Hemolytic disease of the newborn is a pathological condition of the child, accompanied by the breakdown of red blood cells, which is due to the incompatibility of the fetal blood with maternal biomaterial according to the criterion of erythrocyte antigens.
General information about the disease
Red blood cells are red cells. They are a formed element of human blood. They are designed to perform a very important function, which is the delivery of oxygen from the lungs to the tissues. In addition, they carry out the return transportation of carbon dioxide.
Agglutinogens are located on the surface of red blood cells. They are also called antigen proteins. They are of two types - A and B. Directly in the blood plasma there are antibodies to them, which are called agglutinins α and ß. On the erythrocyte membrane are other various antigens. The most significant of them is the D. antigen. In the event that it is present, it is believed that the blood has a positive Rh factor Rh +. And if it is absent, then we are talking about negative Rhesus.
With hemolytic disease of the newborn, the blood group with a Rh factor is of great importance against the background of pregnancy. For example, the presence of a conflict between the blood of the mother and the child can lead to agglutination, that is, to gluing and to further destruction of red cells. This disease is found in 0.5% of children. Without proper treatment, this pathology can lead to serious consequences.
The causes of the disease
The cause of the hemolytic disease of the newborn, as already noted, is a conflict of blood between the mother and her child. It can occur under the following conditions:
- Mothers with a negative Rhesus factor form a fetus with a positive Rhesus factor.
- In a woman, blood is related to the first group, and in a child to the second or third.
- There is a conflict over other antigens.
The causes of hemolytic disease of newborns are not known to everyone. In most cases, this disease develops due to the presence of a Rhesus conflict. There is an opinion according to which it is noted that incompatibility in the blood group is even more common. True, due to the mild course of the disease, it is not always diagnosed.
Sensitizing factors
The presence of a Rhesus conflict can provoke a hemolytic disease of the newborn only if there is a previous sensitization of the mother's body. Sensitizing factors are as follows:
- Carrying out a blood transfusion with a positive Rh factor for a woman who has this indicator is negative by nature, regardless of the age at which such manipulation was performed.
- The presence of previous pregnancies, including termination of those after the sixth week. I must say that the risk of developing hemolytic disease increases in women with each subsequent birth. This is especially true if childbirth was complicated by detachment of the placenta or surgical intervention.
Against the background of hemolytic disease of a newborn with blood incompatibility, sensitization of the body can occur in everyday life, for example, in the case of the use of certain products, during vaccinations or as a result of various infections. Another factor that increases the risk of pathology is a violation of the barrier function of the placenta, which occurs as a result of the presence of chronic diseases in a pregnant woman, in addition, against the background of malnutrition and abuse of harmful substances and so on.
Pathogenesis of the disease
The pathogenesis of hemolytic disease of newborns in the blood group is associated with the fact that a woman’s immunity perceives fetal red blood cells as foreign agents and produces special antibodies for their destruction. In Rh conflict, the positive red blood cells of the unborn child penetrate the maternal blood.
As a response, the mother’s body produces special antibodies that cross the placenta and enter the baby’s blood. Then they bind to receptors and destroy red blood cells. Because of this, the amount of hemoglobin in the blood of a child significantly decreases and the level of indirect bilirubin increases. Thus, the fetus develops anemia with hyperbilirubinemia, which is hemolytic jaundice of the newborn.
Indirect bilirubin is a bile pigment that has a toxic effect on all organs. Thus, it negatively affects the kidneys, lungs, liver, heart, and so on. In the case of high concentration, it can penetrate the barrier between the nervous and circulatory system, thereby damaging the brain cells and causing encephalopathy, that is, nuclear jaundice. The risk of brain damage due to hemolytic pathology of newborns may increase in the following cases:
- Lowering the level of albumin, that is, a protein that has the ability to bind and neutralize bilirubin in the blood.
- The presence of hypoglycemia, i.e. a lack of glucose.
- The presence of hypoxia is an oxygen deficiency.
- The presence of acidosis, that is, increased blood acidity.
Indirect bilirubin can damage liver cells. As a result, the concentration of conjugated bilirubin in the blood increases . The insufficient development of the bile duct in the fetus leads to its poor excretion, to stagnation of bile and hepatitis.
Due to severe anemia, against the background of hemolytic disease of newborns in the blood group, foci of extramedullary hematopoiesis in the liver and spleen may appear. As a result, these organs will begin to increase, and erythroblasts, which are immature red blood cells, will appear in the blood. Red blood cell products can accumulate in tissues. In this case, metabolic processes are violated, and therefore there will be a shortage of many minerals: from copper to cobalt, zinc, iron, and so on.
The pathogenesis of this disease against the background of incompatibility in the blood group can be characterized by a similar mechanism. The difference will be that proteins B and A will mature later than D. In this regard, the conflict will pose a danger to the unborn child closer to the end of pregnancy. In premature babies, as a rule, the breakdown of red blood cells does not occur.
Hemolytic disease and its symptoms
Hemolytic disease of the newborn in the blood group can occur in one of the following three forms:
- The icteric form occurs in 88% of cases.
- Anemic form is observed in 10% of cases.
- Edematous form. The risk that this type of disease will manifest itself is only 2%.
Symptoms of the icteric form are the following symptoms:
- The appearance of jaundice. Against her background, a discoloration of the skin and mucous membranes will be noted as a result of excessive accumulation of bilirubin in the body.
- Reduced hemoglobin content, i.e. the development of anemia.
- The presence of an enlarged spleen or liver.
- The presence of lethargy along with a decrease in reflexes and muscle tone.
In the event of a conflict occurring due to the Rh factor, jaundice will occur immediately after birth. If the conflict occurs because of the blood type, then jaundice will appear on the second day. The hue of the skin will gradually change - from orange to lemon.
In the event that the value of indirect bilirubin exceeds 300 μmol per liter of blood on the fourth day, a nuclear form of hemolytic jaundice may develop, which will be accompanied by damage to the subcortical core of the brain. Nuclear jaundice can be characterized by the following four steps:
- The development of intoxication. In this case, loss of appetite with monotonous cries, motor weakness and vomiting will be noted.
- The occurrence of nuclear damage. As symptoms, tension in the occipital muscles will be noted along with sharp cries, fontanel swelling, tremors, the disappearance of certain reflexes and bradycardia.
- The appearance of imaginary well-being (improvement of the clinical picture).
- The appearance of complications of hemolytic pathology. Such complications usually occur at the beginning of the fifth month of life. Among them, paralysis, paresis, cerebral palsy, deafness, developmental delay and the like can be observed.
On the eighth day of hemolytic jaundice, symptoms of cholestasis may occur, which will be expressed by the following symptoms:
- Fecal discoloration.
- The presence of a greenish-dirty skin tone.
- Darkening of urine.
- An increase in direct bilirubin in the blood.
Against the background of the anemic form, the clinical manifestations of the hemolytic disease of the newborn in the blood group include symptoms such as anemia along with pallor, hepatosplenomegaly with a slight increase or normal bilirubin level. The anemic form is characterized by a milder course, since against its background, the general well-being of the baby almost does not suffer.
The edematous variant, also called intrauterine dropsy, is the most severe form of this disease. This form of the disease usually manifests itself in the form of the following symptoms:
- The presence of pallor and severe swelling of the skin.
- The presence of a large abdomen.
- A marked increase in the spleen and liver.
- Presence of lethargy in the muscles.
- Muffled heart sounds.
- The presence of respiratory distress.
- The development of severe anemia.
The development of edematous hemolytic disease very often leads to miscarriages and to the death of children.
Features of the forms of hemolytic disease of the newborn
Pathology can occur in three forms. Each of them has its own characteristics:
- The edematous form is the most severe, but it is rare. With this form, the fetus is born pale, edematous, unable to survive due to cardiovascular failure.
- The icteric form is the most common. In newborns, jaundice of the skin and mucous membranes appears - immediately after birth or the baby may already be born icteric.
- Anemic form of hemolytic disease of the newborn is considered the easiest and most favorable according to the forecast. The condition of the child is generally satisfactory. But blood tests reflect signs of a hemolytic disease: they reveal moderate anemia, an elevated level of bilirubin.
Pathology diagnostics
Diagnosis of this disease can be carried out in the prenatal period. It usually includes:
- Conducting an anamnesis collection, in which the number of previous births and miscarriages is specified, information about the health of older children and possible blood transfusions is clarified.
- Clarification of the Rh factor, as well as the blood group of the pregnant and father of the child.
- An ultrasound scan showing a thickening of the placenta along with polyhydramnios, an increase in the liver and spleen of the developing fetus.
If there is a high risk of hemolytic disease of the blood of newborns, amniocentesis is performed at 34 weeks, which is the abstraction of amniotic fluid through a puncture in the bladder. In this case, the density of bilirubin is determined along with the level of antibodies, glucose, iron and other substances. After birth, the diagnosis of this pathology is made on the basis of clinical symptoms and laboratory studies. In this case, a blood test, as a rule, shows:
- The presence of bilirubin levels above 310 μmol per liter of blood. Immediately after birth, this indicator increases by 18 μmol per liter of blood every hour.
- The presence of a hemoglobin concentration below 150 grams per liter of blood.
- Reduced number of red blood cells with a simultaneous increase in erythroblasts - immature forms of blood bodies.
In addition, a Coombs test is performed showing the amount of incomplete antibodies and allogemagglutinins in maternal blood and in breast milk. All these indicators are checked several times a day. Hemolytic disease should be differentiated from anemia, intrauterine infection, as well as from physiological jaundice and other pathologies.
What are the recommendations for hemolytic disease of the newborn?
Disease treatment
Treatment of a severe form of this pathology in the prenatal period is carried out by transfusion of red blood cells to the fetus, which is carried out through the umbilical cord vein. Replacement transfusion can also be carried out, which is a procedure for alternating excretion of children's blood in small portions, in exchange of which donor blood is introduced. This makes it possible to remove bilirubin with maternal antibodies, while simultaneously compensating for the loss of red blood cells. Today, for replacement transfusion, not whole blood is used, but an erythrocyte mass mixed with frozen plasma. The treatment of hemolytic disease of newborns is not a problem today.
There are the following indications for replacement transfusion for children with this diagnosis - hemolytic jaundice of the newborn:
- Cases when bilirubin in umbilical cord blood is above 60 micromoles per liter.
- Situations in which the hemoglobin content is less than 100 grams per liter of blood.
In some cases, this procedure should be repeated after 12 hours. There are other methods used to treat this disease in newborns:
- Conducting hemosorption, which is a filtration of blood through sorbents that cleanse it of toxins.
- Performing plasmapheresis - removing some of the plasma with antibodies from the blood.
- The introduction of glucocorticoids into the body.
Treatment of hemolytic disease of the fetus and newborn in the lung, as well as in the moderate course, usually includes medications with phototherapy. With hemolytic pathology in newborns, the following drugs are used:
- Intravenous use of protein preparations and glucose.
- The use of inducers of hepatic enzymes.
- The appointment of vitamins that improve liver function and activate various metabolic processes.
- The use of choleretic drugs in case of thickening of bile.
- Conducting a red blood cell transfusion.
- The use of sorbents and cleansing enemas.
Phototherapy, in turn, is a procedure in which a child’s body is irradiated with a white light fluorescent lamp. Against the background of this procedure, indirect bilirubin, which is in the skin, is oxidized, and then excreted from the body.
The attitude to breastfeeding with this diagnosis in newborns is ambiguous. It was previously believed that a baby can only be given breasts two weeks after birth, since by this time antibodies are no longer present in milk. But now doctors are inclined to believe that breastfeeding should begin from the first days, because anti-Rhesus antibodies disintegrate and are destroyed directly in the baby’s stomach.
Consider the consequences of hemolytic disease of the newborn?
Pathology prognosis
The consequences of the disease depend on the nature of the course of this pathology. A severe form can lead to the death of the baby in the last months of pregnancy, as well as within one week after birth. In the event that bilirubin encephalopathy is formed , the following complications are likely:
- The appearance of cerebral palsy.
- The development of deafness or blindness.
- Developmental delay.
The transferred hemolytic disease at an older age can provoke a tendency to frequent diseases, to an inadequate reaction of the body to vaccinations and to allergies. In such adolescents, a decrease in performance with apathy and anxiety is observed.
With hemolytic disease of the newborn, clinical recommendations should be clearly observed.
Pathology Prevention
Prevention of this disease is primarily aimed at preventing the sensitization of the expectant mother. The main preventive measures in this case are blood transfusion, taking into account the Rh factor, along with the prevention of abortion.
Since the previous birth is the main sensitizing factor, within a day after the birth of the first child, a woman needs to enter a medicine with a special immunoglobulin - anti-D. Due to this measure, fetal red blood cells are eliminated quite quickly from maternal blood flow, without provoking the formation of antibodies during subsequent pregnancies. An insufficient dosage of the drug or its untimely administration significantly reduces the effectiveness of this procedure. Prevention of this disease during pregnancy also includes:
- Conducting non-specific hyposensitization, in which detoxification, hormonal, vitamin, antihistamines and other drugs are administered.
- Hemosorption and plasmapheresis.
- Performing specific hyposensitization, in the framework of which a skin graft is transplanted from the husband.
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