[Respiratory sites of amyloidosis].
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Biomedical subjects
Publications and source records attributed to B Wechsler.
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Despite its poor specificity, CA 125 is a widely used tumoral marker. Significant rises in serum CA 125 levels are found mainly in ovarian cancer, but they also occur in other malignant or benign pathologies such as chronic liver diseases. However, in benign pathologies the CA 125 concentrations hitherto reported did not exceed 850 U/ml. The authors report two cases of chronic constrictive pericarditis with ascites and pleural effusions in which a very high increase of serum CA 125 levels (above 1,200 U/ml) was directly related to the presence of pleural effusions.
Corticosteroids have transformed the prognosis of systemic lupus erythematosus (SLE), but no further advance has been achieved during the last few years. Treatment of SLE rests on the use, as rational as possible, of those therapeutic tools that are available. However, diagnosing SLE does not necessary, they must be given promptly in effective doses whilst preventing their side-effects, notably by the simultaneous management of atheroma factors. Non-steroidal anti-inflammatory agents and synthetic antimalarial drugs may be used to replace corticosteroids or reduce their dosage. Severe forms of SLE which are function- or life-threatening require immunosuppressants, the best one being cyclophosphamide administered monthly as intravenous bolus injections, as this drug has been shown to be effective and to have low morbidity. Plasmapheresis is exceptionally needed. The risk of thrombogenesis due to the frequently associated "antiphospholipid syndrome" can be avoided by anticoagulants or antiplatelets the respective indications of which have not yet been clearly determined. SLE being a chronic disease, patient's compliance with treatment is very important and justifies the efforts made to train and inform these young male or female patients.
Seventeen patients with Behçet's disease were treated with intermittent intravenous bolus injections of cyclophosphamide. Twelve patients had severe eye lesions, 7 had central neurological disorders and 2 had both ocular and central nervous system involvement. Cyclophosphamide was given during 4 weeks on average, in mean doses of 922 +/- 127 mg. Each patient received an average of 10 +/- 5 injections. The drug was generally well tolerated; only one patient developed moderate and transient hepatocytolysis. Visual acuity was improved in 12 of the 22 eyes with active lesions, and signs of inflammation disappeared in all cases. Neurological improvement was observed in 5 of the 7 patients affected, with complete regression of the disorders in 2 of them. In 16 patients who were also under corticosteroid therapy, steroid dosage could be significantly reduced by 44 +/- 11 mg per day.
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Nine cases of infective endocarditis (IE) on native valves, complicated by arterial embolism of the limbs (AEL), were collected between June 1974 and October 1988 (prevalence 4.3 percent). Among the 11 AEL recorded, 9 involved the lower limbs and 2 the upper limbs. The diagnosis, suspected in patients with acute ischaemia (n = 6), transient (n = 1) or pseudophlebitic (n = 1) pain, or discovered by systematic pulse examination (n = 3), was confirmed by Doppler ultrasound (n = 3), angiography (n = 2) or oscillometry (n = 4). AEL occurred 2.8 weeks on average after the onset of treatment; it appeared 6 months after the end of treatment in 1 case and preceded the diagnosis of IE by 1 to 6 weeks in 3 cases. The causative organisms isolated in 7 cases were: non-haemolytic streptococci (n = 4), Staphylococcus aureus (n = 1), Haemophilus parainfluenzae (n = 1) and enterococcus (n = 1). Vegetations were found in 6 of the 7 patients explored by echocardiography. Two cases of embolism of the femoral artery required embolectomy. Effective heparin anticoagulation was obtained in only one patient. Six patients underwent valve replacement in the acute phase of endocarditis. After a mean follow-up period of 32 months (range 3 to 120 months), only one patient has symptoms (claudication of the left upper limb); 5 patients are asymptomatic with a reduced (n = 5) or abolished (n = 2) pulse. Three embolisms have left no sequelae. Altogether, AEL are not uncommon in infective endocarditis. They rarely influence the functional prognosis and are detected by systematic palpation of the pulses. Anticoagulation in effective doses is discussed. Attempts at removing the obstruction should be made only in cases with poorly tolerated proximal embolism. In patients with multiple or recurrent embolic accidents, valve replacement may be considered.
Prescribing a corticosteroid treatment requires a three-step strategy: a) to chose the appropriate drug, its dosage regimen, and its route of administration; b) to reduce the side effects by taking the status of the patient into account, and by adopting various preventive recommendations; c) to reduce the dosage before deciding to stop treatment according to specific rules. This "good practice" of corticosteroid therapy makes it a well tolerated and observed treatment, and alleviates the fears that is often created in the patient and his physician.
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We describe six cases of cerebral venous thrombosis in patients with systemic lupus erythematosus. In one patient, cerebral venous thrombosis was the initial manifestation of lupus; in the five others, it occurred 1-33 years after the diagnosis of lupus. The main clinical features of cerebral venous thrombosis were persistent headache in all six patients, focal symptoms in four, and seizures in three; papilledema was present in only one patient. Cerebral venous thrombosis was diagnosed based on angiography or magnetic resonance imaging. Both the transverse (in five patients) and the superior sagittal (in three) sinuses were involved. Extracranial arterial and/or venous thrombosis were present in three patients, abortion in two, thrombocytopenia in four, and lupus anticoagulant in three. The neurologic symptoms resolved rapidly in five patients treated with steroids and heparin. Cerebral venous thrombosis should be suspected in patients with lupus who complain of persistent headache, especially in the presence of neurologic symptoms.
Thirty-five cases of biopsy-proven sarcoidosis with neurologic manifestations are reported. Neurosarcoidosis was the presenting symptom in 31% of cases and the only clinical manifestation in 17%. Mean follow-up time was 48 months. Central nervous system involvement was observed in 37% and meningitis in 40% of patients. Other manifestations were cranial nerve palsies (37%), peripheral neuropathy (40%), and myopathy (26%). Multiple neurologic manifestations were present in 51% of cases. All but 4 were treated with corticosteroids. Another immunosuppressive agent or cerebral irradiation was added in 6 and 2 patients, respectively. Complete recovery was observed in 46%, improvement in 46%, 4% remained stable, and 4% worsened. There were no deaths. We advocate treating neurosarcoidosis with corticosteroids as early as possible. If the patient's condition worsens, additional immunosuppressive agents or cerebral irradiation is warranted.
Hemolytic uremic syndrome (HUS) has been reported in patients treated with cyclosporin A (CsA) following bone, hepatic and kidney transplantation. We report two patients with Behçet's disease (BD) under CsA treatment because of severe uveitis, who developed HUS several months after the initiation of treatment. Renal biopsies showed lesions consistent with the diagnosis of the arterial form of thrombotic microangiopathy: vascular thrombosis with extensive glomerular ischemia. Renal failure persisted after withdrawal of CsA: one patient is in chronic renal failure (CRF) with a 4-year follow-up; the other died after refusal of chronic hemodialysis. In our two patients, excessive doses of CsA with high trough levels are likely to have contributed to the development of HUS. A rapid adjustment of CsA doses and an early detection of signs of the microangiopathic process might have prevented this severe complication of CsA treatment.
Between 1974 and 1988, 7 myocardial infarctions occurred in 6 (4 men, 2 women) out of 400 systemic lupus erythematosus patients. Their ages at the onset of lupus ranged from 13 to 44 years (m = 26). Four had renal involvement. Control of lupus in all 6 patients required high-dose steroids (at least 1 mg/kg/d of prednisone). Myocardial infarction occurred 4 to 19 years after the onset of lupus (m = 13). One patient died of cardiogenic shock. When the infarction occurred, only one patient was undergoing a lupus flare, while the disease was quiescent or slightly active in the 5 others. One patient had no risk factors for atheroma but had been taking steroids for 10 years. Among the other 4, one had hypertension, another had hyperlipidemia and 3 were smokers; they had been on steroids for 2, 4, 11 and 13 years. Coronary angiogram showed occlusion in all 4, but atheroma in only 2 patients. Lupus anticoagulant was present in 3 of these 4 patients. The mechanisms responsible for coronary occlusion in lupus patients are probably complex and interwoven. In addition to "classical" factors (i.e., vasculitis or steroid-induced atheroma), other factors, such as antiphospholipid antibodies and/or smoking, may play an important thrombogenic role.
Pregnancy is allowed in an increasing number of women with systemic lupus erythematosus (SLE). In order to determine the influence of pregnancy on SLE and vice versa, a French epidemiological register was established on 1 January 1987, under the aegis of the Société Nationale Française de Médecine Interne. As of 1 December 1989, 55 pregnancies in 51 women coming from 24 different Departments of Medicine were recorded. Intermediate results were analyzed from 42 women (2 successive pregnancies) of which 46 pregnancies (2 twin pregnancies) have come to term. Pregnancy can be allowed in women with SLE. The disease influences its evolution in nearly two-thirds of the patients, but without maternal death. Among 4 women whose disease previously undiagnosed, 2 developed glomerulonephritis. A woman without prior renal involvement developed proliferative diffuse glomerulonephritis during pregnancy. Fetal prognosis appears to be linked to the activity of the disease at the beginning of pregnancy: early spontaneous abortions always occurred when the disease was active, even if the signs of activity were only biological. This fact underlies the importance of complement dosage because the physiological expression of the usual inflammatory parameters increases during pregnancy. In addition, it seems that SLE treatment should be adapted to the evolution of biological tests. Renal involvement regardless of its origin--SLE or not--(it is often impossible to determine the respective contribution of each cause, which leads to increasing the corticosteroid dosage and giving antihypertensive drugs simultaneously) seems to be a factor of prematurity.
In order to evaluate the significance of ungual and conjunctival microvascular abnormalities observed in diabetics prior to retinopathy, nailfold and conjunctival capillaroscopy was performed in 20 controls and 40 insulin-dependent diabetics of the same age. The diabetics were divided into 4 groups according to their state of retinopathy: absent, incipient, non-proliferative and proliferative. No difference was found between controls and diabetics and between groups of diabetics in the frequency of conjunctiva microaneurysms and specific nailfold microangiopathy, nor even in that of the so-called characteristic "fish shoal" image. The percentage of abnormalities detected was concordant with the results of previous studies in diabetics (12.5 per cent of non-specific organic microangiopathy), but it was 2 to 4 times higher than the frequency usually found in non-diabetic controls. This discrepancy could be due to the method used, since in contrast with earlier studies the operator did not know whether the subject was diabetic or not and was unaware of the patient's retina state. In the absence of other blind and prospective studies, capillaroscopic examination for diabetic microangiopathy should be reserved strictly to clinical research.
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