Chronic destructive ulcerative lesion of the midface and nasal cavity due to leishmaniasis contracted in Djibouti.
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Biomedical subjects
Publications and source records attributed to P Godeau.
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We present the case of a 50-year old man who progressively developed tricuspid valve insufficiency with opening of a patent foramen ovale responsible for right-to-left shunt with polycythaemia. The tricuspid valve insufficiency was due to a foreign body, probably of surgical origin as suggested by its radiological image and by the patient's previous history. It would have been introduced, far away from the tricuspid valve (compound fracture of the wrist), several years previously. At surgery, we found the foreign body embedded in the valve system. As a possible mechanism for the mutilation, an undiagnosed endocarditis was suspected but could not be confirmed. Three cases tricuspid endocarditis (with foreign bodies in the right ventricle) and 3 cases of asymptomatic tricuspid valve foreign bodies have been published. Fifty-five cases of foreign bodies introduced peripherally and migrated into the heart, the pericardium and the pulmonary artery are reviewed.
Twenty-five antinuclear antibody (ANA) negative patients with systemic lupus erythematosus (SLE) or lupus-like disease were compared to 91 ANA positive patients with SLE for clinical and biological symptoms. Cutaneous symptoms were infrequent in ANA negative patients (p less than 0.03). Thrombocytopenia (p less than 0.001), venous or arterial thrombosis (p less than 0.02) as well as cerebral infarction (p less than 0.001) were more frequent. Three types of antiphospholipid antibodies were determined by different methods; the VDRL, the lupus anticoagulant and an ELISA for IgG anticardiolipin antibody (aCL). The frequency of a positive VDRL test was significantly higher in the ANA negative group (p less than 0.05). Correlation studies suggest that the 3 methods are not redundant and detect overlapping but not identical antibodies. Of the 3 antiphospholipid antibody assays, only the IgG aCL test was significantly associated with thrombosis in the ANA negative group (p less than 0.02).
A case of myocardial infarction in a 23-year old male patient with Behçet's disease is reported. The infarction occurred 4 years after the onset of the disease, which had been marked by recurrent venous thrombosis. Coronary arteriography showed stenosis of the anterior interventricular artery and occlusion of the first diagonal artery; the other coronary vessels were normal. A search for vascular risk factors, including haemostasis, was undertaken, yielding only moderate cigarette-smoking. About 10 cases of myocardial infarction associated with Behçet's disease have been reported. They concerned young, usually male subjects. Infarction usually occurred late in the course of the disease, and vascular risk factors were seldom elicited. The leukocytoclastic vasculitis of Behçet's disease alone may be responsible for stenosis, thrombosis and false arterial aneurysms, as shown by anatomical studies. The physiopathological mechanisms involved (reduction of endothelial or systemic fibrinolytic activity, rise in fibrinogen and factor VIII) are still unclear; we believe that these abnormalities are inconstant. Behçet's disease may be regarded as a possible cause of myocardial infarction in young subjects.
Anatomical studies have demonstrated the high incidence of vasculitis in SLE, the appearances of which are variable and non-specific, ranging from necrotizing angiitis which is undistinguishable from periarteritis nodosa, to scarring lesions. Micro-angiitis is easily demonstrated in skin lesions and is also encountered to varying degrees in CNS, renal, cardiac, pulmonary and gastrointestinal localisations. Disease of large vessels is more rare and sometimes causes gangrene of the limbs. In SLE, vasculitis should be distinguished from thrombosis related to lupus anticoagulant and from atherosclerosis favoured by chronic steroid therapy but perhaps initiated by vascular deposits of immune complexes during the acute inflammatory stage. The treatment of lupic angiitis is mainly based on steroid therapy. The results are variable, probably due to the fibrous nature of some of the vascular lesions.
Three cases of primary amyloidosis and 2 cases of familial amyloidosis complicated by asympathicotonic orthostatic hypotension are reported. Blood pressure measurements on a tilting table, plasma renin activity and plasma aldosterone or catecholamine concentrations enabled localisation of the lesion of the baroreceptor reflex in some cases. When the pre- and post-synaptic efferent sympathetic pathway was intact, treatment associating Tyramine and Tranylcypromine may provide these bedridden patients some autonomy of movement. When this pathway is affected by the disease the association of Indomethacin, Dihydroergotamine and 9-alpha-fluorohydrocortisone may be tried.
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Twenty-five patients with at least 3 of 1982 ARA criteria of SLE but without the ANA, were compared with 91 patients with 4 or more of the ARA criteria of lupus with positive ANA. The ANA-negative group was characterised by the low incidence of skin involvement, serous effusions and alopecia, and a relatively high incidence of thrombocytopaenia and venous and arterial thrombosis. Three types of antiphospholipid antibodies were looked for: the VDRL, antiprothrombinase and anticardiolipin antibodies by an immuno-enzymatic method. The VDRL was the only antibody which was significantly commoner in the ANA-negative group. Statistical studies showed that the three methods of demonstrating antiphospholipid antibodies detected crossed but not identical specificities. In the ANA-positive group only the antiprothrombinase was associated with a high incidence of venous thrombosis and stroke. In the ANA-negative group, only the anticardiolipin antibodies were associated with a high incidence of arterial or venous thrombosis. Two subgroups may be identified in the group of ANA-negative lupus patients: firstly, those with high anticardiolipin antibody titres with a high incidence of thrombotic and haematological complications, and, secondly, patients with low anticardiolipin antibody levels with a high incidence of cutaneous involvement, serous effusions and Raynaud's phenomenon.
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Pseudoxanthoma elasticum (PXE) is exceptionally associated with abnormal phosphate-calcium metabolism. On the other hand, significant hyperphosphataemia is present in tumoral calcinosis, with an occasional elastopathy resembling that of PXE. We report here the first case of diffuse elastic tissue disease associated with hyperphosphataemia in the absence of tumoral calcinosis. The patient, a 29-year old Gabonese, had severe arteritis of the lower limbs with linear calcifications of the aorta and major arteries of the limbs. Angioid streaks and calcifications were present in the retinas. The skin appeared to be normal, but histological examination of specimens from flexures showed abnormalities of the elastic tissue with many fibres being broken into small pieces and curled up. In a morphometric study of cutaneous elastic tissue these abnormalities were compared with those observed in a PXE patient and with normal elastic tissue. The abnormalities in our patient and in the PXE patient were similar. Compared to normal elastic tissue, there was a slight decrease in the total number of oxytalan and elaunin fibres. The elastic fibres were distinctly wider, longer and separated from each other by smaller spaces, but not more numerous than normally. The histopathological appearance of an artery removed during bypass surgery was similar to that of a PXE artery. The hyperphosphataemia was due to an increase in tubular absorption of phosphates without abnormalities of parathyroid hormone, or resistance to its phosphaturic effect or any other tubular disorder. It was little altered by aluminium carbonate, and it partially decreased after addition of calcitonin.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors report a series of 11 patients who consulted or were admitted to a hospital medical unit for organic symptomatology, especially hypertensive, diagnosed as panic attacks based on the criteria of the DSM III. Cardiovascular symptoms were dominant in 10 out of the 11 patients. In 7 cases, the symptoms were very suggestive of pheochromocytoma. Treatment based on tricyclic antidepressive drugs associated with behavioural therapy in 5 cases, led to the total regression of acute panic attacks in 9 out of 11 patients with a follow-up period ranging from 1 to 2 years. However, it did not prevent 2 of the patients from developing a chronic anxiety state. After reviewing the principal clinical features of panic attacks, the authors discuss their underlying physiopathological mechanisms. The results of catecholamine metabolic studies in 2 out of 3 patients suggest the possible role of an inhibitor of catecho-o-methyl-transferase, but this requires confirmation by further studies. Panic attacks are very prevalent in the general population (2 to 5 p. 100). The diagnosis must be born in mind to avoid long and costly investigations, and for specific treatment to be instituted. The main risk is the development of chronic severe morbidity with serious socio-familial consequences.
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Numerous drugs have been suggested for the treatment of systemic scleroderma. They may be studied and classified according to their site of action on the chain of events that leads from vascular abnormalities to sclerosis of the skin. Thus, proline analogues, colchicine, lathyrogenic agents, D-penicillamine, coagulation factor XIII and oestrogens are thought to act on collagens and their metabolism. Ketanserin has been suggested by the discovery of tryptophan abnormalities. Corticosteroids exert an inhibitory effect on fibroblasts. The use of calcium antagonists, angiotensin-converting enzyme inhibitors, prostacyclin and anti-platelets rests on the presence of vascular abnormalities. The purpose of treatments with immunosuppressive drugs or plasma exchanges is to act on possible lymphocytic and/or macropageal factors.
The authors report a case of acute intermittent porphyria in a 31-year old woman, revealed by inappropriate secretion of the antidiuretic hormone, a diagnosis confirmed by radioimmunoassay. Measurements of red cell urosynthetase made the diagnosis of porphyria certain and showed that the disease was familial, although clinically asymptomatic. Antidiuresis is not the only cause of hyponatraemia in acute intermittent porphyria. Similarly, psychic disorders are frequent in SIADH, but they are not necessarily due to acute intermittent porphyria.
The authors report the case of a 33-year old male Tunisian who, 7 years after the onset of complete Behçet's disease (diagnosed on international criteria) developed a nephrotic syndrome diagnosed as renal amyloidosis on histological evidence. Attenuation of Congo red staining by potassium permanganate pointed to AA type amyloidosis. Despite treatment with colchicine and cyclophosphamide the patient's condition deteriorated. Amyloid deposits were found in a small bowel biopsy performed for persistent diarrhoea. The patient died 3 years after amyloidosis was diagnosed. The authors have reviewed the literature concerning this exceptional association: in all cases where it was typed the amyloidosis was AA. Attention is drawn to the failure of colchicine in this patient.