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[Fetal hemolytic disease in a patient immunized by six antibodies: diagnosis and treatment].

A case of severe haemolytic disease of the fetus due to six rare alloantibodies is described. In a pregnant women at 23 weeks gestation after the finding of a positive indirect Coombs test maternal antibodies have been precisely identified and titrated: anti-I: 1/23, anti-c: 1/64, anti-S: 1/16, anti-Fya: 1/128, anti-M: 1/64, anti-Jka: 1/32000. Fetal blood group, free and red blood cell adherent antibodies have been investigated on fetal blood samples obtained by means of cord centesis. A compatible donor has been found. Severe fetal anemia has been corrected by ultrasound guided intrauterine transfusions (one abdominal and two intravascular transfusions) with positive outcome of the pregnancy.

Adult↗

Amniotic fluid bilirubin and fetal hemolytic disease.

Six different methods for assessing amniotic fluid bilirubin were compared in 111 samples from 37 patients. When the Liley methods were compared, the chloroform extraction and 5% correction methods resulted in 20% and 4% reductions, respectively, in the mean change in optical density at 450 nm when compared with the uncorrected mean (0.086 +/- 0.06 [p less than 0.05] and 0.104 +/- 0.07 [p, not significant] vs 0.108 +/- 0.08). This reduction was observed in spite of significant visual blood contamination being rarely noted. The predictions based on amniotic fluid analysis were compared with the first fetal or neonatal hematocrit. The chloroform-extracted change in optical density at 450 nm accurately predicted fetal status in all patients, whereas lesser degrees of accuracy were observed with other methods. Hydrops fetalis did not occur during the observation period, and fetuses needing transfusion (n = 5) or early delivery (n = 10) were indicated correctly. The chloroform extraction change in optical density at 450 nm accurately predicts fetal status, and its use should continue.

Amniotic Fluid↗

Evaluation of the preload condition of the fetus by inferior vena caval blood flow pattern.

Using pulsed Doppler ultrasound, blood flow in the inferior vena cava (IVC) was studied in 47 normal fetuses from 24 to 40 weeks of gestation and 35 abnormal fetuses, with the exception of those with arrhythmias. The abnormal fetuses were divided into 4 groups according to diagnosis, i.e., 6 cases of heart disease with hydrops (group 1), 9 cases of heart disease without hydrops (group 2), 11 cases of hydrops without heart disease (group 3), and 9 cases of other fetal diseases (group 4). By measuring the velocity of IVC blood flow, we defined a new index, the change in parallel with reverse flow velocity, and called it the preload index (PLI). In normal fetuses, PLI values ranged from 0 to 0.37 and had no relation with gestational age. The PLI was significantly higher in groups 1-3 than in normal fetuses. In group 1, the PLI was also higher than in group 2. In group 3, the PLI values in 4 cases of chylothorax, 1 of chyloascites and 1 of cytomegalovirus infection were significantly lower than in the remaining 5 cases where the cause of hydrops was undetermined. The PLI was normal in 9 fetuses with other diseases and no hydrops. The PLI was increased in conditions in which excessive preload, tricuspid regurgitation, or some kind of structural heart disease were present.

Blood Flow Velocity↗

Management of fetal hemolytic disease by cordocentesis. II. Outcome of treatment.

Forty-eight of 128 pregnancies complicated by maternal red blood cell alloimmunization (49%) received a total of 142 intravascular transfusions (range, 1 to 7) for treatment of severe anemia (hematocrit, less than or equal to 30%). Thirteen fetuses (27%) had hydrops when therapy was initiated. The overall survival rate was 96%. Eighty-five percent of survivors received two or more transfusions before delivery. The mean gestational age at initiation of therapy was 28 weeks (range, 18 to 36 weeks). Bleeding from uterine and umbilical cord puncture sites was not of clinical significance. The most common complication was fetal bradycardia (8%). Simple intravascular transfusion resulted in the replacement of fetal red blood cells with adult red blood cells and suppression of fetal erythropoiesis. By the completion of the second transfusion, on average, less than 1% of circulating red blood cells were fetal. Within 3 weeks of the second transfusion, the mean reticulocyte count was less than 1%. The rate at which the fetal hematocrit declined after a transfusion (exclusive of the first) was inversely related to gestational age (r = -0.84, p less than 0.0001), permitting a 4- to 5-week interval between transfusions after 32 weeks' gestation. A total of 78% of surviving neonates were delivered at term. Neonates transfused more than once antenatally required less phototherapy (75.8 +/- 54 vs 165 +/- 101 hours, p less than 0.003) and, when delivered at term, fewer hospital days (4.8 +/- 2 vs 8.6 +/- 6 days, p = 0.01) compared with those transfused once. We conclude that the treatment of fetal anemia by intrauterine simple intravascular transfusion permits a term delivery in the majority of cases and is associated with high perinatal survival and low perinatal morbidity.

Blood Transfusion, Intrauterine↗

[Embryofetal diseases in the tropical zone].

The treatment and the prevention of acquired fetal diseases in developing countries are difficult. Nevertheless the deleterious consequences of fever, alcohol consumption, drug intake and malaria on the embryo and the foetus can be avoided by appropriate measures. As for the prevention and the diagnostic in utero of rubella and toxoplasmosis, they may be considered lately when the current priority objectives of public health are reached. Epidemiological studies on these diseases are therefore justified.

Bacterial Infections↗

Detection and management of anatomic congenital anomalies. A new obstetric challenge.

Whereas once the obstetrician was primarily concerned with diseases affecting the mother, the scope of antenatal fetal care has expanded to include a broader range of fetal diseases such as growth disorders and congenital anomalies. The feasibility of identifying congenital anomalies before birth has created an entirely new field of obstetrics. The specialist is now expected to be informed about the differential diagnosis, pattern of inheritance, mechanism of disease, prognosis, optimal obstetric management of the pregnancy, and counseling for future pregnancies.

Congenital Abnormalities↗

Evaluation of prenatal diagnosis of associated congenital heart diseases by fetal ultrasonographic examination in Europe.

Ultrasound scans in the mid trimester of pregnancy are now a routine part of antenatal care in most European countries. With the assistance of Registries of Congenital Anomalies a study was undertaken in Europe. The objective of the study was to evaluate prenatal detection of congenital heart defects (CHD) by routine ultrasonographic examination of the fetus. All congenital malformations suspected prenatally and all congenital malformations, including chromosome anomalies, confirmed at birth were identified from the Congenital Malformation Registers, including 20 registers from the following European countries: Austria, Croatia, Denmark, France, Germany, Italy, Lithuania, Spain, Switzerland, The Netherlands, UK and Ukrainia. These registries follow the same methodology. The study period was 1996-1998, 709 030 births were covered, and 8126 cases with congenital malformations were registered. If more than one cardiac malformation was present the case was coded as complex cardiac malformation. CHD were subdivided into 'isolated' when only a cardiac malformation was present and 'associated' when at least one other major extra cardiac malformation was present. The associated CHD were subdivided into chromosomal, syndromic non-chromosomal and multiple. The study comprised 761 associated CHD including 282 cases with multiple malformations, 375 cases with chromosomal anomalies and 104 cases with non-chromosomal syndromes. The proportion of prenatal diagnosis of associated CHD varied in relation to the ultrasound screening policies from 17.9% in countries without routine screening (The Netherlands and Denmark) to 46.0% in countries with only one routine fetal scan and 55.6% in countries with two or three routine fetal scans. The prenatal detection rate of chromosomal anomalies was 40.3% (151/375 cases). This rate for recognized syndromes and multiply malformed with CHD was 51.9% (54/104 cases) and 48.6% (137/282 cases), respectively; 150/229 Down syndrome (65.8%) were livebirths. Concerning the syndromic cases, the detection rate of deletion 22q11, situs anomalies and VATER association was 44.4%, 64.7% and 46.6%, respectively. In conclusion, the present study shows large regional variations in the prenatal detection rate of CHD with the highest rates in European regions with three screening scans. Prenatal diagnosis of CHD is significantly higher if associated malformations are present. Cardiac defects affecting the size of the ventricles have the highest detection rate. Mean gestational age at discovery was 20-24 weeks for the majority of associated cardiac defects.

Abnormalities, Multiple↗

High activity of fatty acid oxidation enzymes in human placenta: implications for fetal-maternal disease.

As the human fetus and placenta are considered to be primarily dependent on glucose oxidation for energy metabolism, the cause of the remarkable association between severe maternal pregnancy complications and the carriage of a fetus with an inborn error of mitochondrial long-chain fatty acid oxidation (FAO) has remained obscure. We analysed human term placenta and chorionic villus samples for the activities of a variety of enzymes involved in FAO, and compared the results with those obtained in human liver. All enzymes were found to be expressed, with a very high activity of two enzymes involved in the metabolism of long-chain fatty acids (CPT2 and VLCAD), whereas the activity of medium-chain acyl-CoA dehydrogenase (MCAD) was found to be low, when compared to liver. These results suggest that fatty acid oxidation may play an important role in energy generation in human placenta, and that a deficiency in the placental oxidation of long-chain FAO may result in placental dysfunction, thus causing gestational complications.

Acyl-CoA Dehydrogenase↗