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Basal ganglia and thalamic calcification following intrauterine intravascular transfusion.

In recent years intrauterine intravascular transfusion has become the method of choice for the treatment of severe fetal erythroblastosis. Despite the association of the procedure with fetal complications such as bradycardia and overtransfusion, later neonatal neurological consequences have been only rarely reported. A case is described of a neonate who underwent repeated intrauterine intravascular transfusion for treatment of erythroblastosis and who later developed calcification in the thalamus and the basal ganglia. The possible pathogenetic mechanisms are discussed.

Basal Ganglia Diseases↗

[Blood picture findings in children with Parvovirus B19 infections (fifth disease/erythema infectiosum)].

The human parvovirus B19 provokes erythema infectiosum ("e.i."); moreover there is a wide range of diseases due to parvovirus B19 without exanthema/rash. The erythropoietic blast cells of the bone marrow seems to be the main target cells for this virus. Therefore in cases of prenatal infection the consequences are extremely similar to fetal erythroblastosis ("non-immunological" fetal hydrops). In postnatal life the parvovirus B19 infection causes hyporegenerative phases of the erythropoiesis with anaemia after 3-4 weeks. We studied the white blood cell count (WBC), erythrocytes and thrombocytes in children suffering from (serologically well documented) parvovirus B19 infection with exanthem/"e.i." (group 1; n = 23), without exanthem (group 2; n = 46) and with unknown febrile exanthematous rashes (group 3; n = 76). We did not find any characteristic data in the WBC for a diagnosis of parvovirus B19 infection. However we have for the first time documented a significant thrombocytopenia in "e.i." (group 1) not found in group 2. The thrombocytopenia appears earlier than the anaemia, because the lifespan of thrombocytes is considerably shorter than that of erythrocytes. These data suggest that parvovirus B19 attacks not only "erythropoietic" blast cells but also immature bone marrow cells, which are later responsible for the thrombocytopoiesis.

Adolescent↗

[Cardiotocogram findings in antenatal monitoring of fetal hematoblastosis and during intrauterine intrafetal transfusion].

34 falls with Morbus haemolyticus fetalis were controled from the 30. to 40. week of pregnancy by cardiotocography. The order of the severity of fetal erythroblastosis and certain types of oscillation devoted: In slight cases of Morbus haemolyticus fetalis preponderated (Hb greater than 15 g%) a undulating curve of oscillation (80%). Middlesevere cases (Hb 9-15 g%) showed in 57.2% an undulating and in 42.8% a saltatoring types of oscillation. In cases of severe fetal anemia (Hb less than 9 g%) we found at 64.2% a silent to narrowed undulating and at 21.4% saltatoring types of oscillations. A normal type of oscillation we not observed in this group. The various degrees of a chronical fetal hypoxia caused by an anemia and a placental dysfunction have been discussed as a reason for the typical CTG-curves. At hydrops fetus et placentae are bearing silent and sinusoid heart frequency curve and the CTG-sign late deceleration. A fetal intrauterine transfusion seems not very successfully, at this findings. During the intrauterine transfusion were established in all cases to a restriction of fluctuation at simultaneous increase of frequency.

Blood Transfusion, Intrauterine↗

[Blueberry muffin baby: the pathogenesis of cutaneous extramedullary hematopoiesis].

Two neonates exhibited the clinical picture of the "blueberry muffin baby" at delivery. The integument manifested petechiae and purpuric magenta-colored macules, papules, and plaques, as well as blueberry-colored ecchymoses. These findings led to the diagnosis of a connatal cytomegalovirus infection and fetal erythroblastosis, respectively. The hemorrhagic-purpuric looking skin lesions reflected extramedullary hematopoiesis with ultrastructural study disclosing evidence of both erythro- and granulopoietic lineage. For the first time, we were able to demonstrate that complexes of red cells in various stages of maturation can occur in the skin, similarly to the erythroblastic islands of the bone marrow. In the pathogenesis of extramedullary hematopoiesis, mechanisms underlying the reconstitution of blood cells must be considered. These may reactivate hematopoiesis in organs where it previously occurred in embryonic and fetal life. Possible causative factors may be great compensatory demand, deficient replacement, or loss or dysfunction of corpuscular blood elements. This would explain the occurrence of this disease entity in conjunction with etiologically completely heterogeneous systemic diseases.

Cytomegalovirus Infections↗

Fetal lactic dehydrogenase variation in normal pregnancy and in cases of severe intra-uterine growth restriction.

Physiological and pathological fetal levels of lactic dehydrogenase (LDH), including its five different iso-enzymes are still poorly known. Our objectives were to compare total LDH levels and its five iso-enzymes between a control group of healthy fetuses and a group of fetuses with severe intra-uterine growth restriction (IUGR), and to determine the biochemical associations and the prognostic value of elevated LDH activity in fetuses with IUGR. Total LDH levels, haematologic values and liver enzyme activities were measured in 108 healthy fetuses from 17 to 37 weeks of gestation and in 44 fetuses with severe IUGR. Total fetal LDH in plasma from the healthy fetuses were constant throughout pregnancy (mean (SD)= 305.09 (46.97)). Total LDH values in plasma significantly increased in cases of IUGR (p=0.003), and the degree of increase was significantly correlated with fetal erythroblastosis (n =44, r=0.80, p<0.001). LDH 5 significantly decreased in the IUGR group (p=0.03). Total LDH values strictly above 400 IU/l (a value equal to the mean+2 SD in the healthy fetus group) were found to be significantly associated with thrombocytopenia (p<0.001), erythroblastosis (p=0.008) and an increase in AST value (p=0.03). These results suggest that the fetal LDH value in plasma is a useful biological marker for severe chronic distress.

Case-Control Studies↗

The management of severe erythroblastosis fetalis by fetal transfusion: survival of transfused adult erythrocytes in the fetus.

The survival of adult erythrocytes transfused into the fetus was measured after intravascular and/or intraperitoneal transfusion. We performed 80 fetal transfusions on 20 severely anemic fetuses, 18 of whom survived. The survival of adult erythrocytes in the fetal circulation was similar to that in the adult circulation and did not depend on the route of transfusion, gestational age, or presence of hydrops. Eighty percent of blood transfused into the fetal peritoneal cavity appeared in the fetal circulation.

Adult↗