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Hemolytic disease of the newborn



Hemolytic disease of the newborn (erythroblastosis) is caused by an immunological conflict between the mother and the fetus due to incompatibility with erythrocyte antigens, which is associated with the development of hemolytic anemia and jaundice. Hemolytic disease occurs in approximately 0.5% of newborns.

Although there are more than 60 systems of red blood cell antigens, most often hemolytic disease of newborns develops when the fetus and mother are incompatible due to Rh or ABO factors. The most common cause of hemolytic disease in newborns is Rh factor incompatibility. Among the many antigens of the rhesus system (C, D, E, c, d, e), the D-antigen is of primary importance in the occurrence of conflict. Hemolytic disease develops if the fetus inherits from the father erythrocyte antigens that are absent in the mother's body. The ingress of fetal red blood cells into the mother’s blood leads to the formation of antibodies in her body, which, in turn, penetrate the placenta to the fetus, are fixed on its red blood cells and lead to their hemolysis. In uncomplicated pregnancy and the absence of previous sensitization, the transplacental penetration of Rh antigens to the mother and, accordingly, anti-Rh antibodies to the fetus is not pronounced. Therefore, the first pregnancy with Rh incompatibility of the mother and fetus, as a rule, ends safely. Most intensively, fetal red blood cells penetrate the mother’s blood during childbirth, after which the active production of antibodies begins. The previous sensitization of the mother (repeated birth, abortion, blood transfusion), as well as the complicated course of pregnancy, accompanied by damage to the placental barrier, contribute to the development of more severe forms of the disease.

Incompatibility with ABO antigens is the second most common cause of hemolytic disease in newborns. Although ABO incompatibility is observed in approximately 20-25% of pregnant women, laboratory signs of hemolytic disease are found in only 1 out of 10 such cases, and forms requiring therapeutic intervention are found in only 1 out of 200. This is due to three reasons: anti-A- and anti-B antibodies primarily relate to IgM that do not cross the placenta; expression of A- and B-antigens on fetal red blood cells is low; in addition to red blood cells, antigens A and B are produced by other cells, on which antibodies partially penetrated transplacentally are fixed. Hemolytic disease with ABO incompatibility occurs almost exclusively in newborns from mothers with 1 (0) blood type, since they sometimes determine anti-A and anti-B-IgG without prior sensitization.

In the pathogenesis of hemolytic disease of the newborn, two processes are leading, due to the excessive destruction of red blood cells: anemia and jaundice (Scheme 22.7). The severity of the disease in these processes varies significantly depending on the degree of hemolysis and the maturity of the fetal organs. Anemia can stimulate extramedullary hematopoiesis, which leads to an increase in the size of the liver and spleen. Anemia is also associated with hypoxic damage to the heart and liver. Myocardial damage leads to the development of heart failure with the subsequent occurrence of edema. Impaired liver function causes hypoproteinemia, which exacerbates the degree of swelling. Bilirubin resulting from hemolysis is an indirect form (see chapter 17). Its conjugation is extremely slow due to imperfection of the enzyme systems of the liver of the newborn. Indirect bilirubin, insoluble in water and possessing affinity for lipids, easily penetrates the blood-brain barrier (imperfect in the newborn) and, being toxic, causes damage to the central nervous system of the child. The maximum concentration of bilirubin is observed in the subcortical nuclei, which turn yellow, and therefore this severe complication of hemolytic disease of the newborn is called nuclear jaundice.

Clinical signs of hemolytic disease usually appear shortly after birth, in the 1st week of life. But with

Scheme 22.7.

The pathogenesis of hemolytic disease of the newborn





a high level of antibodies and pregnancy diseases that contribute to the violation of the permeability of the placental barrier, the disease can develop in the prenatal period and lead to fetal death before childbirth or to the birth of a child with severe manifestations of the disease.
There are three main forms of hemolytic disease, depending on the prevalence of pathological changes: edematous, icteric and anemic. The edematous form is the most severe, characterized by anasarca and accumulation of fluid in the body cavities, pallor of the skin, a significant increase in the liver and spleen. Death occurs from heart failure in the womb or shortly after birth. The icteric form is the most common form. The first symptom is jaundice, developing on 1-2 days after birth. This form is often complicated by nuclear jaundice. Anemic form occurs in 10-15% of children with hemolytic disease, with this form, hyperbilirubinemia is slightly expressed.

Pathological changes in hemolytic disease depend on the severity of hemolysis and, therefore, on the clinical form of the disease. A common sign for all forms is an increase in the liver and spleen, due to a reaction to hemolysis and compensatory extramedullary erythropoiesis. Intravascular hemolysis leads to the formation of hemosiderin and its accumulation in the liver, spleen, bone marrow, lymph nodes (general hemosiderosis). With nuclear jaundice, the brain is swollen, a bright yellow stain is determined on the incision mainly in the area of ​​the basal ganglia, thalamus, cerebellum, spinal cord, less often in the gray matter of the brain. Pigmentation is unstable and disappears within 1 day even with optimal fixation of the material. The morphological diagnosis of hemolytic disease of the newborn is based on the detection of abnormally high erythropoietic activity. Bone marrow activity increases, foci of extramedullary (extramedullary) hematopoiesis are found in the liver and spleen, as well as often in the lymph nodes, kidneys, lungs, and even in the heart. At the same time, the number of reticulocytes, normoblasts, and erythroblasts in the peripheral blood increases. The placenta with hemolytic disease is increased, pale. Microscopically determined pronounced immaturity of its tissue and swelling of the villi. In fetal vessels there are many normoblasts.

There are many diseases in which fetal edema can be observed, not caused by an immunological conflict. They should be distinguished from edema in hemolytic disease of the newborn. The main causes of non-immune fetal dropsy are as follows.

Chromosomal abnormalities of triploidy

trisomy 21 (down syndrome)

monosomy X (Shereshevsky-Turner syndrome)

Gene diseases

Glucose-6-phosphate dehydrogenase deficiency

Homozygous alpha thalassemia

Foam-Shockey Syndrome

Noonan Syndrome

Multiple Pterygium Syndrome

Achondrogenesis

Achondroplasia

Tanatoform dwarfism

The cardiovascular system

Congenital heart defects

Heart rhythm disorders

Vena cava thrombosis

Arteriovenous Shunts

Anomalies of the chest cavity

Congenital cystic adenomatous lung disease

Diaphragmatic hernia

Asphyxic chest dysplasia

Infections

Viral Pancarditis Coxsackie

Congenital Syphilis Cytomegaly Toxoplasmosis

Genitourinary System

Malformations of the kidneys and urethra

Congenital Nephrotic Syndrome

Chorioangioma of the placenta

Placental transfusion syndrome

Nuclear jaundice is also not a state inherent only in hemolytic disease, it can accompany a number of diseases and conditions in which there is an increase in blood levels of indirect bilirubin (hereditary hemolytic anemia, hereditary defects of the liver enzyme systems, their immaturity in deeply premature babies, etc.) .

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Hemolytic disease of the newborn

  1. Hemolytic disease of the newborn.
    Hemolytic disease of the newborn (erythroblastosis) is caused by an immunological conflict between the mother and the fetus due to incompatibility with red blood cell antigens. Hemolytic disease occurs in approximately 0.5% of newborns. Hemolytic disease develops if the fetus inherits from the father erythrocyte antigens that are absent in the mother's body. Most often
  2. Hemolytic disease of the fetus and newborn
    SYNONYMS Erythroblastosis of the fetus and newborn. DEFINITION OF GBN - isoimmune hemolytic anemia that occurs in cases of incompatibility of the blood of the mother and the fetus by erythrocyte Ag, while Ag is localized on the erythrocytes of the fetus, and antibodies to them are produced in the mother's body. CODE ICD-R55 Hemolytic disease of the fetus and newborn. P55.0 Rhesus isoimmunization of fetus and newborn P55.1
  3. HEMOLITIC DISEASE OF THE NEWBORNS
    REASONS AND MECHANISM OF DEVELOPMENT Hemolytic disease of the newborn is a disease caused by an immunological conflict due to the incompatibility of the blood of the fetus and mother with erythrocyte antigens. Hemolytic disease of the newborn is diagnosed in approximately 0.6% of all newborns. GBN usually causes incompatibility of the fetus and mother for Rh or AB0 antigens. Incompatibility
  4. Hemolytic disease of the newborn
    Hemolytic disease of the newborn (GBN) is one of the urgent problems of modern obstetrics and neonatology. Currently, fetal death and death of newborns due to hemolytic disease, their disability as a result of irreversible central nervous system processes are often noted. According to WHO, the incidence of HDN is at least 5 per 1000 births, with more than 60-70% of newborns needing
  5. HEMOLITIC DISEASE OF THE NEWBORNS (ERYTHROBLASTOSIS OF THE FETUS)
    Unlike physiological jaundice of newborns (icterus neonatorum simplex), which is characteristic of the vast majority (80–90%) of newborns and gives a good prognosis, the described pathological form, also known as “icterus neonatorum guavis”, is characterized by exclusively severe course and, as a rule, ends with the death of the child. Etiology and pathogenesis. Origin
  6. Hemolytic disease of the newborn
    Hemolytic disease develops as a result of isoimmunization caused by incompatibility of the blood of the mother and the fetus. In 80–85% of cases, the cause of the disease is iso-immunization according to the Rhesus factor, in 20–15% - according to the ABO system. Allocate anemic, icteric and edematous forms of the disease. From the first hours of life, the anemic form is manifested by pallor of the skin, hypodynamia, enlarged liver and
  7. Hemolytic disease of the fetus and newborn
    Changes in the fetal body with hemolytic disease Hyperbilirubinemia does not significantly affect the condition of the fetus, since the mother’s liver assumes the function of neutralizing the resulting bilirubin. Hyperbilirubinemia is dangerous for the newborn. According to the autopsy of fetuses who died from hemolytic disease, a characteristic dropsy with bloating and severe
  8. HEMOLITIC DISEASE OF THE NEWBORN (HDN)
    HEMOLITIC DISEASE OF THE NEWBORNS
  9. Rhesus conflict, HDN - hemolytic disease of the newborn and bilirubin encephalopathy
    Often in the history of children suffering from cerebral palsy, there is GBN (hemolytic disease of the newborn), which occurs as a result of an immune conflict between the mother and fetus, due to the incompatibility of their blood with various red blood cell antigens. As early as 1932, LK Diamond et al. reported that dropsy of the fetus, severe jaundice and anicteric anemia in
  10. Task 44. HEMOLITIC DISEASE OF THE NEWBORN. JAUNDICE FORM
    A woman in labor D., 24 years old, was taken from the Central District Hospital to the hospital on December 22 in connection with the development of labor. Complaints of cramping abdominal pain. From the anamnesis. Menarche from 13 years old, established immediately, regular, 6 days, after 28 days. The last menstruation is March 23-27. Pregnancy is the third. The two previous ones ended in medical abortion in the period of 8 weeks. Observed on FAP from a 20-week
  11. THEME. PATHOLOGY OF THE PERINATAL PERIOD. MATTERING, MATURITY. BIRTH INJURY. HEMOLITIC DISEASE OF THE NEWBORN (for pediatric faculty)
    Motivational characteristic of the topic. Knowledge of the topic materials is necessary for successful assimilation of them in the departments of the clinical cycle. In the practical work of a pediatrician, it is necessary for the clinical anatomical analysis of sectional observations. The general purpose of the lesson. On the basis of knowledge of the morphological manifestations of diseases and syndromes of pathology of the perinatal period, learn to recognize and conduct them
  12. Perinatal pathology, prematurity, tolerance. Birth injury. Hemolytic disease of the newborn. Intrauterine infections: cytomegaly, toxoplasmosis, herpes.
    1. The perinatal period is called period 1. from 12 weeks to 40 weeks of gestation 2. from 22 weeks to 40 weeks 3. from 18 weeks to 5 days after the birth of a child 4. from 22 weeks to 7 days after the birth of a child 2. Make a correspondence: FORM OF HEMOLYTIC DISEASE SIGNS 1. fetopathy without edema and jaundice a) in the liver and spleen erythroblastosis 2. edematous b) pathological immaturity of the placenta 3.
  13. DISEASES OF THE PERINATAL PERIOD. Gestational age and fetal mass. Intrauterine Hypoxia. BIRTH INJURY. GENERAL DAMAGE TO THE HYPOXIC GENESIS. DISEASES OF THE LUNG PERINATAL PERIOD. Congenital malformations. Intrauterine infections. HEMOLITIC DISEASE OF THE NEWBORNS
    The perinatal period of development is called the period from the 22nd full week of fetal life to 7 full days after the birth of the baby. The gestational age of the fetus is determined by the gestational age. Pregnancy duration is measured from the first day of the last normal menstruation. The average duration of pregnancy is 280 days (40 weeks). Full-term is a child born
  14. Diseases of the Newborn Asphyxia of the Newborn
    Asphyxia of newborns can develop both during normal childbirth with pelvic presentation of the fetus, and in the pathology of the birth process. In pigs, asphyxiation of the fetuses located in the tops of the uterine horns can occur with head presentation. A similar situation is often observed in carnivores, especially with weak contractions and attempts, when the fetus moves too slowly along the uterine horn. Asphyxia
  15. HEMOLITIC DISEASE
    Serological incompatibility of the blood of the mother and the fetus can lead to a number of pathological changes in the fetus and cause it to die in the uterine cavity or cause the death of the newborn in the first days of life. Pathological changes in the fetus, called erythroblastosis, or hemolytic disease (morbus haemoliticus neonatorum, erythroblastosis foe-
  16. The terminological dictionary for the topic: “Diseases of the newborn. Skin diseases. Belly button. Sepsis".
    Aseptics - a set of measures aimed at preventing the entry of m / o into the human body. Bacteremia - the presence of bacteria in the circulating blood. A vesicle is an element of a rash in the form of a vesicle filled with serous exudate. Hyperemia - local plethora. Hyperthermia - overheating of the body. Dermatitis is an inflammation of the skin. Omphalitis is an inflammation of the umbilical wound. Pyoderma - pustiform
  17. Microspherocytic hemolytic anemia (Minkowski-Shoffar disease)
    MICROSPHEROSITAL HEMOLITIC ANEMIA (MINKOVSK-SHOFFAR DISEASE) refers to hereditary diseases, which are based on a violation of the protein structure in the erythrocyte membrane. The German therapist Oscar Minkowski identified the disease from the group of hemolytic syndromes, and after 7 years (in 1907), the French therapist Anatole Schoffar found a decrease in resistance in these patients
  18. Methodical manual for students. Nursing process in diseases of newborns (skin diseases, navel, sepsis), 2007

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