Чт, 10 авг 2017 Автор: врач терапевт ЕленаKornilov
Hemolytic disease in newborns (HDN,
synonyms: fetal eristoblastosis, hemolytic anemia) is
disease manifested by massive destruction of red blood cells,
the main cause of jaundice in a child, spontaneous
abortions and stillbirths.
According to WHO statistics, the disease is detected in 0.6% of children,
mortality is 2.5%. Pathology is related to the immune system,
since it is the result of a conflict of maternal blood antigens and
fetus.
Contents
Factors causing hemolytic disease in newborns
It is revealed that all main antigens contain in erythrocytes.
A total of 6 antigens of the rhesus system were detected. One of them is
rhesus factor called antigen D. It is the cause
incompatibility in 30% of the identified disease, absent in the mother,
available to the fetus when inheriting from the father. Therefore to him
antibodies are produced in the mother’s body. This happens if u
mothers, the rhesus factor is negative, and in the child –
positive.
In other cases, the disease is associated with antibodies that form
blood groups (by ABO system). Hemolytic disease in
infants associated with group incompatibility develops
when there is a blood group of 0 (I) in the mother, and A (II) or B in the fetus
(Iii)
Pathogenesis of the disease
The disease is still developing in utero, its manifestations
different, but the result – hemolysis of red blood cells and fetal death (or
newborn).
Гемолиз — это разрушение мембраны эритроцитов с
release into blood plasma of hemoglobin. The process of destruction itself
red blood cells – normal, as it occurs every 120 days:
so blood cells are updated. But when hemolysis is released into the blood
huge amounts of hemoglobin, resulting in plasma
high bilirubin and iron levels are formed and disturbed
mechanism of the circulatory system. In addition, it is developing
anemia.
All this amount of hemoglobin gets into the liver where it happens.
its neutralization (conjugation with bile acids). With this
huge amounts of released hemoglobin the liver is not
copes with the load and free bilirubin possessing
neurotoxic properties, penetrates all organs, slows down
oxidative reactions causing destructive processes in the tissues and
organs at the cell level, destroying them.
When the content in the blood plasma is 340 µmol / l bilirubin, it
overcomes the blood-brain barrier, changing the structure of cells
the brain and leading to bilirubin encephalopathy. AT
it further leads to disability. For premature babies
the threshold concentration is 200 µmol / L. It happens in
due to the defeat of toxic indirect bilirubin base nuclei
brain (subtalamic, hippocampus, cerebellum, cranial nerves).
Symptoms of hemolytic disease in newborns
Hemolytic disease in newborns протекает в трёх
clinical forms:
• edematous;
• icteric;
• anemic.
The most severe form of the disease
The edematous form is characterized by the accumulation of fluid in the cavities.
organism (pleural, abdominal and pericardial). Going on
increase in mass of the fetus and placenta in 2 times. With an objective examination
are detected:
• pale skin and mucous membranes;
• hepato-and splenomegaly (significant enlargement of the liver and
spleen);
• bruising and petechiae on the skin.
AT общеклиническом анализе крови — тяжёлая
anemia:
• hemoglobin – 30-60 g / l;
• erythrocytes -1×1012 / l;
• respectively expressed anisocytosis, poikilocytosis,
erythroblastosis;
• leukocytosis;
• neutrophilia with a pronounced shift to the left.
Such anemia combined with hypoproteinemia and impaired
capillary permeability causes pronounced cardiac
failure to cause fetal mortality or
newborn
The most common form of hemolytic disease in
newborns
Желтушная — самая часто встречающаяся
clinical form of the disease. It develops by the end of pregnancy.
as a result of exposure to antibodies in the mother’s body to the fetus. Weight
the child is not disturbed, the pregnancy proceeds normally, the child
born with normal skin color. AT дальнейшем развиваются
following manifestations:
• jaundice – the first symptom of the disease – occurs 1-2 days after
birth; intensity and color change as a result
disease progression (orange – bronze – lemon –
unripe lemon;
• hepato – and splenomegaly;
• pastos of tissues in the lower abdomen;
• decrease of reflexes (sucking, swallowing), lethargy,
adynamia.
AT периферической крови:
• anemia;
• pseudo-leukocytosis (due to an increase in young red cells,
perceived in the calculation as leukocytes);
• increase in reticulocytes.
AT биохимических анализах: непрямой билирубин — до 350 мкмоль/л.
It destroys hepatocytes, myocardial cells, selectively affects
on the neurons of the brain. Excess bilirubin is deposited in
renal tubules, leading to renal infarction. Bilirubin
encephalopathy develops in 5-6 days.
AT связи с acute damage to liver cells disrupts the synthesis
proteins and blood clotting factors, which leads to a fall
prothrombin and increased bleeding. Viscosity increases and
the density of bile due to the high content that leads to the formation
stones and the development of obstructive jaundice.
AT течении желтушной формы выделяют несколько
phases:
1. • intoxication in the form of lack of appetite, drowsiness,
lethargy, reduced muscle tone, adynamia, vomiting;
2. • impact on the brain nucleus – convulsive syndrome,
reduction of reflexes, bulging of fontanel, tension of the muscles of the neck,
nystagmus, decrease in heart rate, apnea;
3. • imaginary well-being – develops by 10-14 days of life,
expressed in the reduction of pathological neurological
symptoms, disappearance of seizures;
4. • complications – develop by 3-5 months of life in the form of paralysis,
paresis, convulsions, deafness.
A day can be fatal due to toxic
effects of bilirubin on the brain nucleus. ATыжившие дети отличаются
significant backwardness from peers in mental and physical
have hearing and vision problems, low immune
status, and therefore often suffer from various infections,
complicated by sepsis.
Anemic form
Anemic form развивается в 15% больных.
Appears:
• lethargy;
• pallor;
• hepatolienal syndrome.
Pallor of skin and mucous membranes is determined by 5-7 days, before that it
disguises yellowness.
Laboratory data:
• hemoglobin – 60-100 g / l,
• red blood cells – 2.5-3.0 T / l;
• reticulocytosis;
• bilirubin – normal or slightly elevated
level.
With this form of the disease, recovery quickly occurs with
transfusions of small amounts of Rh negative blood.
Diagnosis and prevention of hemolytic disease in
newborns
Для определения гемолитической болезни у newborns, в первую
turn, study:
• during previous pregnancies;
• miscarriages or stillborn babies;
• gaps in the development of existing children;
• Rh factor of the father of the unborn child.
The antibodies are determined in Rh-negative women.
If they are, for the purpose of prophylaxis a specific
antirhesus gammaglobulin weakens their aggressive
impact.
In the unborn child hemolytic disease is detected when analyzing
amniotic fluid, which is extracted by amniocentesis.
Suspected pathology occurs when it detects
bilirubin and increased specific weight.
Principles of treatment
Treatment requires the immediate removal of dangerous
hemolysis products and the antibodies that led to it. At the same time
protection against toxic effects of indirect bilirubin is necessary
on the organs and systems of the child, stimulation and maintenance of functions
liver and kidney.
For this purpose are used:
• transfusion of fresh blood;
• detoxification measures through the esophageal tube and
parenteral;
• to improve the functions and protection of liver tissue – phenobarbital,
ATP, Methionine, Xylitol, Prednisolone weekly course;
• витаминотерапия (витамины группы AT, С, Е);
• phototherapy – treatment with a blue lamp enhances oxidation
indirect bilirubin, turning it into a non-toxic compound.
With timely treatment, the development of the child does not suffer.
Modern perinatal centers have facilities for
внутриутробного кровезамещения fetus. This is possible with
timely treatment and examination of a pregnant woman, her
conscious approach to the health of the unborn child.
