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Between 1966 to 1984 a total of 636 intrauterine foetal transfusions were performed at the Hamburg University Hospital on 280 foetuses suffering from severe erythroblastosis. 54.6% of these foetuses survived. Ascitic fluid was found in 29,6% of the foetuses at the beginning of therapy. The survival rate improved to 22%.
Recent developments and improvements in prenatal diagnostic methods, and in particular antenatal ultrasonography, have made intrauterine detection of fetal abnormalities possible. Most defects are best treated after birth, only a few disorders are potentially amenable to surgical treatment in utero. Studies in animal models have helped us to define the pathophysiology, to recognize the interference of the abnormality with organ development and to determine whether elimination of the anomaly might allow fetal development to proceed normally. In-utero repair of congenital diaphragmatic hernia has been practiced on a fetal lamb model but has not yet been attempted in human beings. Treatment of fetal hydrocephalus has been investigated in sheep and rhesus monkeys, and the pathophysiology and intrauterine treatment of fetal hydrocephalus has been studied in fetal lambs and monkeys, but the question of the reversibility of renal dysplasia has not been resolved. Animal models have been described for possible intrauterine treatment of skeletal abnormalities like spina bifida. But more knowledge of embryology and of the pathophysiology of the malformation is needed, as well as about the efficacy and feasibility of this method before it can be attempted in human beings. Prerequisites for fetal surgery include the selection of those fetuses who might benefit from intrauterine treatment, counselling of the family concerned, and a highly experienced multidisciplinary team including a perinatal obstetrician, an ultrasonographer, a pediatric surgeon and a neonatologist. In human beings intrauterine treatment has been performed in erythroblastosis fetalis, urinary tract obstruction and hydrocephalus with encouraging results. There are many ethical, legal and social questions which cannot be answered; new guidelines and rules will have to be laid down.(ABSTRACT TRUNCATED AT 250 WORDS)
Severe foetal Rh erythroblastosis is treated by intrauterine transfusion, in most cases with ACD adenine erythrocyte concentrates. In two cases where delivery took place shortly after the last intrauterine transfusion, metabolic acidosis developed post partum, which could possibly be attributed to the erythrocyte concentrates in the transfusion. Multiple pH level measurements in the ACD adenine erythrocyte concentrates revealed pH levels between 6.4 and 6.8. Erythrocyte concentrates prepared with heparin showed pH levels between 7.18 and 7.25 and should therefore be used for intrauterine transfusion in place of ACD adenine erythrocyte concentrates.
Glycophorin A, the major human erythrocyte sialoglycoprotein, is found exclusively on cells of the erythroid lineage. The amino acid sequence is known, and glycophorin A isolated from mature erythrocytes contains a single N-glycosidic and 15 O-glycosidic oligosaccharides. Monoclonal antibodies against erythrocyte glycophorin A reacted weakly with erythroid precursors while a monospecific rabbit antiserum reacted strongly with immature and mature red cells. Glycophorin A was isolated from cells representing various stages of erythropoiesis in normal bone marrow, from blood cells of neonates with erythroblastosis fetalis, and from the erythroleukemic cell lines K562 and HEL before and after induced differentiation. Analysis of the oligosaccharides showed less O-glycosylation of glycophorin A in erythroid precursors. The degree of glycosylation increased concomitantly with differentiation.
A case of moderately severe erythroblastosis fetalis due to anti-Dib is reported. The mother was a Mexican-Indian woman who had received three units of blood following the delivery of a normal infant three years previously. The affected infant required phototherapy and two exchange transfusions. Although the units were incompatible, the infant tolerated the procedures well and appeared normal when last seen at the age of 2 1/2 months. This case brings to 11 the total number of instances of anti-Dib reported.
The study sought to determine what proportion of the perinatal mortality excess associated with prolonged gestations was due to placental insufficiency. Using data from a large prospective study of pregnancy, the perinatal mortality rate was 20.9/1000 births for post-term and 11.7/1000 for term infants. Only a quarter of the perinatal mortality rate excess in the post-term pregnancies was due to disorders related to inadequate uteroplacental perfusion, i.e., abruptio placentae, large placental infarcts and marked placental growth retardation. Twenty-six per cent of the mortality excess was due to congenital malformations, 19% to amniotic fluid infections, 8% to Rh erythroblastosis fetalis and the remaining 22% to a variety of other disorders. The post-term mortality excess due to congenital malformations was in infants who had hypoplastic adrenal glands, a well-known cause of prolonged gestation. The 4205 placentas of the post-term infants did not show any significant increases in those microscopic lesions that are characteristic of uteroplacental under-perfusion.
Prior to the 1970s and the advent of Rho (D) immune globulin (RIG) for Rh negative women, hemolytic disease of the newborn led to morbidity, long-term disabilities, and mortality. Antepartum RIG administration has been a standard of practice since 1983. Yet, Rh isoimmunization (sensitization) and its sequelae have not been completely eradicated. Rh-related issues remain clinical challenges facing perinatal and neonatal nurses. Evidence for the administration of RIG prenatally and during the postpartum period is presented including controversies and challenges. Current information about fetal and neonatal care of erythroblastosis fetalis and immune hydrops is also presented.
Platelet-derived growth factor (PDGF) affects the growth, migration, and function in vitro of mesenchymal cells, but little is known about its normal physiological functions in vivo. We show here that mice deficient for PDGF B die perinatally and display several anatomical and histological abnormalities. Kidney glomerular tufts do not form, apparently because of absence of mesangial cells. Instead, a single or a few distended capillary loops fill the glomerular space. The heart and some large arteries dilate in late-stage embryos. Most PDGF B mutant embryos develop fatal hemorrhages just prior to birth. Their hematological status includes erythroblastosis, macrocytic anemia, and thrombocytopenia. On the basis of these findings, we conclude that PDGF B has crucial roles in vivo in establishing certain renal and circulatory functions.
Anti-k is a Kell-related antibody. There is little correlation between the maternal antibody titre and the severity of haemolytic disease of the foetus and newborn, and anaemia is usually associated with low bilirubin levels. Severe erythroblastosis has been reported with a low titre anti-k (IAT 8-16). We report a case of severe haemolytic disease of the newborn (HDN) due to anti-k. HDN was associated with a normal bilirubin level and reticulocytopenia. The foetus was monitored by ultrasound, and delivery by elective caesarean section (CS) was planned. The mother was admitted 1 week before the expected date of delivery, and the infant was delivered by urgent CS. The infant required exchange transfusion. As suitable plasma-reduced (k antigen(-)) red cell units were not readily available, k- SAGM red cell units (preserved in extended storage media: SAGM sodium chloride, adenine, glucose and mannitol) were provided. The post-transfusion Hb remained stable, and the infant did not require further transfusion support. Our findings (reticulocytopenia and normal bilirubin levels) support the hypothesis that the pathogenesis of anaemia and haemolysis in anti-k HDN may be similar to that in anti-K (suppression of erythropoesis and immune destruction of K+ erythroid progenitor cells by macrophages in the foetal liver). The ideal product for exchange transfusion is plasma-reduced RBC, less than 5-days old. We provided a 4-day-old SAGM red cell unit for exchange transfusion in a term infant, and this was uneventful. Caution should be taken, however, and renal function and electrolyte levels should be monitored closely. More information is required regarding the safety of SAGM units for exchange transfusion.
The Lutheran blood group system consists of several genes of which Lua and Lub are the best characterized. Over 98% of the Western population is Lu (b+), and consequently antibody formation against Lub is rare. Cases reported in the literature suggest that the expression of Lutheran antigens on red cells of the newborn is weak, and Lutheran antibodies are not known to have caused severe haemolytic disease of the newborn. We describe a case of anti-Lub immunization during pregnancy. Despite increasing anti-Lub antibody titre and antibody-dependent, cell-mediated cytotoxic activity of the anti-Lub, two parameters associated with haemolytic disease of the newborn, the baby did not suffer from erythroblastosis.
In 79 pregnancies with erythroblastosis fetalis, fetal blood was sampled to assess the level of anaemia. Significant correlations were found between the haemoglobin concentration and umbilical artery pH, bicarbonate concentration and base excess. Compensatory mechanisms help to maintain oxygen supply in anaemia but their failure is associated with hydrops fetalis.
A case of rhesus isoimmunization with spontaneous improvement in severity of erythroblastosis with advancing parity is presented. The isoimmunized pregnant woman after having 3 perinatal deaths all due to severe hydrops fetalis, subsequently delivered 2 Rh-positive surviving infants. In her last pregnancy, the timing of delivery was indicated by cessation of fetal movements, followed by a sinusoidal fetal heart rate pattern. The newborn, needing 5 exchanging blood transfusions, subsequently did well.
This paper reports what we believe to be the first case of intrauterine transfusion for severe erythroblastosis due to anti-Duffy(Fya).
Extracorporal detoxication methods in newborns are most unavailable, contraindicated because of technical problems. Herein are reported the experiences with a blood pump unit that was miniaturized for hemodialysis, hemofiltration, and plasmapheresis in newborns. In three premature infants with acute renal failure of different etiology (two newborns with severe Rh-erythroblastosis, 1 premature infant with hypoproteinemia) 13 single-needle hemofiltrations and 6 single-needle plasmaphereses were performed with double head pump, special tube systems, and small modules. The age of the patients ranged from 1 to 14 days, body weight was between 800 and 2,800 g. Four umbilical veins and two femoral veins were used as vascular access. The ultrafiltration rate during the treatment averaged 0.3 ml/min in single-needle hemofiltration and the plasma filtrate flow rate 1.3 ml/min in single-needle plasmapheresis. All treatments were well tolerated. Four patients died due to complications unrelated to the treatment, two patients recovered. These preliminary results show that both hemofiltration and plasmapheresis may be carried out without major problems in premature infants and newborns.
Seventeen infants with severe (14) or moderately severe (3) erythroblastosis were given daily oral supplements of 2-5 or 5 mg folic acid from day 16 (average) to 3-2 months (average). Their rate of weight gain, expressed as weight centiles, was followed for 1 year and was compared with that of a very similar group of 34 erythroblastotic infants without folic acid supplements. By the end of the 4th month, just after stopping additional folate intake, the median centiles for weight had risen from the 40th to the 80th centile, while in the untreated control group they rose during this period from the 35th to the 50th centile. During the second half of the year both groups declined in weight centiles, the 'treated' group ending up at the 50th centile for weight, while the control group fell to the 25th.
Six patients with pregnancies of 19-31 weeks' duration showing evidence of erythroblastosis fetalis were treated with 25 fetal intracardiac blood transfusions. Complications related to the procedure occurred on five occasions in three patients. In two of the six patients the fetus died, but it was unlikely that death was related to the intracardiac transfusions. Fetal intracardiac blood transfusion may result in potentially severe complications but offers an alternative when transfusion cannot be performed into the umbilical cord.
We evaluated the concentrations of vascular endothelial growth factor (VEGF) and angiogenin in the umbilical cord blood from 14 fetuses with erythroblastosis or alloimmune thrombocytopenia and at birth from 28 preterm fetuses, from 42 healthy term fetuses, and from 24 term fetuses born to mothers with insulin-treated diabetes. A correlation appeared between VEGF and angiogenin levels (r = 0.44, p = 0.038). The gestational age correlated with both VEGF (r = 0.38, p = 0.0008) and angiogenin levels (r = 0.80, p = 0.0001). The concentration of VEGF was lower in fetuses born to mothers with insulin-treated diabetes than in the healthy term fetuses (p = 0.0028), but this difference was absent for angiogenin (p > 0.05). In conclusion, in umbilical cord plasma, a developmental increase was evident in concentrations of VEGF and angiogenin during the last trimester of gestation. That the umbilical cord VEGF level was lower in term fetuses born to mothers with diabetes than in term fetuses of healthy mothers may be associated with an aberrant fetal vascular development in diabetic pregnancies.
This study reports the Hb, erythropoietin (Epo), and reticulocyte course at different postnatal ages (range, 8-83 d) of 56 blood samples from 30 newborns (range, one or two blood samples from each newborn) with erythroblastosis fetalis (18 intrauterine transfused (IUTd) and 12 non-IUTd). Hb, Epo, and reticulocyte values were not statistically different in IUTd and non-IUTd cases. As age increased, decreasing Hb values and increasing Epo levels were observed. The reticulocyte count was relatively low in all but six cases. There was an inverse correlation between Epo and Hb (r = -55, p < 0.001), but no correlation was found between Hb and reticulocytes or between Epo and reticulocytes. When groups were formed on the basis of Hb levels, anemic newborns showed higher serum Epo than did nonanemic patients (p < 0.02). In the present study we found a significant increase in Epo levels in anemic newborns without a rise in the reticulocyte count. These results suggest that other mechanisms, rather than low Epo production, could be involved in the pathogenesis of this anemia. Persisting anti-D antibodies, probably at a medullary level, may destroy erythrocyte precusors, leading to anemia with a low reticulocyte count.