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Erythroblastosis fetalis produced by anti-k.

We report an instance of transfusion-induced anti-k so severe that three intrauterine intravascular fetal transfusions were required. The pretransfusion circulating hemoglobin level in the fetus was 60 g/l and hematocrit was 0.19. This, to our knowledge, is the first example of the rare alloantibody, anti-k, producing erythroblastosis so severe that fetal transfusions were required in order to prevent hydrops fetalis from developing. Anti-k alloimmunization, which in a period of 20 years and 8 months occurred only once in 3,246 alloimmunized pregnancies in Manitoba, can cause severe fetal disease. The k-alloimmunized pregnant woman should be managed in the same manner as the D-, c-, or K-alloimmunized pregnant woman.

Adult↗

Fetal intravascular transfusion for severe erythroblastosis: effects on haematology and survival.

Since February 1984, 8 fetuses (including a set of hydropic twins) with severe erythroblasts in the second trimester have received intravascular transfusions guided by ultrasound. These transfusions were associated with a decreasing fetal reticulocytosis, a decreasing proportion of circulating fetal haemoglobin and a decreasing mean fetal red corpuscular volume. All infants were born alive at an average of 5.5 weeks after the first transfusion; 3 infants died, including the hydropic twins and another with lethal congenital anomalies. All 5 survivors required simple transfusions for up to 54 days after birth because of prolonged bone marrow suppression. In severe erythroblastosis in the second trimester, direct intravascular transfusion using ultrasound guidance promises to improve fetal outcome.

Blood Transfusion, Intrauterine↗

Ultrasound-guided fetal intravascular transfusions for severe erythroblastosis, 1984-1993.

The results of the first 10 years' experience in ultrasound-guided fetal intravascular transfusions at the Royal Women's Hospital were reviewed. Since the first transfusion, a variety of techniques have been employed in 78 fetuses, all with severe erythroblastosis. A total of 288 intrauterine transfusions have been attempted with an overall survival rate of 75.6% (59 of 78). The overall survival rate for delivered fetuses improved from 64.3% (18 of 28) in 1984-1987, to 82.0% (41 of 50) in 1988-1993. There was a total of 33 hydropic fetuses, of whom 20 (60.6%) survived, significantly fewer compared with 86.7% (39 of 45) of the nonhydropic fetuses (odds ratio [OR] 0.25, 95% confidence interval [CI] 0.09 to 0.70, p < .01). Fetuses who were sicker at the time of transfusion, as reflected by larger haemoglobin deficits, had lower survival rates, as did those requiring transfusions at earlier gestational ages. When these variables were allowed for, the survival rate significantly improved over time (OR 6.3, 95% CI 1.3 to 30.4, p < 0.05), probably reflecting the increased skill of the ultrasonologists, but the presence of hydrops per se was no longer important. Variations of the technique employed, such as exchange or intraperitoneal transfusion, or different sites for transfusion, were not significantly related to survival.

Blood Transfusion, Intrauterine↗

Blood glucose and plasma insulin and glucagon response during intravenous glucose tolerance test in newborn infants affected by erythroblastosis foetalis.

Intravenous glucose injection (1 g/kg b.w.) was performed in eight newborn infants affected by erythroblastosis foetalis (IEF) and in seven controls during the first day of life in order to study insulin and glucagon response. The IEF infants were affected by mild or moderate hemolytic disease and their blood glucose values and plasma insulin concentrations before and throughout the test did not differ significantly from those of the controls. After the glucose injection the plasma glucagon concentrations showed great variations in both groups. The control infants did not show any significant changes; in the IEF infants, significant decreases were seen at 3 and 20 min of the test. These data seem to indicate that the alpha-cell sensitivity to glucose is greater in IEF than in normal infants and is not dependent on the development of the glucose-mediated insulin release mechanism.

Bilirubin↗

Rupture of the spleen in erythroblastosis fetalis.

A male infant of 36 weeks' gestation, weighing 3080 g, with erythroblastosis, ruptured spleen, and bilateral suprarenal haemorrhages is described. The infant survived after exchange transfusions and splenectomy.

Blood Transfusion↗

Colloid osmotic pressure in erythroblastosis fetalis.

Measurements were made of total proteins, albumin, and colloid osmotic pressure on cord blood samples from 15 infants with erythroblastosis fetalis (six of whom were hydropic) and from 151 non-rhesus non-hydropic control infants. The erythroblastotic infants had levels of total protein and albumin which fell within the normal range for gestational age, but their colloid osmotic pressures were abnormally low. It seems that low colloid osmotic pressure may provide a reasonable explanation for the occurrence of hydrops fetalis.

Birth Weight↗

Prenatal asphyxia, hyperlacticaemia, hypoglycaemia, and erythroblastosis in growth retarded fetuses.

The umbilical venous oxygen and carbon dioxide tensions, pH, lactate and glucose concentrations, nucleated red cell (erythroblast) count, and haemoglobin concentration were measured in 38 cases of intrauterine growth retardation in which fetal blood sampling was performed by cordocentesis. The oxygen tension was below the normal mean for gestational age in 33 cases; in 14 it was below the lower limit of the 95% confidence interval for normal pregnancies. The severity of fetal hypoxia correlated significantly with fetal hypercapnia, acidosis, hyperlacticaemia, hypoglycaemia, and erythroblastosis. These findings indicate that "birth asphyxia" is not necessarily due to the process of birth.

Blood Cell Count↗

Erythroblastosis models. II. Materno-fetal incompatibility in chimpanzee.

Erythroblastosis fetalis represents a significant hazard for successful management of pregnancy in man and in marmosets, but not in crab-eating macaques. Materno-fetal blood group incompatibility in chimpanzee is described as a contributing factor in the death of an infant. The findings indicate that parental blood groups should be taken into consideration when breeding chimpanzees.

Animals↗

Intravascular intrauterine transfusion for severe erythroblastosis fetalis using different techniques.

Over a 3-year period, 44 ultrasound-guided intravascular transfusions were performed between 18 and 32 weeks on 15 patients with severe erythroblastosis fetalis due to Rh immunization. In 4 fetuses, the first transfusion was performed before 20 weeks, in 6 between 20 and 25 weeks and in the remaining 5 between 25 and 31 weeks. Eight of the 15 fetuses were hydropic at the time of referral. Five transfusions were done in the intrahepatic umbilical vein, 6 were simple transfusions via percutaneous umbilical cord puncture, and 33 were partial exchange. There were 4 intrauterine deaths before 26 weeks, despite successfully performed transfusions: 3 of these fetuses were severely hydropic, while in the remaining fetus hydrops had been reversed in utero. Following delivery by cesarean section at 32 weeks of gestation, 1 of the neonates developed respiratory distress syndrome and died 17 h after birth. The overall survival rate was 67% (10 of 15 cases): 4 of the 8 hydropic fetuses (50%) and 6 of the 7 nonhydropic fetuses (83%) were alive at birth and survived the perinatal period. Three of the 5 losses occurred among the first 4 cases, while in the last 11 cases the survival rate increased to 82% (9 of 11).

Blood Transfusion, Intrauterine↗

Pancreatic endocrine cell fractions in erythroblastosis fetalis.

Pancreatic sections from 21 cases of rhesus disease and 20 control newborn infants of 30--40-wk gestational age were stained by the immunoperoxidase method for insulin, glucagon, somatostatin, and pancreatic polypeptide (PP). The fractional area occupied by each cell type was estimated, taking note of whether the gland contained PP-rich (ventral lobe) or PP-poor islets (dorsal lobe). In the PP-rich part of the pancreas, the volume fraction of all four endocrine cell types was significantly greater in the rhesus cases than in the controls. No difference was found between the two groups in the PP-poor part of the gland. The results show that abnormal development of the PP-rich part of the pancreas occurs in erythroblastosis fetalis. The localization of the changes to one part of the pancreas may explain some of the earlier conflicting reports on this topic.

Erythroblastosis, Fetal↗

Immunohematological study in newborn infants with erythroblastosis fetalis transfused in utero.

The present study reports immunohematological data (anti-erythrocyte titer, anti-erythrocyte functional activity, percentage of sensitized erythrocytes) in 11 patients with erythroblastosis fetalis transfused in utero (IUTd). At birth it was possible to define two groups of newborns: one with low (group 1) and one with high (group 2) percentage of circulating sensitized erythrocytes, respectively. The presence of a low rate of sensitized red cells at birth in IUTd infants did not reduce the number of exchange transfusions required postnatally. On the contrary, babies of this group were affected by a more severe disease as shown by higher anti-erythrocyte maternal titer, higher anti-erythrocyte functional activity and a higher degree of fetal hemolysis. The persistence of hemolysis after birth, in spite of the absence of sensitized circulating erythrocytes, may be due to intramedullary hemolysis.

Blood Transfusion, Intrauterine↗

[Specificity and incidence of erythrocyte antibodies in pregnant patients with intrauterine transfusions for fetal erythroblastosis].

The specificity and frequency of irregular erythrocyte alloantibodies in serum obtained from 85 pregnant women managed by a total of 480 intrauterine transfusions for treatment of fetal erythroblastosis was examined over a 4-year observation period. 138 alloantibodies reactive in the indirect antiglobulin test were detected. Their specificities were widespread. The frequency of non-anti-D alloantibodies primarily responsible for fetal immunohemolysis confirmed by elution from fetal red cells increased to 8% compared with studies performed in the 70s. 16 (19%) patients developed additional alloantibodies after onset of intrauterine transfusion therapy. Regarding the fact of the high incidence of secondarily induced alloantibodies, the high prevalence of antibody mixtures and the occurrence of rare alloantibodies against blood group antigens with weak immunogenic potency, we concluded that many of the patients were 'high responders'. Therefore the role of fetomaternal transplacental hemorrhage induced by invasive intrauterine examination methods and transfusions is discussed here. It obviously has to be considered as the main cause of the immunohematologic complications.

Blood Grouping and Crossmatching↗

[ABO incompatibility and newborn haemolytic disease: two cases with major erythroblastosis].

Two cases of newborn haemolytic disease because of ABO incompatibility are reported. In both cases, blood erythroblastosis exceeded 300 per 100 leukocytes at birth. Both newborns developed early jaundice and one presented with anaemia. Intensive phototherapy begun soon after birth was an effective treatment. The diagnosis of newborn jaundice and the patho-physiological basis of haemolytic disease because of ABO incompatibility are discussed.

ABO Blood-Group System↗

High-dose intravenous gamma globulin: does it have a role in the treatment of severe erythroblastosis fetalis?

The role of high-dose intravenous (IV) gamma globulin in the treatment of erythroblastosis fetalis was assessed in five pregnancies with severe Rh (four) or Kell (one) isoimmunization. These women were treated with IV gamma globulin (1.0 g/kg body weight) once a week. In addition, fetal blood transfusions were performed when indicated. In four patients with Rh sensitization, high-dose IV gamma globulin treatment had no apparent effect on the total number of intrauterine transfusions required, the interval between transfusions, or the volume of blood required at each transfusion. The treatment did not prevent fetal hydrops and had no effect on maternal antibody titers. In one patient with Kell sensitization, however, the course of the disease was less severe than anticipated, suggesting that IV gamma globulin treatment may have modified the severity of the disease. We conclude that high-dose IV gamma globulin does not appear to be useful in the treatment of severe Rh disease. Its role in Kell and other types of red-cell isoimmunization deserves further evaluation.

Adult↗

Intrauterine intravascular transfusion for severe erythroblastosis fetalis: how much to transfuse?

Intrauterine intravascular transfusion is now believed to be a more precise method for treating fetal anemia in erythroblastosis fetalis than is intraperitoneal transfusion. Previously established guidelines for the volume of blood to be given in intraperitoneal transfusion at a specific gestational age are not applicable for intravascular transfusion. In 28 patients, intravascular transfusion was performed on 81 occasions between 19-34 weeks' gestation. The total number of transfusions ranged from one to six per patient. The aim at each procedure was to achieve a final hematocrit of 35-50%. Factors examined as likely to determine the volume of blood required included pre-transfusion hematocrit, post-minus pre-transfusion hematocrit (hematocrit increase), the hematocrit of the transfused blood, gestational age, estimated fetal weight, and interval from last transfusion. The factors found to be most predictive of total volume of blood required for transfusion were the hematocrit increase and either estimated fetal weight or gestational age.

Blood Transfusion, Intrauterine↗

[Rhesus immunization in a rhesus-positive pregnant woman: fetal erythroblastosis caused by the rhesus antibody anti-C].

A rhesus-D-positive woman aged 32 years with two previous pregnancies was delivered of an infant with erythroblastosis foetalis caused by the rhesus antibody anti-c produced by alloimmunization during pregnancy. Screening for antibodies in the 12th week of pregnancy yielded negative results. No screening was undertaken in the 35th week of pregnancy. The infant was born with severe anaemia. Following delivery, the anti-c alloimmunization was diagnosed and three exchange transfusions were administered. On the fourth day of life, the infant developed the inspissated bile syndrome. No cerebral disorders were demonstrated. Antibody screening of rhesus-positive women has now been introduced at the 35th week of pregnancy to avoid similar cases.

Adult↗

Conjugated hyperbilirubinemia in infants with erythroblastosis fetalis.

In an attempt to identify the incidence of conjugated hyperbilirubinemia in infants with erythroblastosis fetalis the records of 67 infants were reviewed. Twenty-two infants were found to have direct bilirubin concentrations greater than 1.0 mg./dl. Among 11 infants who underwent intrauterine exchange transfusion, nine (82%) had conjugated hyperbilirubinemia. Of the remaining 56 infants without the procedure, only 13 (23%) showed evidence of this complication. To predict infants at risk of developing conjugated hyperbilirubinemia, the ratio of hematocrit and total bilirubin concentration in cord blood (H/B ratio) was examined.

Bilirubin↗