Haemolyticus neonatorum disease
| Classification according to ICD-10 | |
|---|---|
| P55 | Hemolytic Disease in Fetuses and Newborns |
| ICD-10 online (WHO version 2019) | |
Morbus haemolyticus neonatorum (also: Fetopathia serologica , fetale Erythroblastose or Erythroblastosis fetalis ) is a serious and complex health disorder of the fetus and the newborn, which occurs already before birth and at this time is called Morbus haemolyticus fetalis . The cause is usually a blood group incompatibility in the rhesus system. However, other conditions in which the child's erythrocytes are damaged and dissolved ( hemolysis ) can also be responsible. The unborn child already develops considerable anemia in the womb and, as a result, an insufficient supply of oxygen to the entire organism with pumping weakness of the heart ( heart failure ), effusions in the chest and abdominal cavity ( pleural effusion and ascites ) and water retention in the entire body (generalized Edema ). The physician calls the full picture of this condition hydrops fetalis . If detected early, attempts can be made to treat the disease by means of a blood transfer ( transfusion ) in the womb. After birth, the newborns need at least phototherapy , if not an exchange transfusion . For the most common cause, Rhesus incompatibility, there is systematic prevention in Germany and Austria by administering antibodies against the Rhesus blood group characteristic to all Rhesus-negative mothers immediately after delivery.
Cause (pathogenesis)
The prerequisite is that the mother has already had contact with a blood group characteristic that is foreign to her - usually the rhesus characteristic ( rhesus incompatibility ). This may have happened, for example, in the case of a previous pregnancy (including a miscarriage) or a previous blood transfusion. This sensitization leads to the production of antibodies of the IgG class against these blood group characteristics of the child, which, after passing through the placenta, lead to an increased breakdown of the erythrocytes loaded with antibodies in the child's spleen . The fetus tries to compensate for the loss by producing more blood. If the breakdown is faster than the new formation, the child gets anemia, which leads to an undersupply of oxygen ( hypoxia ) and thus to general tissue damage.
frequency
Blood group intolerance occurs - in addition to rhesus intolerance - with a significantly lower frequency with other blood group characteristics, which is why the antibody screening test is used to search for a large number of sensitizations during pregnancy. The sequence of the EMH-causing blood group antibodies depends on various factors, since (with the exception of AB0) only sensitization leads to antibodies and, depending on the genotype frequency of the population, an incompatible blood group occurs in the child. In addition, an intolerance, for example with AB antibodies, leads to a mild course that almost never requires treatment. Anti-D antibodies were the cause of 98% of the newborns infected with MHN. In the remainder of the group, two other rhesus intolerances follow first - Anti-c with 66%, Anti-E with 14.6% - followed by Kell intolerance with 9.8%, as well as Anti-C, Anti-Duffy and Anti-Kidd with 2.7% each (each in the remaining group of 2% of all cases). It should be noted that most antibody screening tests are negative - clinically significant antibodies are only detected in 0.24% of pregnant women.
Symptoms
In the womb, there is initially increased water retention in the tissues and finally also effusions in the abdominal cavity (ascites) and the pleural cavity (pleural effusion). The amniotic fluid is also significantly increased ( polyhydramnios ). The pumping weakness of the heart - the cause of most symptoms - can also be detected in the ultrasound in the womb. The full picture of these symptoms is also called hydrops fetalis . After delivery, in addition to the anemia and edema, premature and particularly severe neonatal jaundice (icterus praecox) is particularly noticeable.
Diagnosis
As part of prenatal care, a blood group determination and an antibody test for irregular blood group antibodies are carried out in early pregnancy. If the result is negative, the latter is repeated in the 24th to 27th week of pregnancy. The development of hydrops fetalis is monitored through regular ultrasound examinations so that treatment can also be initiated intrauterine if necessary. Blood can be drawn from the umbilical cord to determine whether anemia is present or a blood transfer can be carried out. After the birth (postpartum), in addition to determining the blood count, a so-called Coombs test is carried out, with which antibodies are detected on the erythrocytes. A determination of bilirubin and various hemolysis parameters ( LDH , reticulocytes ) are useful as further blood tests .
therapy
If haemolyticus fetalis disease is noticed during pregnancy, it is generally possible to carry out blood transfusions via the umbilical cord in the womb (intrauterine) in order to avoid the development of hydrops fetalis. Postpartum jaundice , which is present in around half of the newborns with a Rh incompatibility, does not require any treatment or phototherapy is sufficient. The other half with severe jaundice will need a blood exchange transfusion . The administration of immunoglobulins can possibly alleviate the hemolysis. The full picture of a hydrops fetalis is always an emergency for the neonatologist , which results in a variety of intensive care measures even in the delivery room. As a rule, the children must be intubated and ventilated immediately, receive blood transfusions immediately, and the effusions in the chest and abdominal cavity are punctured for relief .
prophylaxis
In addition to the regular antibody screening tests during pregnancy for the early detection of haemolytic disease, rh-negative pregnant women are injected with antibodies against the Rh trait (anti-D immunoglobulin) in the 28th week of pregnancy and no later than 72 hours after the birth of a Rh-positive child. This is also after miscarriage ( abortion ), amniocentesis ( amniocentesis ), chorionic villus sampling necessary or bleeding of the placenta. The antibodies load the erythrocytes transferred from the fetus and lead to a rapid breakdown in the mother's spleen before the mother's immune system can recognize the rhesus antigen and form its own antibodies. This avoids sensitization.
history
Haemolyticus neonatorum disease was first described in twins by a French nurse in 1609: one newborn was stillborn with hydrops fetalis and the second had severe jaundice and died of what we now call kernicterus . These two conditions weren't brought back together until 1932 when Diamond et al. showed that hemolysis of red blood cells in the fetus leads to extramedullary erythropoiesis followed by hepatosplenomegaly and an inundation of the bloodstream with erythroblasts, a condition they called erythoblastosis fetalis.
It is believed that this type of blood group incompatibility affected the relationship between Goethe and Christiane Vulpius : only the first son August survived, all other children died shortly after birth.
literature
- R. Roos et al.: Checklist neonatology. The neo-ABC. Stuttgart, 2000. ISBN 3-13-125051-8
Web links
Individual evidence
- ↑ a b c Sandra Wienzek: Morbus haemolyticus neonatorum. (No longer available online.) University of Gießen, 2009, formerly in the original ; Retrieved January 9, 2010 . ( Page no longer available , search in web archives ) Info: The link was automatically marked as defective. Please check the link according to the instructions and then remove this notice.
- ^ JM Bowman: RhD hemolytic disease of the newborn. In: The New England Journal of Medicine . Volume 339, Number 24, December 1998, pp. 1775-1777, ISSN 0028-4793 . doi : 10.1056 / NEJM199812103392410 . PMID 9845715 .
- ↑ Eduard Seidler: "... less than four weeks of real comfort ..." Goethe's sufferings and diseases. Acta Historica Leopoldina 59, 9–28 (2012) p. 18
