[Sickle cell anemia and the heart].
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Biomedical subjects
Publications and source records attributed to E Bertrand.
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The here presented case was a woman 82 years old suffering for many years of arteriosclerotic Parkinson syndrome. During the last 8 months of her life she exhibited excessive talk flow and states of delirium. Twelve days before death she had a generalized attack of convulsions and the consciousness disorder increased. Moreover, meningeal symptoms were observed, a right side reflex syndrome with Babiński sign and temperature up to 38 degrees C were noted. Death occurred on the 9th day of hospitalization with symptoms of increasing circulatory-respiratory insufficiency. Pathomorphological examination revealed in the myocardium, lungs, liver, kidneys and thyroid multiple purulent foci containing actinomycotic granules. Neuropathological examination revealed both in the cortex and white matter the presence of numerous disseminate bodies staining intensively with HE, PAS- and Gram-Weigert positive and negative in the Ziehl-Nielsen method. Their structure, compact or fine-powdered corresponded to actinomyces colonies. They either showed no reaction or were surrounded by a wall of inflammatory cells such as: neutrophilic leucocytes, lymphocytes, plasmatic cells and histiocytes. The microabscesses were not encapsulated. The case was classified to a diffuse form of brain actinomycosis. The etiopathogenesis of the changes and the clinical-morphological correlations of this very rare infectious disease of the central nervous system are discussed.
Clinical-morphological analysis of cases in which, in the course of vasogenic necrotic brain lesion, hemorrhagic foci appeared within the infarcted area, was performed. The material comprised 355 cases with encephalomalacia (223 women and 123 men). The patients died at age between 39 and 96 years. In the clinical picture a sudden onset of the disease prevailed, with hemiparesis or hemiplegia associated in 216 cases with disturbances of consciousness. The course of the disease was in general progressive. Only in 30 patients improvement of clinical state was observed. The time of survival varied from several hours to several weeks. Most patients (140) died in the first week in deep cerebral coma or state of decerebration. In nine cases clinical symptomatology of herniation and secondary hemorrhages into the brain stem was found. A sudden onset and violent course of the disease resulted in a diagnosis of primary hemorrhage with penetration to the subarachnoid space or ventricular system in 32 patients. Only in six cases was a secondary hemorrhage into the primary infarction diagnosed clinically. Hemorrhagic malacia was most frequently (in 255 cases) located in region vascularized by the middle cerebral artery and very seldom, the basilar artery or cerebellar arteries. Malacia was accompanied by considerable brain edema. This was particularly intense in extensive infarctions and persisted even in cases with considerable long survival. In 45 cases secondary hemorrhagic foci were found within brain stem. The morphology of the infarction foci was variable in most cases. Independently to fresh unreactive diffuse necrosis, sometimes areas with various advancement of resorption and organization processes were observed in the same case. Both in areas of fresh reactionless malacia and of advanced macrophage-glial disintegration, or even in areas of advanced gliomesodermal organization, stand-like or spherical hemorrhages could be seen merging sometimes into extensive pools. The morphology of hemorrhagic foci was largely dependent on their size and localization. The hemorrhagic foci located close to the depth of the cortical sulci very often destroyed the cortex and white matter, and penetrated to the subarachnoid space (141 cases). In was usually almost impossible to detect the source of bleeding within the necrotically changed area. The resorption reaction at the edge of extensive hemorrhagic foci was in general minimal. A lesion of the vessel wall, caused by stasis of long duration, resulting from disorders of the vascular function in the course of defficient blood supply and ischemic brain edema are considered the main causes of bleeding into the pale infarction.
From 22 patients with left chronic parietal endocarditis (CPE), conducted an epidemiological, clinical, paraclinical and per-operative study. The CPE is frequently encountered in the Ivory Coast in Akan children living in forest areas in 81 p. cent of the cases. It causes a mitral insufficiency, of the which the essential mechanism is fibrosis of the sub-valvular apparatus. The clinical examination is that of a severe advanced mitral insufficiency. Cardiomegaly is the rule. In 31 p. cent of cases, the ECG shows a Qr in V1. The dip plateau on the apexocardiogram is never found and the protodiastolic endocardial resonance is inconstant on phonomechanical processor. The aspect in M of the interventricular septum, found in 72 p. cent of the cases on echo TM is of major importance when direct signs are missing on bidimensional sonograms. Angiocardiography remains the reference diagnostic examination. But only the surgical survey enables to demonstrate limited forms.
A standardized Flack's test with continuous electrocardiographic recording was performed in 351 moderately trained athletes (317 men and 34 women; mean age 23.7 years). The test gave normal results in 310 subjects (88.3%). Tachycardia in excess of 100 beats/min was observed in 12 subjects (3.4%), and 56 abnormalities which were not present on previous ECG recordings at rest were noted in 29 subjects (8.3%); 27 of these abnormalities appeared immediately after a previous one. Out of these 56 abnormalities, 26 (44.6%) were associated with sinus dysfunction: sinus bradycardia in 8 cases, second degree sinoatrial block in 2 cases, third degree sinoatrial block in 3 cases and sinus arrest in 13 cases. In 16 cases of third degree sinoatrial block or sinus arrest escape rhythms were observed, including coronary sinus rhythm (3), junctional rhythm (12) and accelerated idioventricular rhythm (1). Five of the 56 abnormalities (8.9%) were disorders of conduction, i.e. first or second degree atrioventricular block, atrioventricular dissociation and blocked P waves. These disorders are known to be frequently associated with sinus dysfunction. In 9 out of 56 abnormalities (16.1%) disorders of excitability were present as atrial, junctional and ventricular extrasystoles. In 8 subjects the Flack's test resulted in the disappearance of disorders of conduction or excitability of the "athlete" type present at rest, as the Ruffier-Dickinson test, or the cycloergometric exercise test.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors have studied 50 Ivorians and 50 Europeans people, all living in Abidjan for at least 10 years. Platelet aggregability with increasing ADP concentration (0.6, 1.2, 2.4 mumoles/l), collagen (0.4 mg/l), or ristocetin (1 g/l) was examined. Fibrinolysis and the euglobulin test were also studied before and after anoxia. Other blood parameters measured were: hematocrit, hemoglobin level, platelet count, bleeding time, Howell coagulation test, cephalin tests, prothrombin activity ratio, fibrinogen level. Metabolic tests included: glycemia, cholesterolemia, triglyceridemia, uricemia, A1 and B apoproteins, protidemia, gamma globulinemia. Environmental factors such as physical activity, alcohol and smoking habits, fish consumption, chloroquine prophylaxis were evaluated. The most evident result was lower platelet aggregability in Ivorian people as compared to Europeans. A more precocious and important fibrinolysis activity, either spontaneous or after anoxia was noted in the Ivorian group. Lower platelet number, fibrinogen level, and prothrombin activity were present in the Ivorian group as compared to the European people. The authors eliminated the influence of age, and considered environmental factors as predominant in the genesis of such difference, i.e., hypocholesterolemia, lower smoking and drinking levels. They emphasized the higher fish consumption in Ivorian people.
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The authors have studied three groups of patients: 12 reference patients (REF) leading a sedentary life; 22 athletes of international fame, practising an endurance sport (END), including 10 long distance and middle-distance runners, 12 cyclists and 12 top (RES) athletes practising a resistance sport i.e. 12 sprinters. There is no age difference between the two groups. The RES have a larger body area than the REF and END. The cardiac frequency is lowered in the athletes without any difference between RES and END. There is no difference as far as the athletes' blood pressure is concerned. On the EKG, the Sokoloff index is significantly higher in END than in REF and RES. Sonographic data were especially analyzed. END present a hypertrophic myocardium and ventricular dilatation. The hypertrophy, more assymmetrical than that of RES, is more marked at the level of the septum. There is a non-significant tendency toward an excentric hypertrophy. RES present a marked hypertrophy with little or no dilatation. This hypertrophy is more symmetrical and concentric than in END patients. As far as the pump function is concerned (FR, FE, VES): it is not altered in RES (this fact is found in the literature). On the contrary, in END patients, the pump function (FR, FE) is diminished (this fact is debated in the literature), in spite of an increased systolic ejection volume. The total contraction function is not altered in RES nor in END patients. An increased septal contraction rate is noted, which is significant in RES patients and non-significant in END patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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Silent coronary heart disease (CHD) has been researched among a sample of 50 black African diabetic patients in a prospective study. The diabetic patients were chosen on the following criteria: male sex, age ranging from 40 to 60 years, absence of history of CHD, normal EKG. A stress test was performed in all cases. A coronary arteriography was done each time the exercise test was positive, doubtful or non-conclusive. This study has shown a prevalence of 10% CHD:31 exercise test negatives, 17 exercise test non-conclusives, 2 exercise test positives, 5 coronary arteriography with significant narrowing. There were 3 patients with one vessel disease (LDA: 2 cases, CFX: 1 case), one patient with double vessel disease (CFX, RCA) while another had a triple vessel disease (RCA, LDA, CFX). Proximal lesions were encountered in 5 cases and distal lesions in 3 cases. The authors conclude that CHD exist in a silent state among black African diabetics. Its prevalence is lower than in white diabetics. The coronary lesions are mostly limited. Proximal narrowing and one vessel disease were mostly encountered.
Twenty-six patients with infective endocarditis were operated upon during the active phase. The endocarditis was native in 24 cases and developed on cardiac valve prosthesis in 2 cases. Depending on the valve involved, the patients were divided into 3 groups: Ao (aortic valve, n = 13), M (mitral valve, n = 10) and T (tricuspid valve, n = 3). The overall mortality rate was 26% (group Ao 20%, group M 20%); death was due, in most cases, to haemodynamic failure. The duration of pre-operative antibiotic therapy, the functional stage of the disease and the cardiothoracic ratio had no influence on post-operative prognosis. In contrast, the presence of vegetations (notably on the aortic valve) at echocardiography and the pumping and aortic clamping times played a role in operative mortality. Twelve patients were followed up for a mean period of 23.9 months. They are all in stage I or II with significant decrease in cardiothoracic index. In Africa, where bacteriological facilities are often inadequate and cardiac valve diseases are diagnosed at a late stage, infective endocarditis is active in many cases. Under these conditions, early surgery is justified when heart failure is present and the infection is not clinically controlled.
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The authors reviewed the operative and function results of 24 isolated tricuspid valve replacements with bioprostheses in 22 patients. The patient population was young (average age 15 +/- 8 years). The surgical indication was massive tricuspid regurgitation due to chronic parietal endocarditis in 19 cases and to bacterial endocarditis in 3 cases. Tricuspid valve replacement was associated with 19 right ventricular endocardectomies, 2 direct closures of ventricular septal defects, 2 Wooler mitral valvuloplasties and 1 pericardectomy. The operative mortality was 13.5% and the secondary mortality 13.5%. Of the 16 survivors, 13 are in the NYHA Class I with no regular medical therapy. Their cardiothoracic ratio has slightly decreased. Two patients have permanent atrial fibrillation, and 12 have acquired definitive complete right bundle branch block. Eight of these patients had significant improvement of atrial and right ventricular pressures, of Yu's index and cardiac index at postoperative catheterisation. Three of the 16 patients developed progressive calcific degeneration of their bioprostheses. They are among the 6 patients who have been followed up for more than 3 years. There was no mortality at reoperation. Isolated tricuspid valve replacement by bioprosthesis was chosen despite the young age of these patients because of the disadvantages of mechanical prostheses which are associated with a much higher mortality related to incarceration and thrombosis of the prosthesis. The relatively high operative and secondary mortality in this series of isolated tricuspid valve replacement compared to mitral, aortic or micro-aortic valve replacement, is related to the gravity of the underlying causal pathology.
A case of pulmonary valve endocarditis in a patient with patent ductus arteriosus is reported. The diagnosis was made by echocardiographic examination. The M mode echocardiogram showed "shaggy" echoes on the pulmonic valve and the two dimensional echocardiogram disclosed a big vegetation in the right outflow tract. The patient underwent surgery with complete cardiopulmonary bypass. The ductus arteriosus was closed by endopulmonary approach and the pulmonic vegetation was removed.
Some particular features of the cardiomyopathies (CM) observed in the tropics, especially in Africa, are emphasized in this study. Chronic parietal endocarditis is excluded from the CM group. The author presents facts that justify the linking of that affection to endocardial diseases. Myocardiopathies are acute ailments presenting with congestive lesions, reversible under etiological therapy. Anemic and beri-beri myocardiopathies are not unusual in the tropics and present a hyperkinetic syndrome before the stage of advanced cardiac insufficiency. Infectious or parasitic myocarditis seem frequent in the tropics. The author recalls the characteristics of the myocarditis in the human african trypanosomiasis which he opposes, particularly, to the american trypanosomiasis. The reality of bilharzial myocarditis is more debatable while bilharzial pulmonary hypertension is well documented. Chronic congestive CM presents a few specific characteristics in the tropics. The features, well described in temperate regions, are found in the tropics with a particularly unfortunate prognosis. Some alcoholic myocardiopathies have been observed. The rare occurrence of hypertrophic CM in the tropics results, seemingly, from a lack of exploratory means. The author studies briefly a recent series of 31 cases in Abidjan. Post-partum myocardiopathy seems to be the clinical appearance of a latent myocardial insufficiency of the normal post-partum in women presenting with associated risks factors (anemia, malnutrition, overwork, excessive sodium intake, etc.). An early diagnosis enables a cure only by resting, but it is sometimes necessary to associate a medical treatment. Death by embolism or the passing to chronicity are however possible. Drepanocytic CM is debatable and in many cases, seems hardly differentiated from anemic myocardiopathy.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors report two observations of concentric hypertrophic cardiomyopathy associated with Fallot's tetralogy in one case and with pulmonary atresia complicated by atrial septal defect in the other. The association of Fallot's tetralogy with hypertrophic cardiomyopathy is rare, as only 5 cases have been reported in the literature. In this connection, the mechanism of myocardial hypertrophy and the therapeutic problems posed by this association are discussed.
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