11/209. "Bloodless" treatment of a Jehovah's Witness infant with ABO hemolytic disease. An ABO-incompatible term infant girl born to parents who are jehovah's witnesses was admitted to our neonatal unit with a high serum bilirubin level necessitating exchange transfusion. The parents signed a request that blood should not be administered under any circumstances. However, they authorized us to apply the possible alternative treatments of orally administered D-penicillamine (300 mg/kg per day divided in three doses for 3 days), phototherapy, intravenous fluids, and recombinant human erythropoietin (200 U/kg subcutaneously on every second day for 2 weeks). Herein, we report the outcome of this baby, who was discharged from the our unit in good condition after treatment. Her physical growth and motor milestones at 14 months of age revealed no red flags for neurodevelopmental maturation. To our knowledge, this is the first case of an infant who received such a combined alternative (and "bloodless") treatment of serious ABO hemolytic disease of the newborn. ( info) |
12/209. Interpretation of the direct antiglobulin test in neonates with haemolytic disease of the newborn. Neonatal blood grouping and direct antiglobulin test results need to be interpreted with caution following multiple intrauterine blood transfusions for haemolytic disease of the newborn (HDN). Except for a few reports published about 20 years ago, this fact is not well documented. We report a case emphasising the problem and its consequences. ( info) |
13/209. ABO hemolytic disease of the newborn: a unique constellation of findings in siblings and review of protective mechanisms in the fetal-maternal system. Two siblings born 6 years apart presented with similar findings of hepatosplenomegaly, dermal hematopoiesis, hemoglobinuria, and increased platelet consumption, but only moderate anemia and normal serum bilirubin. ABO incompatibility was identified, and other causes were excluded. A review of the current understanding of mechanisms that promote and prevent antibody-mediated hemolysis in the fetus is reviewed. Due to the low ratio of observed to expected significant clinical events among ABO incompatible mother-infant pairs, and the multiplicity of mechanisms that diminish hemolysis, we speculate that severe ABO hemolytic disease of the newborn occurs when there is a specific failure in one of these preventive mechanisms. ( info) |
14/209. Hemolytic disease of the newborn due to anti S antibodies. We report a case of hemolytic disease in a newborn due to anti S antibodies. Baby R was born at term to an O mother whose antibody screen was positive for phenotype big S. Cord blood eluate revealed anti-S RBC; antigen: RBC typing for S- was positive. physical examination of baby was unremarkable. The infant's HCT was 44.2 at 6 hours of age. At 48 hours, the HCT decreased to 33.5, bilirubin peaked to 5.4, retic had peaked to 6.8. By seven days, all these values reverted to the normal, and baby has remained asymptomatic. ( info) |
15/209. Umbilical artery regression: a rare complication of intravascular fetal transfusion. BACKGROUND: Intravascular fetal transfusion is an important therapeutic advance but is associated with several complications. Regression of an umbilical artery associated with transfusion is rare. CASE: A case of red blood cell alloimmunization managed by serial transfusions was complicated by functional loss of an umbilical artery during pregnancy. Refractory fetal bradycardia occurred during the last transfusion procedure, requiring emergency preterm cesarean delivery. Coagulative necrosis, thrombosis, and focal calcification of one umbilical artery was confirmed after delivery. CONCLUSION: Umbilical artery regression associated with transfusion therapy is rare and may complicate subsequent fetal transfusions. ( info) |
16/209. Benefits and risks of fetal red-cell transfusion after 32 weeks gestation. OBJECTIVE: To compare the outcome after intrauterine transfusion (IUT) between fetuses treated before and those treated after 32 weeks gestation. SETTING: National referral center for intrauterine treatment of red-cell alloimmunization in The netherlands. Study DESIGN: Retrospective evaluation of an 11 year period, during which 209 fetuses were treated for alloimmune hemolytic disease with 609 red-cell IUTs. We compared fetal and neonatal outcome in three groups: fetuses only treated before 32 weeks gestation (group A, n=46), those treated both before and after 32 weeks (group B, n=117), and those where IUT was started at or after 32 weeks (group C, n=46). RESULTS: survival rate was 48% in group A, 100% in group B, and 91% in group C. Moreover, fetuses in group A were hydropic significantly more often. Short-term perinatal loss rate after IUT was 3.4% in the 409 procedures performed before 32 weeks and 1.0% in the 200 procedures performed after 32 weeks gestation. CONCLUSION: Perinatal losses were much more common in fetuses only treated before 32 weeks gestation. Two procedure-related perinatal losses in 200 IUT after 32 weeks remain a matter of concern because of the good prospects of alternative extrauterine treatment. ( info) |
17/209. Subclinical hemolytic disease of the newborn due to anti-e. A case of subclinical hemolytic disease of the newborn resulting in reticulocytosis and positive direct and indirect antiglobulin tests in an infant both ABO- and Rh(D)-compatible with its mother is reported. The antibody was an IgG, non-complement-binding molecule present in low titer with a low avidity for the antigen, acted optimally in enzyme systems, did not manifest dosage effect, and caused slight extravascular destruction of fetal erythrocytes. Routine antibody screening of all pregnant women should be performed and neonatal cord blood specimens analyzed to avoid unexpected infant morbidity and mortality, and to characterize hemolytic disease of the newborn due to rare atypical antibodies more fully. ( info) |
18/209. First example of hemolytic disease of the newborn caused by anti-Or and confirmation of the molecular basis of Or. BACKGROUND AND OBJECTIVES: The rare MNS antigen Or (MNS31) is sensitive to ficin, papain and sialidase, but partially resistant to trypsin (0.05%); the effect of alpha-chymotrypsin is not known. A point mutation, 204C --> T in exon 3 of GYPA, is associated with the Or phenotype. We report here the first case of hemolytic disease of the newborn (HDN) caused by anti-Or, and expand the information on the nature of the Or determinant. MATERIALS AND methods: A woman, gravida 4, para 0, delivered a baby whose red blood cells (RBCs) were positive (2 ) on the direct antiglobulin test (DAT). The mother's serum, an eluate made from the baby's RBCs and the RBCs of the baby's father were investigated. Exon 3 of GYPA, extracted from the father's genomic dna, was amplified and sequenced. RESULTS: The mother's serum reacted at room temperature, 37 degrees C and on the indirect antiglobulin test with RBCs from the baby's father. The father's RBCs were M N S-s Or . The antibody in the mother's serum and in the baby's eluate was identified as anti-Or. The serum did not react with the father's RBCs treated with trypsin (180,000 U/ml), but did react with his alpha-chymotrypsin-treated RBCs. Amplification and sequencing of dna from the father revealed a single point mutation, 204C --> T, in GYPA exon 3. At birth, the baby had clinical symptoms of HDN and was transfused with 36 ml of packed RBCs and received phototherapy for eight days. At week 11, the baby's M N S s Or RBCs were negative on the DAT. CONCLUSION: This is the first case of HDN caused by anti-Or. The observed point mutation, 204C --> T, confirms that of a previous report and predicts a change of Arg (Or-) to Trp (Or ) at amino acid 31. ( info) |
19/209. Treatment of hemolytic disease of the newborn caused by anti-Kell antibody with recombinant erythropoietin. Recent data suggest that antibody-mediated suppression of erythroid progenitors may contribute to the anti-Kell-induced alloimmune hemolytic disease of the newborn (HDN). A 32-week-old girl who was positive for Kell was born to a mother who was negative for Kell but known to have anti-Kell antibodies. After birth, the baby had HDN and hyperbilirubinemia develop (peak bilirubin 21 mg/dL at day 9 of life). which was treated with phototherapy. Although the hyperbilirubinemia resolved, she became progressively anemic (hematocrit 22%) with an inappropriately low reticulocyte response (1.1%) and erythropoietin (EPO) level (20 mU/mL). To avoid the need for a blood transfusion, she was treated with recombinant erythropoietin (rEPO) and oral iron supplements. One week after starting EPO, the reticulocyte count increased to 9.1%. erythropoietin therapy was continued for a total of 9 weeks, with resolution of her anemia at the end of therapy (hematocrit 35%). Thus, we were able to successfully treat the anemia with rEPO with avoidance of blood transfusion. This patient demonstrates that the antibody-mediated erythroid suppression in Kell alloimmune anemia can be overcome by rEPO. Recombinant erythropoietin should therefore be considered in the management of infants with severe or hypoproliferative anti-Kell-associated anemia. ( info) |
20/209. Massive fetomaternal transplacental hemorrhage as a perinatology problem, role of ABO fetomaternal compatibility--case studies. BACKGROUND: Massive fetomaternal transplacental hemorrhage is not simply a problem of possible alloimunization in Rh incompatibility but also endangers the fetus (newborn) by massive anemization. Bleeding from placental vessels can occur after small trauma to the gravid uterus with mild or no clinical signs (bleeding or spotting, pain, hypertonus). The rupture of anchoring villi related to early uterine contractions is also possible. In the case of slow blood loss, the fetus reacts by adequate or inadequate compensatory reactions (hydrops fetus). Rapid and massive blood loss is followed by perinatal hypoxic damage and finally death. Our goal was to map out the diagnostic and therapeutic possibilities in regard to specific neonatal care. CASE REPORT: We evaluated four cases of fetomaternal transfusion during a 2-year period with special regard to postpartum adaptation of the newborn and the perinatal outcome. The incidence of adverse outcomes following massive fetomaternal transplacental hemorrhage was 50% (2 of 4). There was one perinatal death and one infant was affected by spastic quadriplegia. CONCLUSIONS: For diagnosis, it is possible to use cardiotocography (decreased variability, sinusoid pattern), ultrasound (biophysical profile) and special hematological tests for quantitative determination of fetal erythrocytes in the maternal blood. For the treatment of such cases one should consider premature termination of pregnancy or intraumbilical transfusion. ( info) |