Hemolitic disease of a fruit

Description:

Hemolitic disease of a fruit and the newborn — the immune conflict of an organism of mother and a fruit. The organism of mother synthesizes antibodies in response to erythrocyte antigens of a fruit, alien for it. The most frequent type of immunological incompatibility of organisms of mother and a fruit is incompatibility on a Rhesus factor — 97%, incompatibility on blood groups meets much less often. The Rhesus factor is found in a fruit on 8 — the 9th week of pre-natal development, the amount of antigens a Rhesus factor gradually increases. Through a placenta they get into blood of mother and to them anti-Rh antibodies are formed if mother is Rh-negative. Getting through a placenta into an organism of a fruit, maternal antibodies cause an erythrocytolysis. The second name of this pathology — эритробластоз is connected with it. Anemia develops, at hemolysis the increased amount of bilirubin is formed; as compensatory mechanisms in fight against anemia in a liver and a spleen of a fruit are formed the blood formation centers that leads to increase in these bodies and disturbance of their function.

Reasons of a hemolitic disease of a fruit:

The immunological havoc leading to a hemolitic disease of a fruit most often develops at isoserological incompatibility of blood on the Rh system when mother has Rh-negative blood, and at a fruit – Rh-positive. In that case it carries the name a Rhesus factor conflict. Isoimmunization at the same time can occur in two ways: iatrogenic (at the woman's sensitization transfusions of Rh (+) blood in the past) or at fruit and maternal transplacental transfer of erythrocytes of a fruit in a maternal blood stream in the course of pregnancy and childbirth. In case of Rh-incompatibility the hemolitic disease of a fruit is seldom accompanied by the first pregnancy; develops from the 2nd or 3rd pregnancy with increase of risks with each subsequent gestation more often.
As other possible reason of a gemoloitichesky disease serves incompatibility of blood of a fruit and mother on the AB0 system, i.e. at a blood group of mother 0 (I), and at a fruit any other. At the same time antigens A and B from a fruit get through a placenta into a maternal blood stream and cause development immune α-and β-antibodies with the subsequent conflict "antigen antibody". The hemolitic disease of a fruit at AVO-incompatibility has easier current, than at Rh-incompatibility. At AB0-incompatibility the hemolitic disease of a fruit can develop already during 1 pregnancy.
In rather exceptional cases the hemolitic disease of a fruit can be connected with immunological havocs on systems of Daffi, Kel, MNSs, Kidd, Lutherans, etc. or to antigens P, S, N, M.

Symptoms of a hemolitic disease of a fruit and newborn:

Hemolitic anemia  — the easiest form. At the newborn hemoglobin level is lowered., as well as  quantity of erythrocytes. Integuments are pale, the liver and a spleen are slightly increased. A hemoglobin content and bilirubin — on the lower bound of norm. The GMB anemic form appears because of  impact of a small amount of Rh antibodies on the full-term or almost term fetus. Isoimmunization of a fruit occurs more often in labor. The main sign of not heavy damage – anemia.

Hemolitic anemia in combination with jaundice – meets more often, is characterized as more severe form of a disease.    Hyper - or normokhromny anemia, jaundice and a gepatosplenomegaliya is characterized. Icteric coloring, as well as  a vernix caseosa, an umbilical cord, fruit covers and an umbilical cord have Okloplodny waters. The maintenance of an indirect bilirubin is more than upper bound of norm on 10-20 ¼¬¼¼«½ý/l.  The condition of the newborn heavy also worsens. If the hourly gain of bilirubin goes at 5-10 times, then "kernicterus" develops that speaks about defeat of TsNS. In such cases there can be a blindness, deafness, intellectual inferiority. The hemoglobin content makes below norm. The icteric form arises if antibodies affect a mature fruit short time. Quite often infectious complications  — pneumonia, a respiratory distress syndrome, an omphalitis join. After 7 days of life pathogenic influence of the antibodies which got into blood of a fruit stops.

Hemolitic anemia in combination with jaundice and dropsy – the most severe form of a disease. Newborns either are born the dead, or die in the early neonatal period. Symptoms of a disease are: anemia, jaundice and the general hypostasis (ascites, an anasarca), the expressed splenomegaly. Maybe the hemorrhagic syndrome develops. Penetration of maternal antibodies through a placenta happens not always, weight of defeat of a fruit not always corresponds to a caption (concentration) of Rh antibodies in blood of the pregnant woman.

Diagnosis:

All women at whom children with a hemolitic disease, and all women in whom it is found a Rhesus factor sensitization were born are subject to observation of the obstetrician and immunologist. In blood of women define a caption of Rh antibodies till 32nd week of pregnancy once a month; 2 times a month from 32 to the 35th week and since the 36th week weekly.

In antenatal diagnosis of a hemolitic disease are important ultrasonography (ultrasonography) at which such signs can be revealed as: increase in the sizes of a liver and spleen, placenta thickening, hydramnion, fruit edema. According to indications and appointment of the doctor investigate amniotic liquid which is received by a puncture of a bag of waters (amniocentesis). The puncture is carried out through an abdominal wall. Previously at ultrasonography the arrangement of a placenta and a fruit, in order to avoid their injury is defined at a puncture. In amniotic waters define the maintenance of antibodies, a bilirubin indicator, a fruit blood group — which have essential value for assessment of its state and purpose of the corresponding therapy. According to indications it is made also кордоцентез — capture of blood from a fruit umbilical cord.

Treatment of a hemolitic disease:

The following methods are most effective:

1. zamenny hemotransfusion  to the newborn in whose investigation it is removed Rh antibodies and bilirubin. Increase of an antiserum capacity at mother during pregnancy and has to guard till 1:16 a neonatolog concerning GMB  at the newborn above. It is the most adverse when antibodies decrease before childbirth that says that they moved to a fruit.  The severe form of a hemolitic disease can be assumed in the presence of icteric coloring of skin, syrovidny lubricant, an afterbirth, amniotic waters, puffiness and increase in a liver of a fruit.
Treat risk factors of bilirubinovy encephalopathy:
o the low weight of the newborn – is less than 1500 гр.
o the Low content of crude protein is less than 50 g/l —  — a hypoproteinemia
o the Low content of glucose – is less than 2,2 mmol/l — a hypoglycemia
o the Low hemoglobin content – less than 140 g/l — anemia
o Pre-natal infection of a fruit, emergence of jaundice in the first days
Indications to zamenny hemotransfusion:
o emergence of jaundice right after childbirth or during the first hours life;
o the maintenance of an indirect bilirubin in umbilical blood above normal values
o bystry hourly gain of bilirubin during the first hours lives of 6,8 mmol/l
o the level of hemoglobin is lower than 30 g/l.

  For zamenny hemotransfusion use odnogruppny or 0(1) groups the Rh-negative eritrotsitny weight and plasma [odnogruppny or AB(IV)].
At the conflict on the AB0 system eritrotsitny weight has to be 0(1) groups, suspenzirovanny in group AB (IV) plasma. At incompatibility of blood on rare factors carry out individual selection of donors.
At the newborn remove 40-50 ml of blood and enter as much eritrotsitny weight. At repeated zamenny transfusion of a dose reduce twice. The total amount of eritrotsitny weight makes 70 ml on 1 kg of body weight of the child.
Zamenny hemotransfusion is effective for removal from an organism of the newborn of toxic products of hemolysis, an indirect bilirubin, antibodies and nedookislenny products of interstitial exchange. Possible complications: heart failure, air embolism, infection, anemia, hemorrhagic syndrome.

2. The phototherapy  — radiation of the newborn by means of a fluorescent lamp Phototherapy promotes transformation of free bilirubin in skin and capillaries in non-toxic metabolites (biliverdin) which are allocated with urine and bile of a fruit. At the same time albumine loses ability to connect bilirubin. Light gets into the newborn's skin on depth to 2 cm.
Indications to phototherapy:
o pairing jaundice (at the full-term newborns) at  an indirect bilirubin in serum to 170-188 µmol/l
o a hemolitic disease of the newborn on a Rhesus factor and group incompatibility;
o a state after zamenny hemotransfusion at a severe form of a hemolitic disease;
o prevention at newborns from risk group on development of a hyperbilirubinemia (a perinatal hypoxia, unripe and premature newborns, thermal control disturbance).
At newborns toxic effects – an erythema, a dispensiya, burns, a hydropenia can develop. Stops at values of direct bilirubin  more than 85 µmol/l.
3. Therapy by intravenous immunoglobulins. Immunoglobulins in high doses block Fs-receptors which participate in cytotoxic reaction antigen — an antibody and by that reduce a hyperbilirubinemia.
The newborn intravenously enter Imbio-immunoglobuliny in a dose of 800 mg/kg a day within 3 days. Combine with phototherapy.


Complex of therapy of GMB  of the newborn —  zamenny hemotransfusion, phototherapy and intravenous administration of immunoglobulin – reduces the frequency and weight of pathology, improves the forecast of development of children.

It is considered that after the first pregnancy by a Rh-positive fruit the sensitization occurs at 10% of Rh-negative women. At each subsequent pregnancy the Rh-positive fruit immunizes 10%.
From 1000 giving birth at 170 there will be a Rh-negative blood. From them 100 women will have a Rh-positive child.
At strict performance of a technique of specific prevention a Rhesus factor sensitization it is practically possible to solve a problem a Rhesus factor - conflict pregnancy.