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Biomedical subjects

Y Dumez

Publications and source records attributed to Y Dumez.

At least 145 records · Page 8Linked to original sources

Prenatal diagnosis of sickle-cell anemia in the first trimester of pregnancy.

To investigate the usefulness of chorionic biopsy for prenatal diagnosis of sickle-cell anemia by restriction-endonuclease analysis of fetal DNA, we studied 30 pregnancies before elective abortion. When the reproducibility of the technique for obtaining adequate DNA samples was established, we successfully applied the test to five pregnancies at risk for sickle-cell anemia. In two cases, sickle-cell disease of the fetus led to a decision to terminate the pregnancy. In three other cases, a normal or AS genotype was demonstrated. One normal infant has been born, and one other pregnancy is continuing normally. In one case in which fetal death was observed three weeks after sampling, placental abnormalities found on histologic examination were compatible with a chromosomal aberration. Our study shows that chorionic biopsy is feasible for the prenatal diagnosis of sickle-cell disease before the 10th gestational week. If subsequent experience demonstrates this technique to be safe enough for mother and fetus, the ability to test in early pregnancy may make prenatal diagnosis acceptable to more couples at risk for serious genetic disorders.

Abortion, Therapeutic↗

[Prenatal diagnosis of a harlequin fetus using electron microscopy].

We have diagnosed a Harlequin fetus at 22 weeks' gestation. Harlequin syndrome is a fatal, congenital disorder of keratinization whose biochemical basis is unknown. The parents were second cousins and had had four children, two with the Harlequin syndrome who had died at birth and two normal children. During the fifth pregnancy the parents asked us to find out if the fetus was affected. Multiple skin biopsies were done by fetoscopy during the 22nd week of gestation. On binocular loupe examination the skin appeared thickened and fragile to the touch; light and electron microscopy revealed a remarkably thickened stratum corneum with involvement of both follicular and interfollicular epidermis, a feature characteristic of a Harlequin fetus at term. The pregnancy was terminated in the 24th week of gestation: the fetus had all the characteristic clinical features of Harlequin fetus.

Abnormalities, Multiple↗

[Arterial hypertension during pregnancy. 531 cases treated in one centre (author's transl)].

Between 1977 and 1979, 531 consecutive cases of hypertension complicating pregnancy were observed at the Maternité de Port-Royal, Paris. All patients were treated preferentially with methyldopa, and most women with moderate hypertension were treated as out-patients. Perinatal mortality in these hypertensive women was the same as for normotensive women who delivered at the Maternité and for the French population as a whole during the same period.

Female↗

[Percutaneous nephrostomy in utero for bilateral foetal hydronephrosis].

Left percutaneous nephrostomy was performed in a 30-week foetus with bilateral hydronephrosis due to abnormal pyelo-ureteral junction. Puncture of the pelvis and insertion of a 5 cm long F8 Malecot catheter were carried out under ultrasonography. The end of the catheter was left free in the amniotic fluid. Drainage was perfect during 3 weeks, until the skin of the foetus grew over the end of the catheter. At 34 weeks, a much longer catheter was introduced, but it was dislodged from the kidney within 48 hours. Vaginal delivery was induced at 37 weeks, giving birth to a normal male child. On the 4th day after birth the catheter was removed and resection-anastomosis of the left pyelo-ureteral junction was performed.

Adult↗

Prenatal testing for inherited immune deficiencies by fetal blood sampling.

We attempted to develop a prenatal diagnosis in fetuses at risk for immunodeficiency by fetal blood sampling performed under fetoscopy at 18-22 weeks of gestation. In order to obtain normal values, we first investigated thirty-five control fetuses whose blood punctures were undertaken for the diagnosis of haemoglobinopathies. Surface markers and in vitro mitogen-induced proliferation of the fetal lymphocytes were studied using micromethods. We then examined two fetuses at risk for two different types of severe combined immunodeficiency and established their immunological integrity, hence avoiding an unjustified termination of pregnancy. This immunological integrity was confirmed after birth.

Antigens, Surface↗

Antenatal diagnosis of haemoglobinopathies by Biorex chromatography of haemoglobin.

Biorex chromatograhpy of haemoglobin has been compared to the standard chromatographic separation of radioactive globin chains in 60 fetal blood samples obtained for the antenatal diagnosis of haemoglobinopathies. Biorex chromatography of haemoglobin permitted two measurements, the optical density at 418 nm and the radioactivity incorporated into fetal and adult haemoglobin. The two measurements were highly correlated (r2=0.96) and enabled a distinction between homozygous from heterozygous states of the diseases to be made, particularly in beta thalassaemia. A single column was used for 50 analyses. This fast and very sensitive method is proposed for the antenatal diagnosis of haemoglobinopathies using fetal blood.

Anemia, Sickle Cell↗

[Intra-uterine diagnosis of a central nervous system abnormality (author's transl)].

The authors report a case of intra-uterine fetal convulsions. This is a very rare happening in which the cause is not easy to differentiate between a congenital or a traumatic factor. Some features have already been published. Most of them are connected with the syndrome of lack of pyridoxine, which is a familial condition transmitted by a recessive autosome. This diagnosis should be throught of because such a lack is lethal when not treated, and is curable without any after-effects simply by taking pyridoxin. The authors point out the importance of a qualitative analysis and not only a quantitative measurement of active movements that alone can lead to the detection of such abnormalities in fetal behaviour.

Adult↗

Neonatal effects of maternal administration of acebutolol.

Systolic blood pressure, heart rate, and blood glucose concentration were measured in the first three days of life in 10 infants born to mothers who had received acebutolol, a cardioselective beta-adrenergic-blocking agent, for hypertension in pregnancy and compared with values in 10 infants whose mothers had received methyldopa. The blood pressure was expressed as a percentage of the expected value. Blood pressure was significantly lower in the infants of the mothers given acebutolol (p less than 0.02, less than 0.01, and less than 0.01 respectively during the three days of observation). Heart rate was also lower, but the significance was only at the 0.05 level. Blood glucose was not significantly different between the two groups. These results suggest that care should be taken in prescribing beta-adrenergic-blocking drugs during pregnancy.

Acebutolol↗

New techniques for the prenatal diagnosis of hemoglobinopathies.

Two new techniques have been devised for the prenatal diagnosis of hemoglobinopathies performed on fetal blood samples. Isoelectric focusing (IEF) of hemoglobins was compared to the classical chromatography of labelled globin chains for 51 fetal blood samples, 40 being obtained for prenatal diagnosis of hemoglobinopathies, in Paris. In all cases the two methods provided identical results. Adult hemoglobin was quantitatively evaluated. In addition blood samples obtained in other centers after abortion of 22 fetuses homozygous for beta thalassemia did not exhibited measureable amounts of Hb A by IEF. The fetal blood must be free of maternal contamination. If present maternal red blood cells can be completely eliminated by selective lysis using the 0RSKOV reaction. A chromatography of hemoglobins on Biorex 70 has been devised very recently to overcome the two limitations of IEF: First the contamination of fetal blood samples by maternal cells, and second the impossibility to evaluate Hb A when present in proportion below 1%. The chromatography of hemoglobins on Biorex 70 is performed in 75 minutes with 0.1 mg of hemoglobin present in membrane free hemolysate. The optical density recording allows to evaluate .5% of Hb A and to detect .1% of Hb A. In addition, the radioactivity profile of the chromatography can be determined. It has to be used in case of maternal contamination.

Anemia, Sickle Cell↗

[The antenatal diagnosis of Bonnevie-Ullrich's syndrome. The role of ultrasound (author's transl)].

The authors report four cases of Bonnevie-Ullrich's syndrome where the diagnosis was made by ultrasound. The typical picture is of a cystic retrocervical hygroma with generalised lymphoedema which is more marked in the lower limbs and on the backs of the hands and feet, together with ascites. This syndrome adds up to Turner's syndrome. The diagnosis was definite in one case because it was confirmed by amniocentesis with monosomy X caryotype. It is very likely that the three other cases were the same because the anatomo-pathological features of the external and internal genital organs showed that the baby was feminine. In two cases raised alpha-foetoprotein was found. The levels were 1000 micrograms/ml and 2100 micrograms/ml and the authors discuss the origin of the alpha-foetoprotein. It is advisable to terminate the pregnancies in these major forms of Turner's syndrome because they usually die in utero (in two cases) at different stages of the pregnancy.

Abortion, Therapeutic↗

[Fetoscopy].

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Female↗