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Biomedical subjects

B Le Marec

Publications and source records attributed to B Le Marec.

At least 55 records · Page 3Linked to original sources
PubMed1989

[Neonatal mortality at the Ille-et-Vilaine department. Results in 1987].

All 56 neonatal deaths that occurred during 1987 in Ille-et-Vilaine department (France) were systematically analysed, and the history of pregnancy, delivery, neonatal resuscitation, circumstances under which death occurred in neonatal care unit as well as autopsy findings were studied. Each case was then discussed by a multidisciplinary staff and consensus was obtained regarding diagnosis and cause of death. There were 2 separate categories: 26 deaths (46%) were due to related to malformation, or to metabolic dysfunction; 30 deaths were a result of other causes (54%): 9 low birth weight, 5 respiratory disorders, 5 neurologic disorders, 6 infectious diseases, 2 hemorrhagic shock and 3 were of unknown etiology. Information on causes of neonatal death in a specific region may aid in determining public health priorities for that region; if similar studies were carried out in each department, and results compared, this could aid in setting the guidelines for more efficient health treatments and lead to national choices about neonatal public health.

France↗
PubMed1989

[Stillbirths in Ile-et-Vilaine].

The study of stillbirths is a way to approach foetal medicine and a good opportunity to foster a closer relationship between obstetricians, pediatricians and public health physicians, which will lead to preventive measures known to be effective in decreasing mortality rates. Four hundred and ninety pregnancies which ended in stillbirths were investigated: 61 before 27 weeks of amenorrhea, 228 between 28th and 36th weeks, 194 after 37 weeks. The different causes were identified: intrauterine growth retardation and pathological pregnancies remain the most important causes but the authors attract attention to a category of foetal deaths occurring at the end of the pregnancy without any evident cause and for which "postmaturity" was likely. The data attract attention on the necessity to carry out research on stillbirths through a closer collaboration between obstetricians and pediatricians and suggest that appropriate preventive measures may lead to a significant reduction of the 25% of avoidable deaths.

Female↗
PubMed1989

[Congenital diaphragmatic hernia with a late disclosure].

Two further cases of congenital diaphragmatic hernia with delayed presentation are reported: a 6-month-old male presented a posterolateral diaphragmatic hernia with small bowel in left hemithorax masquerading as pleural effusion; an 11-year-old boy with Down's syndrome presented a retrocostoxyphoid hernia revealed by vague faintness. The authors emphasize the deceptive clinical aspect, the different means for diagnosis, the risk of wrong diagnosis and pleural drain, the usual good outcome of these late-onset diaphragmatic herniation.

Child↗
PubMed1989

[Autopsy in neonatal death].

Among 125 neonatal deaths which occurred between 1985 and 1987 in a neonatal intensive care unit, 90 autopsies (72%) were performed. Autopsies in 58 cases (64%) confirmed antemortem conclusions. In 26 cases (28%) post-mortem examination rectified antemortem findings whereas in 6 cases (6.6%) it did not contribute to a better understanding of the cause of death. When there was a risk of an heritable disease and genetic counselling was necessary (31 cases), the autopsy permitted to provide the diagnosis in 4 cases and to reassure the family in 17. In 23 cases the post-mortem examination had to be completed by perimortem studies to be contributive. As no antemortem data allowed to predict the value of the autopsy, a postmortem study should by systematically requested in cases of death during the neonatal period.

Autopsy↗
PubMed1989

[Selective interruption of pregnancy and embryonic malformations of the limbs].

The authors report 3 cases of fetal malformations consisting of amputation of the limbs. These were found in their cases of selective abortion carried out around the 10th week of amenorrhoea by injection under ultrasonic needle guidance of hypertonic saline into selected sacs. These malformations seem to be associated with either the existence of amniotic bands or with a toxic mechanism (direct contact of the limbs of the embryo with neighbouring hyperosmolar membranes). This technique is to be given up in favour of transcervical aspiration, or direct needling of the fetus. The different techniques for selective abortion have been reviewed as well as the so-called "alternative" techniques (such as follicular aspiration) which are preferable.

Abortion, Therapeutic↗
PubMed1988

[Robertsonian translocation and genetic counseling].

A collaborative study on 92 Robertsonian translocations is analysed in relation with the methods of ascertainment, the type of rearrangement and potential imbalance of the anomaly. The results are useful in genetic counselling.

Chromosome Aberrations↗
PubMed1988

[Esophageal duplication with pleural effusion in antenatal diagnosis].

The authors report the case of a patient in whom an antenatal ultrasound showed a mediastinal tumor then a pleural effusion. At birth, the baby was intubated for adequate ventilation and pleural effusion was drained. Ultrasound, oesophagogram, CT scan permitted the diagnosis of tubular oesophageal duplication which was confirmed by surgery and pathologic examination. Review of the literature did not find such antenatal finding. The role of oral contraception during the first weeks of pregnancy is discussed.

Esophagus↗
PubMed1988

[Hyperosmolar coma in a premature newborn infant: iatrogenic complication of parenteral feeding].

The authors report the case of a 34-week preterm newborn weighing 1,545 g receiving a parenteral mixture by a central venous catheter. A mistake in the flow-rate program led to the infusion of 200 ml within 2 h. Coma occurred quickly and the patient showed intracellular dehydration, polyuria, metabolic acidosis, hyperglycemia and hyponatremia. The therapeutic aim was the correction of dehydration and acidosis, as well as the prevention of secondary hypoglycemia. Clinic and biologic symptoms disappeared 6 h later Volumetric infusion pumps need permanent safe and accurate control of volume flow-rate.

Diabetic Coma↗
PubMed1988

[Stenosis of the common bile duct on a common biliopancreatic duct].

A 16 month-old girl presented with congenital common bile duct stenosis revealed by vague faintness. Percutaneous transhepatic cholangiography and surgical biliary opacification showed an abnormal junction of the pancreatico biliary ductal systems with an abnormally long common channel. These anatomic findings seem to be similar to those seen in congenital choledocal cysts and biliary tract dilatation.

Bile Ducts↗
PubMed1987

Genetic counselling in unexpected familial recurrence of achondroplasia.

Two additional pedigrees with familial recurrence of achondroplasia are described. Genetic risk for children of sibs of affected individuals or premutation carriers seems to be low, but it is advisable to monitor at risk pregnancies by midtrimester ultrasonography to diagnose fetal achondroplasia.

Achondroplasia↗
PubMed1987

Hereditary epidermolytic palmoplantar keratoderma associated with breast and ovarian cancer in a large kindred.

We report a large kindred in which palmoplantar keratoderma occurred in association with breast or ovarian cancer or both. This kindred consisted of 61 members of four generations. Thirty-five individuals had palmoplantar keratoderma, including eight women who also had breast or ovarian cancer or both. Five of the six women with breast cancer and both the women with ovarian cancer have died. The proband presented with a yellowish, uniform hyperkeratosis surrounded by a red border covering the entire surface of the palms and soles. Light microscopic examination showed features of epidermolytic hyperkeratosis, and ultrastructural examination revealed the typical markers for this entity, cytolysis and clumps of keratin filaments. This kindred is the first, to our knowledge, to have an association of epidermolytic hyperkeratosis and internal malignancy.

Adult↗
PubMed1987

[What genetic risk should suggest prenatal diagnosis for cystic fibrosis?].

Prenatal diagnosis of cystic fibrosis is today possible by chemical study of amniotic fluid during the 18th week of pregnancy. We have, among 90 families seen for genetic counseling between 1972 and 1985, estimated the risk of recurrence; it was of 0.25 in 55 cases; between 0.05 and 0.16 in 30 cases, greater than 0.01 in 16 cases. Before suggesting a prenatal diagnosis, it is necessary to take as basis the risk of abortion and the reliability of the method. The first risk is well known, less 0.005, in these young women; reliability can be estimated: almost 0.01 of false wrong negative, and this number is probably overvalued if all technical conditions are perfect. In front of these risks, it seems possible to propose a prenatal diagnosis from a risk of 0.01, the families being informed of all risks. We think so to hearten some families and to make possible for them to live quietly these pregnancies, probably non undertaken without our help.

Abortion, Spontaneous↗