the skin in seven out of ten children born. Some babies are already born with jaundice, while others turn yellow hours or even days after birth.

In 90% of cases, everything ends well: the diagnosis of physiological jaundice of newborns is confirmed. But in 10% of cases, doctors are forced to state that the baby has a congenital or acquired, often serious, disease that causes the skin and mucous membranes to turn yellow. One such disease is hemolytic disease of the newborn.

THE CONCEPT OF HEMOLYTIC DISEASE OF NEWBORN (HDN)

Hemolytic disease of the fetus and newborn is a congenital disease that can manifest itself both when the baby is still in the womb and when he is already born.

At its core, this is an immunological confrontation between two related organisms - the mother’s body and the child’s body. The reason for this conflict, paradoxically, is the incompatibility of the mother’s blood with the blood of the fetus, as a result of which the child’s red blood cells are destroyed.

The mechanism of development of HDN

The shell of a human erythrocyte is “populated” with various antigens (AGs), there are more than 100 types of them. Scientists have grouped all AGs into erythrocyte systems, of which more than 14 are already known (AB0, Rh, Kid, Kell, Duffy, etc.).

The Rh (Rh) system includes antigens responsible for the Rh status of the blood: Rh (+) or Rh (-). In the AB0 system - antigens that determine the group affiliation of human blood: B and A. The antigens of both of these systems are capable and ready, when they meet the corresponding antibodies (AT), to instantly trigger an immune response. In the blood, antibodies to antigens are normally absent from their native red blood cells.

What happens with hemolytic disease of the fetus and newborn? Antibodies penetrate into the baby's blood through the placenta from the mother's blood and are suitable, like a key to a lock, for the antigens of the fetal erythrocytes. Their meeting starts an immune reaction, the result of which is hemolysis (destruction) of the child’s red blood cells. But where did the antibodies to the child’s erythrocyte antigens come from in the mother’s blood?

REASONS FOR THE DEVELOPMENT OF HEMOLYTIC DISEASE

Hemolytic disease: causes of conflict according to the Rh system

This form of HDN develops when a sensitized woman with Rh (-) blood becomes pregnant with a fetus with Rh (+) blood.

What does the term "sensitized" mean? This means that Rh (+) red blood cells have already entered the woman’s blood at least once, for example, during previous pregnancies with an Rh (+) fetus that ended in childbirth, abortion or miscarriage. Fetal red blood cells penetrate the placenta into the mother’s bloodstream both during pregnancy (especially active at 37–40 weeks) and during childbirth. Sensitization could occur due to blood transfusion or organ transplantation.

The table shows the probability of developing an Rh conflict between mother and fetus.

The mother’s body reacts to the first “acquaintance” with foreign red blood cells by producing appropriate antibodies. From this moment on, antibodies circulate in the mother’s blood and “wait for a new meeting” with foreign Rh (+) red blood cells. And if the first meeting of antibodies with antigens could end quite happily, then the second and all subsequent ones will represent an aggressive confrontation that gets worse each time, affecting the child.

Hemolytic disease: causes in conflict according to the AB0 system

Conflict according to the AB0 system occurs much more often than Rhesus conflict, but, as a rule, it proceeds more easily than the latter.

In the table: agglutinogens are group antigens (in red blood cells), agglutinins are group antibodies (in blood plasma). The blood of each group is a certain set of antigens and antigens. Note that if A antigens are present in the blood, then α antibodies are always absent, and if B is present, then β is not present. Why? Because their meeting triggers an immune reaction of agglutination (gluing) of red blood cells with their subsequent destruction. This is a conflict in the AB0 system, in which hemolytic blood disease of newborns develops.

Sensitization of a woman by the ABO system can occur both during pregnancy and before it, for example, when the diet is saturated with animal proteins, during vaccination, or during an infectious disease.

The table shows the probability of developing a conflict between mother and fetus based on blood type.

FORMS OF HDN AND THEIR CLINICAL FEATURES

According to the severity of the course, in 50% of cases, hemolytic blood disease of newborns is mild, in 25–30% of cases its course is regarded as moderate, in 20–30% as severe.

According to the type of conflict, there are HDN according to the Rh system, according to the AB0 system and according to antigens related to other erythrocyte systems. The clinical forms of hemolytic disease of newborns are largely determined by the type of conflict that has arisen.

Hydrops fetalis

If there is an Rh conflict, and at 20–29 weeks of pregnancy, antibodies massively attack the immature fetus, hydrops fetalis develops.

With this form of hemolytic disease of newborns, the baby is born without jaundice, but with severe swelling of the body and all internal organs. The child has signs of immaturity, reduced muscle tone, weak reflexes, and moves little. The skin is pale and may have hemorrhages. Breathing disorders and signs of acute heart failure are recorded.

Blood tests show severe anemia and very low total protein levels.

If antibodies begin to attack the baby after the 29th week, then clinical form HDN and whether it will be congenital or acquired depend on the amount and when (in utero and (or) during childbirth) maternal antibodies penetrated into the baby.

This form is the result of a massive influx of Rh antibodies from the mother to the child from the 37th week before birth (congenital) and during childbirth (acquired). Distinctive feature The most common (90% of all cases) jaundice form is the early (in the first hours or days) appearance of jaundice. It reaches its maximum by days 2–4 and is accompanied by mild anemia, some tissue swelling, and an enlarged liver and spleen. The earlier jaundice appears, the more severe the course of the disease.

Anemic form

I diagnose this form in 10% of all children with hemolytic disease; its cause is long-term exposure of the fetus, starting from the 29th week, to small “portions” of Rh antibodies. The child is born very pale, with either no jaundice or very mild jaundice. There are gradually increasing signs of bilirubin intoxication (adynamia, lethargy, “poor” reflexes).

Edema form

If after the 29th week of pregnancy Rh antibodies begin a massive attack on the fetus, an edematous form of HDN develops. Clinical manifestations its symptoms are similar to hydrops fetalis.

HDN according to the AB0 system: clinical features:

• jaundice appears late (on the 2nd–3rd day);
• the liver and spleen are rarely enlarged;
• Congenital icteric and edematous forms develop extremely rarely;
• acquired icteric-anemic forms often occur;
• the incidence of serious complications is approaching zero.

Why is AB0 conflict less common thanRh-conflict results in a manifest severe form of tension-type headache?

1. For AB0 sensitization of a woman, it is necessary that much more fetal blood enter her blood than with Rh sensitization.
2. Unlike Rh antigens, group antigens, in addition to erythrocytes, are contained in all other tissues of the fetus, in the placenta and in amniotic fluid. When encountering maternal antibodies, the immune “blow” falls not only on red blood cells, but is distributed over all these tissues.
3. The mother’s body has its own group antibodies that can cope with fetal red blood cells that enter the blood.

HEMOLYTIC DISEASE: CONSEQUENCES AND COMPLICATIONS

1. Disseminated intravascular coagulation syndrome or DIC develops due to a sharp increase in blood clotting. Blood clots form in small and large vessels, infarctions and organ necrosis, and hemorrhages into organs occur. The reason is the massive entry into the blood of tissue thromboplastin from red blood cells that have undergone hemolysis.
2. Hypoglycemia is a decrease in blood glucose levels.
3. Bilirubin encephalopathy is the result of kernicterus, in which extremely toxic indirect bilirubin “saturates” the structures of the brain, thereby destroying neurons. This manifests itself as neurological symptoms and the subsequent formation of bilirubin encephalopathy (paralysis, deafness, etc.).
4. Bile thickening syndrome, in which the bile ducts are blocked by mucous and bile plugs.
5. Secondary damage to the heart muscle, liver, kidneys.
6. Secondary immunodeficiency - develops due to damage to the components of the immune system by indirect bilirubin and immune complexes.

Prenatal diagnosis aims to identify women with high risk development of hemolytic disease in the fetus, the consequences of which are no less dangerous than the disease itself.

Therefore, the obstetrician-gynecologist carefully and specifically questions the patient regarding HDN, finds out the necessary details of the medical history (abortions, number of pregnancies, etc.). Throughout pregnancy, in women at risk for HDN, doctors monitor the titer of antibodies in the blood and amniotic fluid, conduct ultrasound of the fetus and placenta, fetal CTG, and Dopplerometry.

Postnatal diagnosis involves identifying among newborns those who are at high risk of developing TTH and those who already have TTH. To do this, the neonatologist regularly examines all newborns for jaundice, swelling and other signs of illness.

Laboratory tests include monitoring the level of bilirubin and glucose in the child’s blood over time, determining the blood group and Rh factor, immunological tests for the presence of antibodies in the child’s blood, in the mother’s blood and milk.

HEMOLYTIC DISEASE OF NEWBORNS: TREATMENT AND PREVENTION

For hemolytic disease of newborns, treatment can be surgical or conservative. When choosing treatment tactics, doctors are guided by the severity of the baby’s condition and the level of hyperbilirubinemia.

Surgical treatment is a replacement blood transfusion operation. It is prescribed if a newborn has signs of severe tension-type headache, a complicated medical history, or if symptoms of bilirubin intoxication appear. Hemosorption and plasmapheresis are used.

Conservative treatment is primarily phototherapy, irradiation with a special lamp, the rays of which make toxic bilirubin non-toxic.

Prescribed infusion therapy (albumin, saline, glucose solution) is aimed at relieving bilirubin intoxication and promptly removing bilirubin from the body.

Apply medications(zixorin, etc.), activating the liver enzyme system. Adsorbents (carbolene, agar-agar, etc.), choleretic (via electrophoresis), vitamins (E, ATP, A), stabilizing cell membranes, hepatoprotectors (Essentiale, etc.), antihemorrhagic agents (adroxon, etc.) are used.

Zaluzhanskaya Elena, pediatrician