[Circulating anticoagulants. Clinical conditions of diagnosis].
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Biomedical subjects
Publications and source records attributed to B Wechsler.
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Superior vena cava obstruction (SVCO) is an infrequent syndrome. Clinical diagnosis is often late. 20 cases of SVCO collected in 7 years are being reported. In 17 cases, SVCO revealed the underlying disease. It was mostly neoplastic (12 times), especially pulmonary cancers (5 times) and lymphomas (twice). Among benign causes (5 times), mediastinal fibrosis was the most often recognized (twice). Prognosis depended on etiology: when it was malignant, mortality rate was 50 p. cent (actuarial survival rate after one year: 40 p. 100); when it was benign, there was no mortality (follow-up: 21 months). Thrombo-embolic complications (25 p. 100) were seen during the end-stage of neoplasms and were responsible in 4 out of 7 deaths. Surgery was interesting for diagnosis (histology) and treatment (bypass surgery, the result of which was good only in the 3 operated benign causes). A simple model is proposed for treatment.
A hundred and twenty-six patients with systemic polyarteritis nodosa (PN) were studied over a 23 year period. The clinical symptoms and paraclinical data of PN are reviewed, and the results obtained by the different therapeutic approaches used during this period are presented: steroids alone and then associated with immunosuppressors and, in the most recent cases, associated with plasma exchange. The actuarial survival of patients in this retrospective and unrandomised series was comparable whether steroids were used alone or with immunosuppressors. The different therapeutic approaches in PAN are discussed especially with respect to the role of immunosuppressor therapy and plasma exchange.
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In a series of 120 patients with periarteritis nodosa (PAN), 50 had gastrointestinal manifestations; 34 had transient abdominal pain which regressed spontaneously or in response to corticosteroid therapy and required no further investigation. Thirty one more serious episodes occurred in the remaining 26 patients. Eight of these were in fact the initial signs of PAN and 13 required laparotomy. There were 20 episodes of abdominal pain (peritonitis: 9, pancreatitis: 4, acute cholecystitis: 2, duodenal ulcer: 3, intestinal infarction: 1, unexplained pain without diagnosis at laparotomy: 1) and 11 of gastrointestinal hemorrhage (melaena or hematemesis: 4; hematochezia: 5). Clinical and biological features of patients with and without gastrointestinal manifestation were not significantly different except for cardiac involvement which was significantly more frequent (p less than 0.05) in the second group. Corrected survival rates were significantly lower (p less than 0.05) in patients with gastrointestinal manifestations. These results show that, in patients with PAN, digestive manifestations, particularly perforations, carried a poor prognosis. Nevertheless exploratory laparotomy and surgery unrelated to PAN (eg appendicectomy) were well tolerated.
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The authors reviewed the outcome of 73 operations performed on 60 patients on long-term steroid therapy: Eighteen operations were carried out for gastro-intestinal perforation but the causal role of steroid therapy was only established in 6 cases (4 gastric and 2 colonic perforations). In the other 12 cases there was intercurrent pathology or a recrudescence of the underlying disease process. More selective indications for steroid therapy and the protective action of new drugs on the gastro-intestinal mucosa are probably responsible for the low incidence of iatrogenic complications compared with previously published series. However, steroid therapy did affect the postoperative course: the risk of infection was 20 to 30 p. 100 higher than normal, very common after emergency surgery (75 p. 100) and a major cause of mortality (60 p. 100); the risk seems to be high when the steroid dose exceeds 0.5 mg/kg/day, the disunion of an anastomosis was observed in 6 cases, including 5 "dirty" operations with a mortality of 60 p. 100, healing was slow: 2 cases of evisceration and 2 of abdominal hernia were observed. It was not possible to assess the threshold dose of steroids with regards to this complication, metabolic complications, especially adrenal failure were much less common. Of the diseases requiring steroid therapy the problem of angiitis seemed particularly prominent. Although the prognosis of the intercurrent surgical condition was not aggravated; mortality from a relapse of the angiitis was nearly 100 p. 100. Therefore, medical control of the relapse would seem to be the first priority and should be taken into account when assessing the surgical indications.
Syphilis is a well-established cause of uveal disease and is thought to be responsible for several distinct types of intraocular inflammation. Four cases of acquired syphilitic uveitis are reported. The diagnosis was essentially based on the clinical picture, the elimination of other possible causes, the results of reliable blood immunologic tests eg, the TPHA, FTA and Nelson tests, and the rapid response to penicillin therapy. Evaluation of anti-Treponema pallidum antibodies in the aqueous humor does not contribute to the diagnosis of syphilitic uveitis. Two cases needed large amounts of penicillin (total dosage, 440 million units). Oral steroids were used to control the associated inflammatory reaction and to prevent a Herxheimer reaction. All the patients responded well to therapy.
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Three cases of giant cell arteritis associated with periarteritis nodosa are reported. A review of the literature showed 16 similar reports. Giant cell arteritis features in temporal arteries were associated with periarteritis features in visceral or peripheral arteries in 5 cases only. Association of these two conditions seems to be uncommon and may be coincidental but does not justify including giant cell arteritis within the group of necrotizing angiitis. Awareness of this association nevertheless has practical implications as these cases should be treated as PAN patients.
With reference to a case of vitamin C deficiency in a strict vegetarian, the authors recall the clinical findings and current diagnostic procedures in scurvy. Serum and urine ascorbic acid assays are now available and established the diagnosis. Management rests upon vitamin C given in a curative dosage of 1 to 2 g per day for 15 days followed by a preventive dosage of 10 mg per day.
The authors report their experience with 45 cases of inferior vena cava thrombosis. Diagnosis was delayed for an average of 55 days. One-third of cases were revealed by an embolic complication. Inflammatory diseases were the most common causes (Behcet disease: seven cases, systemic lupus erythematosus: 5 cases). Malignancies accounted for 20% of cases. Abnormalities of coagulation were uncommon: antithrombin III deficiency in one patient and protein C deficiency in another. Estrogen-progestogen combinations could be incriminated in 4 cases. Outcome was fatal in 20% of cases, usually as a result of the underlying disease. Functional status was good in two-thirds of patients without malignancy followed up for an average of 27 months. In 14 patients a clip was inserted to ensure total (3 cases) or partial (11 cases) interruption of vena cava blood flow because of a free thrombus and/or recurrent pulmonary embolism. Three patients had thrombectomy. After clip insertion two embolisms were recorded, one of which occurred in the immediate post-operative period.
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Nailed capillaroscopy was systematically performed in 30 patients with Behcet's disease to search capillary dystrophies with paradoxal normal number of capillary loops; some indirect signs of microvascular disease were noted: pallor of the background; petechiae; sludge; abnormal visibility of the venous plexus and of irregularly arranged venules. Eight patients had a normal capillaroscopy: thirteen had direct signs of microvalvular abnormalities (nine cases with two direct signs and at least two indirect signs; and four cases with two direct and one indirect, or one direct and three indirect signs). Nine patients had only sludge and/or petechiae. We never observed any abnormality in the number of the capillaries, in the sweat droplets nor in the circulatory speed. Two patients had megacapillaries resembling those seen in scleroderma. The petechiae were observed in 50 p. 100 of the cases. We found no correlation between the capillaroscopic abnormalities and the age, sex, duration of the disease, or ethnic background. However, the presence of aphthosis the day of the capillaroscopy seems to be correlated with the cutaneous microvascular abnormalities. Thus, there exist frequent capillaroscopic abnormalities in Behcet's disease. Although aspecific, they point out the vascular tropism of this disease, resembling that of the vascularite. The prognostic importance of the capillaroscopy in Behcet's disease is under study.
Six women, aged 16 to 27 years old at the beginning of their illness suffered recurrent spontaneous abortion (two to eight episodes) and three of them had arteriolar venous thrombosis. These symptoms led to the finding of an antiprothrombinase type of circulating anticoagulant. In two cases, positive dissociated syphilitic serology was observed and all patients presented other haematological abnormalities: thrombocytopaenia and/or autoimmune haemolysis. The diagnosis of disseminated lupus erythematosis was established after an average period of 11 years (range 1 to 27 years) based on at least 4 of the ARA criteria (five out of six cases) and/or characteristic immunological abnormalities (five out of six cases). Thrombosis is more common in lupus when there are associated haematological abnormalities. It is probably directly related to the presence of circulating anticoagulant which inhibits the production and/or secretion of prostacyclin by the endothelial cells.