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Biomedical subjects

M André

Publications and source records attributed to M André.

At least 19 recordsLinked to original sources

[Maturation and neurological distress in the newborn].

In the newborn, structural and functional cerebral abnormalities are described as well as clinical manifestations of neurological distress, change with gestational age, and therefore with the degree of central nervous system maturation. The different maturational stages of the cerebral structure, their vascularization and myelinization from the gestational age of 24-25 weeks to the full-term neonate are briefly reported. The cerebral lesions and mechanisms of hypoxoischemic encephalopathy in the full-term neonate are summarized; an analysis of the various clinical symptoms and additional investigations (in particular the electroencephalogram) allows a precise prognosis to be made. In the premature newborn, there are 2 types of cerebral lesions: intraventricular hemorrhage and periventricular leukomalacia. The latter is primarily responsible for neurological sequelae. Clinical manifestations are poor and atypical; data based on complementary procedures (imaging and electroencephalography) allow an accurate evaluation to be made of the damage and neurological prognosis.

Brain

[Platelet group polymorphism in Provence. Comparison with the frequencies of platelet-specific allo-antigens observed in other populations].

Systems HPA-1 (Pla); HPA-3 (Bak) HPA-5 (Br) are involved in neonatal alloimmune thrombocytopenia and post-transfusion purpura. The frequencies of platelet-specific antigens in these three systems have been studied among one hundred one unrelated blood donors from Provence (South of France) for three generations. Typing was performed by the MAIPA test (monoclonal antibody-specific immobilization platelet antigen). The phenotypes frequencies found were: HPA-1a (PlA1): 97%; HPA-3a (Baka): 88.1%. These frequencies are quite similar to those reported in Europe and North America, but are different compared to Oriental and South American populations. Our Provence population has the highest frequency of HPA-5b (Bra) yet reported: 23.8%. These results define the polymorphism of platelet-specific antigens in the Provence population. Similar studies, among other populations, would provide new data for geographical haematology, which has so far been based on erythrocyte, leucocyte and serum polymorphisms. The variations between populations in these platelet-specific polymorphisms would be so many useful descriptive elements for the epidemiological study of associated diseases.

Blood Donors

[Long-term prognosis of congenital atrioventricular block].

The aim of this study was to assess the long term prognosis of congenital atrioventricular block (AVB). From 1965 to 1990, 42 cases of congenital AVB (22 antenatal or natal diagnoses and 20 presumed congenital AVB according to Yater's criteria). The AVB was isolated in 28 cases and associated with cardiac disease in 14 cases (8 of which were corrected transposition of the great arteries). The average age of the patients was 14 years (range 32 years to 18 months) at the time of the study. There was a clear female predominance (64%). Maternal connective tissue disease was present in 18% of cases (in the group of children born after 1977 when maternal connective tissue diseases was systematically looked for). Cardiac failure was present in 10 cases (8 with associated AVB); syncope and sudden death were observed in 11 cases. The indication for pacemaker therapy was the presence of poor prognostic factors: syncope, poorly controlled cardiac failure, low heart rate, increased QRS duration, prolonged QTc, infrahisian AVB, long pauses or arrhythmias on Holter monitoring. The only significant prognostic factors in this series were a previous history of syncope, increased QRS duration and a QTc of over 0.45 seconds. Fourteen patients were paced (endocavitary pacing only from 1981), usually in the DDD mode: 8 for syncope, 2 for cardiac failure, 4 for a poor prognostic factor.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

[The place of new therapies in the treatment of congestive heart failure. The combination of digitalis and diuretics is always in good place].

In recent years, thanks to a better understanding of the pathophysiology of congestive heart failure and progress made in the pharmacology of cardiovascular drugs, new therapeutics have been advocated in the treatment of congestive heart failure. Among them, converting enzyme inhibitors are the most useful. However, the classical association of digoxin-furosemide and general measures remains a very effective first-choice treatment in most cases. Only in particular situations, such as cardiomyopathy and decompensated atrio-ventricular insufficiency, should priority be given to converting enzyme inhibitors. Phosphodiesterase inhibitors are essentially used within the context of post-cardiac surgery intensive care. Beta-blockers which have been recently proposed for treatment of adult patients must not be used, as there is still no data available on their effectiveness and tolerance in pediatric patients.

Age Factors

[Neonatal cardiac transplantation for hypoplasia of the left heart diagnosed in utero].

The poor results of palliative surgery in the hypoplastic left heart syndrome has led Bailey to propose neonatal cardiac transplantation for this condition. The authors report the case of a neonate who underwent transplantation 2 days after birth for hypoplastic left heart with mitro-aortic atresia, diagnosed in utero. The child was prescribed triple immunosuppressive therapy (steroids, azathioprine, ciclosporine) and is now 24 months old. An episode of acute rejection during the first month was treated with a short intensive course of intravenous steroids. At the fourth month, the child underwent aortic angioplasty for an isthmic stenosis. Growth is retarded (- 2SD) and he has mild renal failure but psychomotor development has been normal. The number of neonatal cardiac transplantations remains limited by parental refusal and the lack of donor organs. Despite encouraging medium term results, questions remain as to the long-term viability of the graft and the patient's renal function.

Female

Clonazepam in neonatal seizures: dose regimens and therapeutic efficacy.

The attempt has been made to define the optimal dose regimen of clonazepam in newborns suffering from neonatal convulsions. Results obtained from 22 patients (GA 28-41 weeks; PNA 4 h to 23 days) indicated that a dose of 0.1 mg/kg every 24 h was satisfactory in the majority of the patients. It is recommended as a starting regimen.

Asphyxia Neonatorum

Eosinophilia-myalgia syndrome associated with L-tryptophan. A case report with pulmonary manifestations and review of the literature.

On October 1989, eosinophilia and incapacitating myalgia, together with arthralgia, dyspnea, cough and edema of the extremities, were shown to be associated with L-tryptophan ingestion. Since then, 1531 cases of eosinophilia-myalgia syndrome have been reported in United States and 22 in Belgium. We report here the unusual pulmonary presentation of this syndrome with a dramatic response of eosinophilia to corticotherapy. The cardio-pulmonary symptoms of eosinophilia-myalgia syndrome and its pathophysiology, which remains unclear, are discussed. The withdrawal of the substance and corticotherapy generally lead to complete recovery although several deaths have been reported.

Eosinophilia-Myalgia Syndrome

Modulation of lipid chylomicron-synthesizing enzymes in rats by the dietary (n-6):(n-3) fatty acid ratio.

The effect of diets with various (n-6):(n-3) polyunsaturated fatty acid (PUFA) ratios and a constant polyunsaturated: saturated fatty acid ratio on the basal activity of chylomicron lipid synthesizing enzymes was investigated in rat intestinal microsomes. Enzymes studied were: acyl-CoA:cholesterol acyltransferase (ACAT); acyl-CoA:lysophosphatidylcholine acyltransferase (MGAT) and acyl-CoA:1,2-diacylglycerol acyltransferase (DGAT). Results showed that after a 4-wk feeding period, ACAT, ACLAT and DGAT basal activities were significantly enhanced by the dietary fatty acids of the (n-3) series, whereas MGAT activity was not affected. When the highest (n-6):(n-3) ratio (51.0) was compared with the lowest (0.8), the increase attained was about 58, 76 and 73% for ACAT, ACLAT and DGAT, respectively. Fatty acid composition of microsomal lipids was drastically altered by the diets because (n-3) PUFA replaced the (n-6) series as the dietary (n-6):(n-3) ratio was lowered. These compositional changes could explain the observed modification in the membrane-bound enzyme activities. We suggest that (n-3) PUFA ingestion leads to an enhanced velocity of chylomicron synthesis in rats.

Acyltransferases

[Surveillance of corrected transpositions by the Mustard or Senning operation].

One hundred eighteen patients who survived Mustard immunités (n = 103) or Senning operations (n = 15) performed between 1972 and 1984 for isolated transposition of the main arteries were followed-up for over a mean period of 3.67 yr (up to 12 yr). The authors have examined the potential complications which can alter the long-term prognosis in this type of atrial correction: heart rhythm disorders (regression of sinus rhythm of 3.82% yr), mechanical complications, haemodynamic dysfunction. After atrial correction the survival curve underwent an actuarial regression of 1.3% yr. All these potential complications should be detected by regular survey and may indicate anatomical correction, the results of which have yet to be fully evaluated.

Arrhythmias, Cardiac

[Varicella myocarditis and junctional ectopic tachycardia].

We report the unexpected occurrence of a lethal arrythmia in an 11 month-old boy who presented varicella. The disorder was found to be a junctional ectopic tachycardia due to a zosterian myocarditis confirmed at autopsy. This exceptional association of varicella, myocarditis, and junctional ectopic tachycardia gives us the opportunity to discuss on the diagnosis and treatment of this uncommon and serious arrythmia (regular tachycardia with normal QRS morphology, ranging 200-250 per min, with atrioventricular dissociation or retrograde conduction to the atria).

Chickenpox

[Cardiopathies in Di George's syndrome].

Based on 11 personal cases a large review of the literature (188 cases), the authors studied the frequency and types of cardiac anomalies in Di George syndrome. A cardiac anomaly was found in 86% of published cases. Interrupted aortic arch (27%), truncus arteriosus (27%) and tetralogy of Fallot (22%) are the most frequent anomalies. Interrupted aortic arch and truncus arteriosus, being rare congenital heart diseases, their discovery in a neonate must lead to a systematic search for Di George syndrome.

DiGeorge Syndrome

Serum immunoreactive erythropoietin level: a new parameter for monitoring transfusion management of thalassaemia.

Serum immunoreactive erythropoietin (EPO) was measured sequentially in 8 patients with thalassaemia major. The EPO levels were distinctly increased before transfusion; they did not significantly change just after transfusion, but subsequently decreased. Pretransfusion EPO levels were similar to those observed in patients with non renal non inflammatory anaemias of the same severity. Our results show that production of EPO is appropriately increased in thalassaemia major and confirm that stimulation of endogenous erythropoiesis is not completely suppressed by the hypertransfusion regimen commonly used in this disease. As hyperstimulated ineffective erythropoiesis may lead to invalidating complications, we suggest that serum EPO measurements, which are now easily and reliably performed, should be used in monitoring the therapy of thalassaemia major.

Adolescent

[Surgery without catheterization of aortic coarctation in newborn infants and infants under 3 years of age].

A retrospective study of 150 children under 3 months of age who underwent repair of coarctation of the aorta in the same center (between 1972 and 1987) was undertaken to assess the problems posed by surgery without cardiac catheterisation or angiography. The patients were divided into two groups: Group A, comprising 104 children operated after invasive investigations, and Group B, comprising 46 children operated on Doppler echocardiographic data alone. The two populations were comparable and "hypoplastic aortic arch" type coarctation was present in over 60% of cases. However, there were more ventricular septal defects in Group B (67.5%) than in Group A (49%). There were no significant diagnostic errors in Group B (one case of interrupted aortic arch diagnosed at surgery). The 1 month survival was the same in the two groups (82%). The indications of pulmonary artery banding were less frequent in Group B although there were more ventricular septal defects in these patients. These results confirm the value of Doppler echocardiography in the context of urgent surgery of congenital heart disease.

Angiocardiography

[Idiopathic dilated cardiomyopathies in children. Development and prognostic factors].

This review is a retrospective study of the cases of 103 patients under 20 years of age with idiopathic dilated cardiomyopathy and represents the whole experience of the Department of Paediatric Cardiology of the Hospital of Cardiovascular Disease, Lyon, between January 1970 and December 1988. The aetiological investigations were constantly negative: hypertrophic, restrictive and secondary cardiomyopathies were excluded. The clinical, radiological, electrocardiographic and echocardiographic data of each patient was noted at the time of admission and at the end of the clinical course. There were many more infants in this series. The onset of the disease was usually sudden. All patients were given medical treatment. The study period ranged over 19 years. Forty one patients (39.8%) died mainly of terminal cardiac failure. Sixty two patients are still alive, of whom 39 are completely cured, 2 have undergone cardiac transplantation, and 21 have a variable degree of chronic invalidity. The six months survival was 77.5%; the 1 year survival was 70% and the 5 year survival was 60%. Over one third of deaths (36.6%) occurred during the first month, 56% during the first 3 months and 70% during the first year. A statistical analysis comparing the initial criteria to death by the chi 2 test revealed the following prognostic factors: age at presentation (32.9% of deaths in patients under 2 years of age compared to 56.6% in the group over 2 years of age, p less than 0.025) and a family history of myocardial disease. The other clinical, radiological and echocardiographic parameters had no predictive value in this series and it was not possible to identify potential candidates for cardiac transplantation.

Adolescent

[In vivo and in vitro study of the action of dopamine on oocyte growth and juvenile hormone production in Blattella germanica (L.) (Dictyoptera; Blattellidae)].

In vivo, within the first ovarian cycle of B. germanica, dopamine (1 microgram/cockroach) enhanced oocyte growth when injected on day 1 or 2, just before vitellogenesis, whereas it induced the inverse effect on day 6-7, at the end of vitellogenesis. In vitro, dopamine (10(-4) M) stimulates juvenile hormone production by incubated corpora allata from 2-day-old females, and inhibited it on 6-day-old glands. The physiological significance of these effects is discussed.

Animals

[Dilatation of the cerebral ventricles diagnosed in utero. 85 case reports].

This study concerns a retrospective analysis of 85 case histories of pre-natal dilatations of the ventricles considered by a multi-disciplinary group whose aim it was to try and identify the most favourable features for prognosis that could be found in the whole of this pathological condition. The prognosis for ventricular dilatations found in utero is very poor. Only 36 of the 85 children were born alive. 16 of those died before they were two months old and of the survivors only 4 children developed normally. The least unfavourable elements that were found seem to be solitary areas of dilatation or the association of the dilatation with agenesis of the corpus callosum, late diagnosis, slow evolution and a ventricular-hemisphere ratio no more than 50% of the normal value. Early diagnosis should improve the methods of treating these patients. In order to achieve this, an ultrasound of the skull should be carried out in the fourth month and in the 26th week of pregnancy. Precise diagnosis must be made. A attempt should be made to work out the aetiology as completely as possible as well as to watch carefully how the condition is evolving. When death has occurred, the assessment should be compared with the autopsy results. Finally, the knowledge about the outcome of all these children should make it possible to criticize the decisions that have been taken for management.

Female