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Biomedical subjects

J Normand

Publications and source records attributed to J Normand.

At least 37 records · Page 2Linked to original sources
PubMed1992

[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↗
PubMed1992

[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↗
PubMed1991

Binding of ubiquitin to experimentally induced murine AA amyloid.

Amyloid enhancing factor (AEF) activity has recently been demonstrated in ubiquitin purified from amyloidotic murine tissues and Alzheimer brain extract. Since AEF is known to bind to amyloid fibrils and 'fibril-AEF' on passive transfer induces accelerated amyloidogenesis in the recipient animals, it was of interest to investigate whether ubiquitin binds to amyloid. Immunohistological studies were carried out on liver sections from amyloidotic mice. Biotin-strepavidin-peroxidase methods using monospecific rabbit anti-mouse AA amyloid IgG (RAAG) and rabbit anti-bovine ubiquitin IgG (RABU) antibodies were employed to immunostain the amyloid and ubiquitin deposits, respectively. RABU-treated liver sections were counterstained with thioflavine S. RAAG reacted strongly with the amyloid, indicating that it is AA type, and RABU-positive immunodeposits were found bound to the thioflavine-S-positive AA deposits. Treatment of the liver sections with 0.1 M sodium acetate containing 0.5 M NaCl, pH 4, for 2-3 h at 37 degrees C nearly completely desorbed the AA amyloid-bound ubiquitin. Since ubiquitin demonstrates AEF activity in vivo and binds non-covalently to AA amyloid, we suggest that ubiquitin may indeed be 'fibril-AEF' and may play a crucial role in the pathogenesis of amyloidosis. To our knowledge, this is the first time that ubiquitin bound to extracellularly deposited amyloid has been demonstrated.

Animals↗
PubMed1991

Alzheimer's disease brain-derived ubiquitin has amyloid-enhancing factor activity: behavior of ubiquitin during accelerated amyloidogenesis.

Amyloid-enhancing factor (AEF) is believed to be a crucial common pathogenetic link in diverse forms of human amyloidosis. Passive transfer of crude AEF is known to trigger accelerated splenic amyloid deposition in mice. We have recently identified AEF activity in ubiquitin isolated from murine amyloidotic tissues. Using similar techniques we have purified ubiquitin, from crude Alzheimer's disease (AD) brain extracts, to apparent homogeneity. Based on the partial amino acid sequence homology, immunochemical and pathophysiological criteria, the approximately 5.5-kDa AD-derived protein was identified as ubiquitin (AD-ubiquitin) with AEF activity. Ten to twenty micrograms of this protein per mouse, with or without CaCl2, in conjunction with four subcutaneous injections of 0.5 ml of 1% aqueous AgNO3, induced accelerated splenic amyloid deposition. By immunohistochemistry, using anti-mouse AA amyloid antibody, the AD-ubiquitin-induced amyloid was identified as AA type. With anti-bovine ubiquitin antibody, using similar spleen sections as above, ubiquitin was found to co-deposit with AA amyloid in the splenic perifollicular areas. These results strongly suggest that ubiquitin may be involved in the pathogenesis of amyloidosis.

Aged↗
PubMed1991

Prolonged QT, atrioventricular block, and sudden death in the newborn: an electrophysiologic evaluation.

An electrophysiologic study was performed in the first month of life in a patient with the congenital long QT syndrome and spontaneous episodes of 2/1 atrioventricular block. The block could be reproduced by incremental atrial pacing, and its infrahisian location was associated with a prolongation of the refractoriness in the ventricular muscle itself. Surprisingly, intravenous propranolol aggravated this phenomenon by further prolonging the QT interval. Sudden death occurred shortly thereafter during Holter monitoring and was due to a sudden resumption of normal AV conduction after an episode of 2:1 block, immediately followed by ventricular fibrillation.

Cardiac Pacing, Artificial↗
PubMed1991

[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↗
PubMed1991

[Treatment of percutaneous angioplasty of restenoses of coarctation and stenosis of bioprostheses in children].

The authors discuss the percutaneous balloon dilatation procedure and the results obtained in 10 children (age range: 2.5-13 years) over a 2 year period (April 1986-April 1988). Six cases of coarctation restenosis were observed and good clinical and hemodynamic results were noted in 2 cases, while in 4 cases the procedure was not totally successful. For the 4 cases of stenosis of bioprosthetic valves in right ventricular-to-pulmonary arterial conduits, good results were obtained with a significant reduction of the trans-stenotic gradient from 50 to 23 mmHg. While long-term effectiveness of the procedure is still uncertain, those encouraging preliminary results suggest that this procedure could be considered as an alternative treatment for coarctation restenosis and stenosis of bioprosthetic valves in right ventricular to pulmonary artery conduits.

Adolescent↗
PubMed1991

[Treatment of congenital pulmonary valve stenosis by percutaneous valvuloplasty].

The purpose of this study was to evaluate the efficacy technique and follow-up results of balloon valvuloplasty for congenital valvular pulmonary stenosis. Percutaneous dilatation was performed in 48 patients aged 0.5 to 21 years (m = 7.5 yrs) from two pediatric cardiology centers (Lyon and Montreal). The right ventricular peak systolic pressure ranged from 42 to 180 mmHg (m = 93) before dilatation and fell from 24 to 105 mmHg (m = 48) immediately after dilatation. The pulmonary valvular peak systolic pressure gradient ranged from 22 to 156 mmHg (m = 73) and fell to 4 to 70 mmHg (m = 27) after the procedure (P less than 0.001). Follow-up continuous doppler data was available from 33 patients at 1 to 37 months (m = 9.2 months) after dilatation. The maximal instantaneous gradient from right ventricle pulmonary artery ranged from 0 to 74 mmHg (m = 27).

Adolescent↗
PubMed1991

[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↗
PubMed1991

[Results of the surgical treatment of critical aortic valve stenosis in the newborn infant].

Retrospective studies have been conducted in Lyon (33 patients) and Montreal (24 patients) in order to compare the results of transventricular valvotomy (TVV, 20 cases) and aortic valvotomy with cardiopulmonary bypass (CPB, 37 cases) in neonatal critical aortic stenosis. Clinical, echocardiographic, catheterization and operative data were analyzed in order to determine prognostic factors. Mortality rate was 59%: 30/34 perioperative deaths in the first month, and 4 late deaths after a reintervention for severe residual obstruction. Long term follow up was available for 23 patients (41%) for a 2 to 16 year period (mean 7.5). Five patients (7%) required a reintervention six years after the initial operation. Two of them required valve replacement. Eighteen patients (31%) surviving the initial operation, displayed a satisfactory result, being free of symptoms, endocarditis, reoperation and sudden death. Factors that influenced the outcome included severe heart failure, a left ventricular end-diastolic diameter below 14 mm, an aortic valve annulus below 8 mm, and a poor shortening fraction. Factors that did not influence the prognosis were age, pulmonary hypertension, and the anatomic type of the valve. Trans-aortic valvotomy with CPB was associated with a smaller operative mortality and a better long term result than TVV.

Aortic Valve↗
PubMed1991

[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↗
PubMed1991

[Phrenic stimulation in respiratory paralysis caused by spinal cord injuries].

Phrenic stimulation is reserved for the rare patients with central neurogenic apnea due to isolated upper spinal cord injuries. Presenting a series of ten cases the authors describe the indications for phrenic stimulation, the technique of implantation and the conditions for diaphragmatic training by the use of stimulation by low frequency trains allowing for an increase in the number of low muscular fibers. The continuation of accompanying neurological rehabilitation and nursing of these severely handicapped patients is not to be neglected because of the length of the training protocole as the aim is their return home. This technique, therefore, seems particularly suitable for application in a few specialized centers where research for material totally implantable and adapted to the physiological requirements of the patient can be conducted parallel to treatment.

Adolescent↗
PubMed1991

[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↗
PubMed1991

[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↗
PubMed1990

An evaluation of preemployment drug testing.

As part of a blind longitudinal study, 5,465 job applicants were tested for use of illicit drugs, and the relationships between these drug-test results and absenteeism, turnover, injuries, and accidents on the job were evaluated. After an average 1.3 years of employment, employees who had tested positive for illicit drugs had an absenteeism rate 59.3% higher than employees who had tested negative (6.63% vs. 4.16% of scheduled work hours, respectively). Employees who had tested positive also had a 47% higher rate of involuntary turnover than employees who had tested negative (15.41% vs. 10.51%, respectively). No significant associations were detected between drug-test results and measures of injury and accident occurrence. The practical implications of these results, in terms of economic utility and prediction errors, are discussed.

Adult↗
PubMed1990

[Total anomalous pulmonary venous connection. Results of repair in 50 infants].

Fifty infants with isolated total anomalous pulmonary venous connection (TAPVC) were operated between 1/01/73 and 31/12/87. The average weight at surgery, which was performed under hypothermia with circulatory arrest in 92 per cent of cases, was 4.5 Kg. The preoperative pulmonary to systemic pressure ratios (PAP/PS) enabled identification of two groups of patients: Group I: TAPVC without severe pulmonary hypertension (PAP/PS less than 0.85) (n = 35), and Group II: TAPVC with severe pulmonary hypertension (PAP/PS greater than 0.55) (n = 15). The hospital mortality was 22 per cent (8 cases) in Group I compared with 73 per cent (11 cases) in Group II (p less than 0.05). Patients in Group II were younger (64 days compared with 137 days, p less than 0.02), lighter (p less than 0.05) and had preoperative mean pulmonary artery systolic pressures of 83 mmHg (p greater than 0.001). Three patients in Group I required early reoperation for stenosis of the pulmonary veins at the site of repair resulting in pulmonary hypertension, and all died. The global survival was 28 patients with an average follow-up of 7 years (range 1 to 15 years). Six of these patients were reoperated (2 phrenoplications, 4 atrial shunts). All survivors are asymptomatic and have no conduction defects. Control echocardiography in 15 of the 28 survivors was judged to be normal. These results show that obstructive forms of TAPVC (Group II) carry a very poor prognosis: immediate results in this group could only be improved by earlier surgery. The clinical long-term results in those who survive surgery are very satisfactory.

Age Factors↗