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Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

At least 271 records · Page 15Linked to original sources
PubMed1989

Cutaneous necrosis associated with the lupus anticoagulant.

Extensive cutaneous necrosis of the limbs with sudden onset was observed in 3 women suffering from benign systemic lupus erythematosus. All 3 had a circulating anticoagulant and a positive venereal diseases reference laboratory test without anticardiolipin antibodies. They were successfully treated with pulse methylprednisolone therapy and plasmapheresis. The necrosis-lupus anticoagulant relationship is discussed due to the absence of another possible etiology for cutaneous necrosis and the latter's histological aspect, i.e., thromboses of the dermal and hypodermal vessels without vasculitis. This cutaneous necrosis resembles that observed in congenital protein C deficiencies and favors the hypothesis of interaction between the lupus anticoagulant and the protein C-S complex.

Adult↗
PubMed1989

[Facial circumscribed ossifying myositis. Anatomo-clinical and tomodensitometric study].

1 case of circumscribed myositis ossificans of the temporal muscle is reported in a 21 years-old man. The initial complaint was a sudden non traumatic swelling. Roentgenography showed a characteristic feature: peripheral radio-opacity around a non-opacified area. Under microscope, we could find 3 zones: a central fibroblastic area, an intermediate zone with immature osteoid bone, a peripheral zone with mature lamellar bone. In addition, we used histoenzymological and electron microscopical methods. The difficulties of diagnosis, the pathogenesis and the prognosis of this disease were also discussed.

Adult↗
PubMed1989

[Cutaneous microcirculation in infectious endocarditis].

Nailfold capillary microscopy was used to study the microcirculation patterns in 26 adult patients with infective endocarditis. Abnormal patterns were found in 13 patients (50%). Enlargement of capillary loops was never observed. Significant correlations were found between the number of capillary abnormalities and both systemic involvement (cutaneous vasculitis, arthritis, splenomegaly and/or glomerulonephritis) and immunological disturbances (circulating immune complexes, rheumatoid factor and/or hypocomplementemia) (p = 0.02 and 0.003, respectively). Capillary abnormalities were significantly reduced in 14 patients studied 4 to 48 months after endocarditis was cured. However, due to the lack of specificity, nailfold capillary microscopy cannot be regarded as a useful tool for the diagnosis of infective endocarditis. Connective tissue disorders are not the sole diagnosis to be considered in patients with abnormal nailfold capillary microcirculation patterns.

Adolescent↗
PubMed1989

Temporal arteritis revealed by upper limb gangrene.

An 80-year-old white man presented with gangrenous lesions involving several distal phalanges of his left hand and an elevated erythrocyte sedimentation rate (ESR). Temporal artery biopsy showed patchy destruction of the internal elastic lamina by mononuclear cell infiltration, consistent with the diagnosis of temporal arteritis. After amputation of gangrenous lesions, he was discharged taking prednisone (60 mg/day). Twelve months after discharge there was no recurrence of ischemic manifestations and ESR was normal. Association of digital gangrene and elevated ESR should alert the clinician toward this diagnosis once other diseases such as atherosclerosis, scleroderma, lupus erythematosus, periarteritis nodosa have been ruled out.

Aged↗
PubMed1989

[Aortic aneurysm with vena cava thrombosis occurring in Behcet's disease].

We report a case of Behcet's disease complicated by aortic aneurysm and contiguous vena cava thrombosis due to compression. Arterial aneurysms are uncommon in the course of Behcet's disease and are associated with a poor prognosis owing to the risk of rupture. Vena cava thrombosis is found in 10% of cases; pulmonary embolism is infrequent. Venous and arterial lesions usually evolve independently. In most cases they are consecutive to vasculitis. The case reported herein is uncommon because of simultaneous and contiguous venous and arterial lesions. Eighteen months after aorto bi-iliac graft and inferior vena cava ligature, there is no recurrence of thrombosis nor aneurysm with a treatment including heparin, colchicine and azathioprine.

Adult↗
PubMed1988

[Wegener's granulomatosis in elderly subjects. 37 cases].

The clinical, biochemical and prognostic characteristics of 37 patients with Wegener's granulomatosis were studied in relation to age. Eleven patients were older than 60 years when the first sign of the disease appeared. Except for the fact that the renal lesions seemed to be more severe in these patients, there was no significant clinical or biochemical difference between them and patients under 60. Five patients over 60 years of age died during the first 2 years of Wegener's granulomatosis. Death was due to infection in 3 cases, to acute renal failure as complication of intra-alveolar haemorrhage in 1 case and to metastatic cancer in 1 case. Age-related comparisons of survival rates showed that the probability of surviving was lower in elderly subjects: 78 per cent at 1 year and 39 per cent at 3 and 5 years, as opposed to 96 per cent at 1 and 3 years and 84 per cent at 5 years in younger subjects (P less than 0.01). This poor prognosis is probably due to the renal lesions which constitute an important cause of death in the published series. Rapidly progressive glomerulonephritis being amenable to an early and intensive treatment, elderly patients should be treated as energetically as younger patients.

Adrenal Cortex Hormones↗
PubMed1988

[Cardiac involvement in Behçet's disease. 12 cases].

Fifteen cardiac manifestations observed in 12 of a series of 196 patients with Behçet's disease are reported. In this retrospective study conducted in an internal medicine department, 5 cases of pericarditis and 4 cases of myocardial infarction were encountered. The other cardiac manifestations were observed only once. They included ventricular aneurysm, endomyocardial fibrosis of the right heart, aortic insufficiency, mitral valve insufficiency, mitral valve prolapse, and right heart failure consecutive to pulmonary arterial hypertension. There was no relationship between the severity of cardiac lesions and that of the extracardiac manifestations of the disease. Comparison of these data with those found in the literature showed that pericarditis is the most frequent pathology, but it usually regresses rapidly. Lesions of the coronary arteries, with or without myocardial infarction, consist of stenosis, occlusion or pseudoaneurysm requiring surgical treatment. Myocardial lesions (with the possibility of pseudoaneurysm) and endomyocardial right heart fibrosis are exceptional but fairly characteristic of Behçet's disease which they should suggest. Hughes-Stovin's syndrome may be complicated by pulmonary arterial hypertension, although death is generally caused by massive haemoptysis.

Adult↗
PubMed1988

[Value of the cyclophosphamide bolus in severe systemic diseases. Preliminary results].

Twenty patients with severe systemic disease were treated intermittently with bolus intravenous injections of cyclophosphamide in doses of 0.5 to 1 g. The diseases were systemic lupus erythematosus in 9 cases, systemic necrotizing angiitis in 5 cases, Behçet's disease in 4 cases, Horton's disease in 1 case and dermatomyositis in 1 case. The rationale for this treatment was resistance to previous therapies in 9 patients, initial severity of the disease in 8 and the need for prompt reduction of corticosteroid dosage owing to side-effects in 3 other patients. No death or haemorrhagic cystitis was observed over a cumulative 120 month-patients period. Bacterial infection occurred in 6 cases, including pneumonia (3), and 1 case each of staphylococcal septicaemia, purulent meningitis and urinary infection: none of these infections were life-threatening, and all were cured without sequelae. The drug was well tolerated biologically, except for a moderate fall in neutrophils, lymphocytes and platelets. The systemic disease was stabilized or improved in 16 patients. It remained active and required corticosteroids at the same dosage level in 4 cases. It is concluded that bolus injections of cyclophosphamide are well tolerated in short-term treatments but that their effectiveness must be confirmed by controlled studies.

Adolescent↗
PubMed1988

Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients.

Factors influencing the prognosis were studied in 165 patients with polyarteritis nodosa (PAN) and Churg-Strauss angiitis. One hundred and forty-seven of the patients fulfilled histological and/or arteriographic diagnostic criteria, and in 18 patients the diagnosis was based on clinical criteria. The patients' mean age on diagnosis was 48.4 +/- 16.4 years. The main symptoms were fever (69%), weight loss (66%), arthritis (44%), mononeuritis multiplex (67%), cutaneous signs (46%), renal involvement (26%), gastrointestinal symptoms (31%), asthma (29%), hypertension (31%) and cardiac failure (18%). Ninety-two per cent of the patients survived for at least 1 year after diagnosis of the disease, 79% for 2 years, and 63% for 5 years. The immediate causes of death were gastrointestinal bleeding or peritonitis in 11 cases, pancreatitis in two, renal insufficiency in six, cardiac failure in five, infectious complications in four, stroke in three and other causes in 11. We studied the prognosis of necrotizing angiitis in relation to clinical symptoms and laboratory findings. The association of four conditions were associated with a poor prognosis: age over 50, gastrointestinal problems, cardiomyopathy and renal signs. The survival rates in patients with these conditions were: for gastrointestinal problems, 55% 5-year survival (versus 67%); and for age over 50, 68% 3-year survival (versus 78%; p less than 0.09). One hundred and fifty-nine patients were treated with steroids for at least 18 months. Forty-eight also received cytotoxic agents (27%) and 46 plasma exchange. Patients who were treated with plasma exchange and prednisone were randomly assigned to additional treatment with cyclophosphamide. Survival rates were comparable in both groups.

Adult↗
PubMed1988

[Behçet's disease, a current disease].

The treatment of Behçet's is essentially symptomatic and depends on the severity of the manifestations. Colchicine is useful in the minor forms, especially the mucocutaneous, forms. It can be safely used at a dose of 1 mg/day as maintenance treatment to prevent or limit the acute episodes. Other immunomodulators have been proposed : levamisole, disulon, thalidomide, the indications for which are limited by the side effects. Non-steroidal anti-inflammatory agents are indicated in the articular forms. Steroid therapy remains essential in the severe neurological and ophthalmological forms at an initial dose of 1 mg/kg/day. It can be preceded by the intravenous bolus administration of high doses of methylprednisone. Immunosuppressants are useful in the same indications. Although some authors propose them right from the start, we prefer to reserve them for second line treatment in the event of steroid dependence or recurrence, because of their short-term infectious and long-term oncogenic risks. Cyclosporin, which has been proven to be effective by randomised studies, is difficult to manage and responsible for complications, in particular renal. It can therefore only be used as second line treatment by experienced users. Plasmapheresis is reserved as an emergency procedure for functionally threatening forms. Venous or arterial thromboses justify longterm anticoagulation, possibly associated which platelet anti-aggregants. In this chronic disease, the quality of follow-up and the patient's cooperation are essential in order to intervene rapidly and to detect as early as possible the complications of the disease and of the treatment.

Adolescent↗
PubMed1988

[Neurological manifestations of Behçet's disease].

Characterised classically by the association of buccal and genital ulceration and uveitis with hypopyon, Behçet's disease has many other manifestations, amongst which the neurological ones (often referred to as Neuro-Behçet) are important in view of their frequency and their gravity. Anatomically, it produces a subacute haemorrhagic and necrotising meningo-encephalitis, which most typically effects the hypothalamus and brainstem. Clinically, there is extreme polymorphism, central manifestations being the most frequent: seizures, organic brain syndromes, disorders of consciousness, aphasia, hemiplegia, cranial nerve palsies, pseudobulbar and extrapyramidal syndromes and meningism. The peripheral nerves and muscles are rarely affected. Alongside Neuro-Behçet per se, attention has recently been directed to the various cerebro-vascular manifestations, dominated by venous thrombosis. A review of the principal neurological manifestations is given, with comment on anatomico-pathological aspects, clinical presentation, investigational techniques, diagnostic difficulties, prognosis, and treatment.

Behcet Syndrome↗
PubMed1988

[Venous thrombosis in Behçet's disease].

Venous lesions in Behçet's disease (BD) were defined by Adamantiades and represent one of the most suggestive signs of the disease. They are occasionally the first sign of the disease and are frequently the basis for the diagnosis in a case of recurrent thrombosis in a young subject, the preferential context of BD. Involvement of superficial vessels is virtually constant. Venous vasculitis is responsible for non-specific hypersensitivity and erythema nodosa, which constitute some of the major diagnostic criteria. Ocular periphlebitis is one of the elements responsible for posterior uveitis. The originality of the venous involvement is due to the involvement of deep territories. Any vein may be affected, but the remarkable features are the size of the thrombosed vessels: superior and inferior vena cava, iliofemoral veins and the unusual site of the involvement: supra-hepatic veins, cerebral vessels, etc. Inferior vena cava thrombosis may be associated with aneurysms of the pulmonary arteries in the context of Hughes-Stovin syndrome. Cerebral phlebitis, which can now be identified more easily by means of digital angiography, is responsible for a typical picture: headaches, bilateral papilloedema and raised CSF pressure. The classical pictures of optic chiasmatic arachnoiditis and so-called benign intracranial hypertension actually correspond to unrecognised phlebitis. They may also be associated with other neurological lesions. In one half of cases, phlebitis cutaneous manifestations. However, they may precede the diagnostic signs or may occur very late in the course of the disease. They are recurrent and affect a number of different territories.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-Inflammatory Agents↗
PubMed1988

[Medical treatment of Behçet's disease].

The treatment of Behçet's disease is essentially symptomatic and depends on the severity of the manifestations. Colchicine is usefull in the minor, particularly mucocutaneous forms. It can be safely used at a dose of 1 mg/day as maintenance treatment to prevent or limit the acute episodes. Other immunomodulators have been proposed: levamisole, disulon, thalidomide, the indications for which are limited by the side effects. Non-steroidal anti-inflammatory agents are indicated in the articular forms. Steroid therapy remains essential in the severe neurological and ophthalmological forms at an initial dose of 1 mg/kg/day. It can be preceded by the intravenous bolus administration of high doses of methylprednisolone. Immunosuppressants are useful in the same indications. Although some authors propose them right from the start, we prefer to reserve them for second line treatment in the event of steroid dependence or recurrence, because of their short-term infectious and long-term oncogenic risks. Cyclosporin, which has been proven to be effective by randomized studies, is difficult to manage and responsible for complications, in particular renal. It can therefore only be used as second line treatment by experienced users. Plasmapheresis is reserved as an emergency procedure for functionally threatening forms. Venous or arterial thromboses justify long-term anticoagulation, possibly associated with platelet anti-aggregants. In this chronic disease, the quality of follow-up and the patient's cooperation are essential in order to intervene rapidly and to detect as early as possible the complications of the disease and of the treatment.

Adjuvants, Immunologic↗