Coronary lesions in young HIV-positive subjects at necropsy.
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Biomedical subjects
Publications and source records attributed to R Loire.
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Twenty anatomico-clinical cases of chronic cardiac rejection (accelerated coronary disease in heart transplant) consecutive to heart transplantation were studied with the view of obtaining detailed information on the anatomical features of coronary lesions, such as histopathological alterations, modalities of their diffusion to the 3 epicardial trunks and to distal intramyocardial branches, thrombotic complications and their consequences: massive (infarct) or disseminated myocardial ischaemia. The lesions observed were correlated with the corresponding coronary angiographic images, and an interpretation of the aetiopathological factors was attempted. Within a few months or years, the coronary lesions are found to progress towards very diffuse circumferential atherosclerous alterations where the plaques are clearly less individualized than in common atherosclerosis but thrombosis is frequent and multifocal in 50 percent of the cases. This produces a restrictive type of ischaemic cardiopathy which is painless since the heart is denervated, resulting in cardiac failure (11 cardiectomies for retransplantation, 9 autopsies) with coronary angiography tending to underestimate the importance of coronary damage. The most original aetiopathological factors seem to be arterial inflammation of immune origin, viral infections facilitated by immunosuppression and platelet hyperactivity, but their respective importance could not be accurately determined in this study.
Lymphangiomyomatosis (LAM) consists of smooth muscle (SM) cell proliferation of unknown origin involving the lymph nodes and the lung interstitium. From morphological studies showing both SM differentiation of the proliferating cells and lymphatic hyperplasia, hypotheses were suggested concerning the origin of the proliferation. Two cases of LAM were investigated by electron microscopy and immunohistochemistry; tissues were obtained by lymph node and open lung biopsies. Cytoplasmic and matrix protein markers were used in order to clarify the pattern of differentiation of the proliferating cells and to characterize their connective tissue environment. The proliferating cells present ultrastructural characteristics of SM cells; they contain vimentin, desmin, and alpha-SM actin and are devoid of Factor VIII, favouring a parieto-arterial origin. The connective tissue matrix inside the infiltrate is composed of interstitial collagens and basement membrane components. At the late stage of the disease, remodelling of the interstitial matrix accompanies the infiltrate and remains perilesional.
Over the period 1983-1990, 10 cases of infective native-valve endocarditis as a result of Chlamydia were seen. All patients were men, with a mean age of 42 years, and none had a history of exposure to Chlamydia psittaci. Symptoms, such as weight loss and anorexia, with fever in eight cases, had persisted for at least 2 months before admission. Haemodynamic failure was present in seven patients, and neurological signs in four. The aortic valve was involved in seven cases, the mitral valve in one and both valves in two. Vegetations, often fingerlike, were observed by echocardiography in nine cases. All patients required valve replacement, and three died in the year following diagnosis. Blood cultures were consistently negative in all cases, and no antibiotics had been given before admission. Significant titres of complement fixing anti-chlamydial antibodies were present in six cases, and micro-immunofluorescence using the three chlamydial species showed cross-reacting antibodies in all nine cases studied, with transient IgM positivity in six cases. Histologically, the leaflets were fibrosed and infiltrated by large macrophages containing dark red granules after Machiavello staining. These granules were specifically stained by immunofluorescence using monoclonal antibody to Chlamydia common antigen, but not by that specific for C. pneumoniae. No organisms were seen after Gram staining, and no positive chlamydial immunofluorescence was seen on sections of valves from patients with staphylococcal or streptococcal endocarditis.
Thoracic splenosis (post-traumatic autotransplantation of splenic tissue) is rare and generally asymptomatic. We report a patient with thoracic splenosis presenting with repeated hemoptysis. The blood supply of the hypervascular splenic transplants originated from a bronchial and an intercostal artery. Hemoptysis improved after surgical exeresis of splenosis. Recognizing splenosis presenting with hemoptysis is important, since percutaneous embolotherapy could be hazardous because of the risk of ectopic splenic tissue infarction.
Accelerated atherosclerosis of cardiac grafts is one of the factors limiting long-term survival after cardiac transplantation. The authors report the case of a patient who had a cardiac arrest associated with severe atherosclerosis 18 months after transplantation. The severity of the coronary lesions was underestimated by coronary angiography. An ergometrine test induced coronary spasm, a phenomenon which has only rarely been observed in transplanted hearts. The patient died one month later despite calcium inhibitor therapy. Autopsy revealed very severe triple vessel disease. This case illustrates the possible rapid evolution of coronary artery disease in cardiac transplant recipients, the difficulty in evaluating the severity of the lesions by coronary angiography and the additional possibility of observing coronary spasm in these cases.
Polyaneurysmal dystrophy is a novel form of arteriopathy which specific clinical, angiographic, anatomic and surgical features which distinguish it clearly from multiple atheromasclerotic aneurysm. It should be considered to be a local, multifocal accentuation of megadolicho-arteries, which constitute the lesions during the early stages of the disorder (fairly general elongation of the elastic arteries, with thin walls and regular increase in the caliber and multiple tortuousness). Arterial angiography identifies polyaneurysmal dystrophy; in the context of a twisted and sinuous system of large arteries, multiple spindle-shaped aneurysms can be distinguished which are frequently bilateral and symmetrical. The usual sites are the trunks of the aortic group and internal carotid, the ileo-femoral trunks and terminal aorta. The progress of the disorder is characterized by the possibility of rupture or thrombosis (particularly in the subcrural territory). The treatment is always surgical. The indication for surgery is inevitable in cases of severe ectasia, but may be avoidable in extensive forms of megadolicho-arteries with no clearly defined aneurysm: annual ultrasound monitoring is then called for. The disorder is of constitutional origin (and totally unrelated to atherosclerosis). Delayed dilatation of the aneurysms is due to the hemodynamic forces brought to bear on the fragile walls over a life-time. Multiple aneurysms occur mainly between the ages of 50 and 70 years, with a predominance of aorto-ileac sites in men, even though these subjects do not show any general elastic dysplasia. Half of the 45 patient undergoing surgery were hypertensive.
The authors present the pathologist's view of Uhl's anomaly based on 100 cases recorded over 31 years, 89 of which came from medico-legal autopsies following sudden unexpected death in young subjects. The anatomic diagnosis is made by macroscopic examination of the whole heart in diffuse forms (parchment heart) and from transverse sections of the mid third of the ventricle in incomplete forms, showing absence of myocardium in the juxta-septal anterior wall of the right ventricle. The wall entirely made up of adipose tissue, may retain its normal thickness. On microscopy, normal adipose tissue is observed between the epicardium and endocardium, sometimes with islets of myocardium dispersed in the subendocardial layer and with, in about half the cases, subendocardial bundles of non-hyalinized collagen without any inflammatory cellular infiltration. Other structural histopathological lesions may complicate the malformation, involving the intraseptal arterioles or the conductive system. In addition to the 89 cases of sudden and unexpected death before the age of 50 (preceded by some modification of the patient's life style in 29 cases), 11 cases were symptomatic and 5 were transplanted with a good result. The clinical diagnosis of Uhl's disease is important because of its consequences: ventricular tachycardia and possible sudden death, more rarely cardiac failure with age. Histological analysis should take into account the constant presence of adipose tissue in the right ventricle, and should only interpret excessive quantities with associated myocytic aplasia as pathological.(ABSTRACT TRUNCATED AT 250 WORDS)
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The authors report two new cases of spontaneous dissection of the coronary arteries in women under 50 years of age without any predisposing factors and responsible for sudden death. One patient had dissection of the left anterior descending artery and the other had dissection of the left anterior descending and right coronary arteries. Special histological stains allow demonstration of abnormalities of the elastic tissue of the media in both cases. In the first case, a plaque of atheroma was present opposite the site of dissection but did not seem to be causal. In the second case, severe periadventitial cellular, infiltration of unknown origin was observed. This pathology of the young woman, often occurring in the postpartum period, is as rare as it is unpredictable.
We report the results of a morphological analysis of 60 pulmonary biopsies gathered from a multi center study, organised by the clinico-pathological research group on Wegener's Disease under the auspices of the French Language Society of Thoracic Medicine. Forty of the sixty cases analysed were retained after indexing the histological aspects in order to specify their diagnostic value. Two groups of lesions were distinguished, which had different significance. Group A: These include the three major diagnostic criteria, which reinforce one another as they associate: 1) The polymorphoneutrophil microabscesses with limited central necrosis or an extended necrosis like the contours of a relief map. 2) An angiitis (arteries, veins, capillaries) with eccentric focal parietal crescent-shaped microabscesses. 3) Polymorphous granulomas with giant cells. Group B: In this group are the minor morphological observations (table II) of a lesser value and significance. 1) Acute or chronic lesions with alveolar haemorrhage, endogenous lipid pneumonia, xanthomatous granulomas, an organising pneumonia with an alveolitis. 2) Bronchial lesions: Bronchitis and necrotising bronchiolitis, which is more rarely follicular. 3) Sero-fibrinous or infiltrative neutrophil pleural lesions with focal microabscesses, elastolysis and elastophagia with giant cells in the elastic lamina. Thirteen cases presented with misleading lesions, which was a possible source of diagnostic error and led to a discussion of several associated disorders (Goodpasture's syndrome, and collagen disorder syndrome) or there may be systemic angiitis (Giant cell or lymphocytic) or also systemic or tissue eosinophilia (Churg-Strauss syndrome, bronchocentric granulomatosis) or necrotising bronchitis (atrophic polychondritis) or other forms of nodular interstitial fibrosis, such as histiocytosis X. We would like to stress the great polymorphic variation of the lesions and the difficulties which confront pathologists in the diagnosis of Wegener's Disease, above all when it is localised to the lung. There is value in finding at least one major diagnostic criteria which is associated with a minor criteria and with the help of the C.ANCA levels may lead to a narrow clinicopathological correlation and allows for a fairly precise approach to the diagnosis and identification of early or unusual lesions and thus to the early treatment of patients before irreversible renal failure appears.
We studied a retrospective series of 25 sub-pleural fibromas (benign localized pleural mesotheliomas) of which 23 where excised, collected over a period of 20 years. The current series confirms the excellent prognostic of these rare tumors. They are sometimes bulky, but not infiltrating and discovered by systematic chest X-ray. Modern imaging using CT-Scan and nuclear magnetic resonance can suggest their diagnostic which is confirmed by histopathologic analysis of the usually pediculate and easily removed tumor. Transthoracic needle aspiration is generally of little help for diagnosis. The histopathologic diversity of the tumor is rather confusing but characteristic features can be found with fusiform cells ans collagen stroma. Immuno-histochemistry is more helpful for diagnosis and histogenesis recognition than electron microscopy.
In a series of 100 patients with intracardiac myxoma, the lesion was in the right atrium in 10 cases. The authors attempt to define the particular features of right atrial myxoma in terms of clinical aspects, outcome and pathology. The diagnostic difficulty raised by complex clinical pictures (including right ventricular failure, pseudopericardial forms and forms with a predominance of systemic problems) has now been resolved by imaging and, above all echocardiography. Consequences for the right side of the heart may involve the pulmonary artery with myxomatous emboli causing multiple fusiform aneurysms and pulmonary hypertension, or, more rarely, the tricuspid (with need for valve replacement), vena cava (Budd-Chiari syndrome) or atrial septum (right/left shunt). Alongside systemic problems due to interleukin 6, erythrocyte abnormalities are a new feature. Frequent etiological inclusion in a Carney complex explains recurrences and multi-cavity forms. The dominant pathological feature is tumor calcifications, no doubt as a result of the long presymptomatic phase of myxoma.
The term "aortic aneurysm" applies to numerous conditions with extremely varied anatomical and aetiological features, which consist of more or less complete and localized destruction of the aortic wall components resulting in a sacciform or fusiform distension induced by blood pressure, with loss of parallelism between the sides of the aorta. The most frequent aneurysms, leading to numerous cardiovascular operations, are encountered mainly in middle-aged men. These aneurysms complicate the infra-renal terminal atherosclerosis of smokers or the parietal elastic dystrophy of the ascending aorta, where a few subjects in their twenties with Marfan's disease join the older men, and which clinically presents as severe aortic incompetence. The aneurysm of syphilis and its destructive effect on thoracic structures is a thing of the past with a purely historical interest. Dissecting aneurysms--which should be called incomplete internal aortic disease with delamination of the media--remains a fearsome disease in subjects over 50, despite advances in surgery. Rare types of aneurysm (traumatic, infectious, or local accentuation of megadolichoarteries, aortitis or connective tissue dysgenesis) complete the list.
The clinician confronted to the diagnosis of infiltrative lung disease has to consider a wide spectrum of aetiologies. Among them, diffuse fibrosing interstitial lung disease is characterized by the deposition of collagen within alveolar structures. Either acute alveolar injury or chronic inflammatory alveolitis can induce pulmonary fibrosis. Basic research investigates the cells, the cytokines, and the extracellular matrix involved in the perpetuation of the fibropoliferative process.
We report a case illustrating the therapeutic consequences of an intravenous metastasis to the left superior pulmonary vein following resection of a voluminous primary lung carcinoma. Arterial spread of malignant cells occurred because the size of the tumor did not allow immediate clamping of the left superior pulmonary vein. The embolism was situated at the aortic bifurcation and lower limb ischemia persisted despite also emergency embolectomy. Distal (lower popliteal) embolectomy was also unsuccessful, and lower limb amputation was inevitable. This case illustrates the problems encountered in surgical treatment of pulmonary vein invasion by lung carcinoma and the role of adjuvant chemotherapy and radiotherapy.
We report a case illustrating the therapeutic consequences of an intravenous metastasis to the left superior pulmonary vein following resection of a voluminous primary lung carcinoma. Arterial spread of malignant cells occurred because the size of the tumor did not allow immediate clamping of the left superior pulmonary vein. The embolism was situated at the aortic bifurcation and lower limb ischemia persisted despite also emergency embolectomy. Distal (lower popliteal) embolectomy was also unsuccessful, and lower limb amputation was inevitable. This case illustrates the problems encountered in surgical treatment of pulmonary vein invasion by lung carcinoma and the role of adjuvant chemotherapy and radiotherapy.
We report here the medical history of a patient diagnosed with teratocarcinoma presenting with a pleural mass without mediastinal involvement as well as typical paraneoplastic hypertrophic osteoarthropathy. This atypical observation is discussed as well as data from the literature on thoracic localisations of extragonadal malignant germ cell tumours.