[Postembolic pulmonary artery hypertension. Necropsy study of 41 cases].
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Biomedical subjects
Publications and source records attributed to P Tranbaloc.
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Visceral involvement in systemic scleroderma is frequent, the most commonly affected organs being the kidney, the lung, the heart and the gastro-intestinal tract; associating different degrees of sclerosis and vasculitis depending on which organ is involved. However, this is not always clinically obvious, and angiospastic phenomena may be assumed to be responsible for certain ischaemic lesions. The diagnosis is also difficult because some visceral lesions may occur at an early stage and appear to be of primary origin. Extensive investigation, including biopsies to demonstrate small vascular changes then becomes essential.
The authors discuss a large series of biopsy and necropsy findings in patients with giant cell arteritis (Buerger's thromboangiitis, Horton and Takayasu's diseases), and then in patients with other forms of arteritis with an inconstant plasmodial factor, such as the Chürg and Strauss syndrome, Wegener's disease, the pseudo-sarcoidosis form of lymphomatoid granulomatosis. The histology, correlated when necessary with ain immunofluorescent study, was completed by a topographical and evolutional study of the lesions in all cases. The pathogenesis of these different forms of arteritis is discussed in the light of the ultrastructural data concerning the formation of the various types of giant cell and the methods of identification of the vascular walls.
Arterial angiodysplasias constitute an ill-defined entity comprising conditions of apparently very variable natures. Apart from arterial fibrodysplasia, the clinical features of which, if not the etiology, are well established, there exists a number of different arterial abnormalities, stenotic or aneurysmal, the significance of which remains unclear (dolicho-arteries, the Moya-Moya syndrome, etc.). However, the role of genetic factors and metabolic abnormalities in these conditions is uncontestable. They demonstrate both the authenticity of this group of diseases and the importance of metabolic disorders of the interstitial tissues in the majority of these vascular abnormalities.
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465 patients with broncho-pulmonary malignant tumors have been autopsied. Small cell carcinoma was diagnosed in 22.5 per cent of these patients. The histo-cytological variants of these tumors (lymphocytoid, polygonal, fusiform and polymorphic) had the same general characteristics (age, sex, survival) and a similar clinical course. Grossly and histologically, the bronchial tumor, always located in proximal bronchial tree, largely involved the mediastinum. Metastases were peculiarly frequent to the liver (69%), to bone (64.2%) and to the central nervous system (36.2%). Three Schwartz-Bartter syndromes and two Denny Brown sensitive neuropathies were noted in this statistical study.
Biopsies of the labial accessory (144 cases) or principal (4 cases) salivary glands were performed in 139 patients with clinically and biologically confirmed Sjögren's syndrome, and 9 patients with rheumatoid arthritis alone to act as control. Confirmation of diagnosis was obtained in both isolated cases (31 p. cent) and those with associated collagen disease (69 p. cent). Histological lesions were classified in 3 stages of increasing severity. Stage I cases had ectasia of several distal canals which, in the absence of significant lymphocytic reactions, permitted early diagnosis. In stage II, a typical appearance was noted: abundant nodular or diffuse lymphocytic infiltration with an increase in numbers of ectasias. In stage III of acini destruction, the dominant feature was diffuse peri- and intralobular sclerosis and only rare lymphocytes. Is, therefore, distal canal dilation the most reliable diagnostic sign of the syndrome, which has been shown by ultrastructural studies to arise from massive destruction, followed by anarchic regeneration, of myoepithelial cells that ensure canal contractility. These 3 histologically-defined stages generally correlate well with those normally employed to grade sialographic images (stage I, initial, showing miliary filling defects; stage II with larger rounded areas and penetration of the contrast medium into tissues around the canal; stage III demonstrating the "dead tree" appearance together with lack of contrast medium extension beyond Stenon's duct and its main branches). However, certain discordances were noted, histological signs sometimes preceding sialographic modifications. Biopsy of accessory salivary glands could be the most simple, reliable method for confirming diagnosis of Sjögren's syndrome in its early stages.
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A human His bundle was studied two hours after death by histoenzymological techniques and electron microscopy. The pathway had a much higher cholinesterase activity than the working myocardium, due to its richness in nerve endings: this was confirmed by electron microscopy which also distinguished "common" contractile cells (working cells) from P type and "intermediary" cells; those were by far the most common, presenting an ultrastructure identical to that of the Purkinje cells, classically described in the bundle branches alone. These findings and the unique longitudinal architectural organisation of the His bundle, confirm the studies of JAMES and may explain the rapidity of conduction in this structure.
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A case of post-traumatic desmoid tumor of the mandible is reported in a 4-year old girl. This tumor was considered typical clinically and by light microscopy. Four years later, the patient was symptomless, without evidence of recurrence. Histoenzymology showed a high level of leucine aminopeptidase activity. On electron microscopy, a part from a few primitive mesenchymal cells, abnormal fibroblasts represented the principal cellular component. These cells showed evidence of intracytoplasmic collagen fibers synthesis and of myoid differentiation (numerous filaments and endocytosis vacuoles). Myofibroblasts, with a microfibrillary network showing areas of dense osmiophilic condensation in the vicinity of cell membrane were also present. This atypical ultrastructural feature suggests, in this case, the non-neoplastic nature of this lesion. It seems to reflect an abnormal reaction to a traumatic aggression, characterized by proliferation of myofibroblasts (which normally disappear in the late stages of healing) and to have, thus, the signification of pathologic scar.
Using an experimental model of genetic diabetes (DBM mouse) a comparison was made of the results of quantitative data obtained by light and electron microscopy (measurement of glomerular and mesangial surface areas, assessment of the thickness of the basal membrane and its irregularities) and was used to demonstrate the actual presence of glomerulosclerosis in the renal parenchyma of 31 diabetic animals. In addition, immunofluorescent investigations in these same animals demonstrated the presence of serum proteins (in particular immunoglobulins and albumin) in the glomerulus and the tubular basal membrane. These substances transude through the vessels as a result of increased vascular permeability. In this area, membrane abnormalities are not a consequence of hyperglycaemia. In the group, one batch of animals in which hyperglycaemia was partially reduced by glycoregulatory therapy showed the same glomerular changes. Amongst other factors, the possible role of hyperinsulinaemia constantly present in these animals, regardless of their blood glucose level, is worthy of considération.
Comparative ultrastructural and histoenzymological study of 10 osteogenic (4 osteosarcomas), osteolytic (3 benign giant cell tumours, 2 malignant osteoclastomas) and fibrosarcomatous osseous tumours of the jaw emphasised the analogies between osteoblasts and fibroblastic stromal cells of giant cell tumours. These two cell types, with their abundant granular ergastoplasm, secrete a collagen interstitial fibrillary substance which is capable of secondary calcification. Their enzyme activities, with regard to oxidative mechanisms and protein synthesis are identical. Osteogenic activity as assessed by ATPase and alkaline phosphatase, more marked than in osteosarcomas, is also found in the stroma of benign giant cell tumours. With regard to the plasmodes characteristic of giant cell tumours, they are certainly original from both a morphological and enzymological standpoint : richness in mitochondria and lysosomes, intensity of oxidative reactions (B.O.H.B.D. +++). Nevertheless, the morphological appearances seen would be more in favour of their stromal origin than in their arising from monocytes-macrophages coming from the blood.
Full renal investigation were conducted in 11 patients with Behçet's syndrome. Biological tests revealed the presence of mild proteinuria in 5 of them. Needle biopsy showed extra-membranous or mesangial fibrinoid deposits in 6 cases, associated in 1 case with segmental and focal glomerulitis lesions. Immunofluorescence studies demonstrated glomerular deposits of mesangial and extra-membranous C3, sometimes with immunoglobulins (IgA-AgG). Ultrastructural examination was carried out in 7 cases and showed "hump" deposits attached to the external face of the basal membrane and rounded nodules of 7 to 15 mu included in the mesangium. The presence of immune complexes in the renal glomeruli, therefore, is not all unusual in this disease, which is probably auto-immune in nature, and in fact appears to be a quite frequent occurrence. These deposits, which were probably well-tolerated by most of the patients in this series, might be involved in the production of progressive glomerulopathies in chronic cases, as observed in one of the cases studied.
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