[Anterior tibial enthesopathy disclosing inflammatory rheumatism related to HLA B27 antigen in a child].
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Biomedical subjects
Publications and source records attributed to M Alcalay.
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Eighty-seven patients (mean age 73 years) with histologically proven Horton's disease were followed up in 5 different specialized hospital units from 1970 to 1984. Except for one patient treated exclusively with antimalarials, all were under corticosteroids. The mean initial dose of prednisone or equivalent was 0.7 mg/kg/day and the mean maintenance dose, 10 mg/day. Steroids could be discontinued in 21 cases after 36 months on average; the other patients could not be weaned, even though 4 of them had been on steroids for more than 10 years. Side-effects were noted in 43 cases. Twenty-four patients died, mostly of cardiovascular diseases. Survival rates were 89 +/- 6.35% at 1 year, 60.2 +/- 8.7% at 5 years and 48 +/- 11.3% at 10 years. In the long run, mortality in our series proved to be exactly the same as in the general population. Nineteen patients had ocular manifestations of giant cell arteritis always from the onset, except for a fall in visual acuity; 26 relapses were observed in 18 patients, either during reduction of steroid dosage (21 cases) or after withdrawal (5 cases). In 2 cases histology showed typical lesions of giant cell arteritis after 41 and 50 months respectively under corticosteroid therapy.
From 1960 to 1984, 78 new patients with progressive systemic sclerosis were followed up: 60 women and 18 men whose ages ranged from 20 to 83 years, with a mean age of 58 years. Twenty nine are known to be dead and 3 were lost of follow-up. Forty six have been followed up to the present time for a mean period of 5 years. The cumulative survival rates were 88 +/- 7 p. 100 at one year, 62,5 +/- 11,5 p. 100 at five years and 50,5 +/- 15 p. 100 at ten years. These figures are significantly different from those found in a matched group from the French general population. Nine features at the time of diagnosis which might influence prognosis were studied. Seven factors apparently have not affected prognosis: sex, age, time elapsed between initial symptom and definitive diagnosis, location of scleroderma, blood pressure, erythrocyte sedimentation rate and creatinine clearance. On the other hand, survival declined significantly faster in the 28 patients with anemia than in the 50 patients without anemia (P less than 0,001). Similarly, the 47 patients with radiological pulmonary involvement or pulmonary function abnormalities were at significantly higher risk for death than the 31 patients without interstitial pulmonary fibrosis. Anemia and pulmonary involvement are predictors of mortality and important prognostic tools in the management of the different drugs that have been recommended for the treatment of patients with systemic sclerosis.
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Meningoradiculitis can present as a very short episode of radicular pain in addition to vertebral column pain without stiffness, as described in these three cases. The diagnosis requires examination of the cerebrospinal fluid, which should be a routine part of the diagnostic work up for back pain when an inflammatory etiology can not be identified.
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Seven patients with advanced multiple myeloma, failing to respond (1 case) or relapsing (6 cases) after conventional chemotherapy with melphalan, cyclophosphamide and prednisone, were treated with double half-body irradiation. Two patients died before the irradiation could be completed. The five patients who received the total dose of irradiation obtained a reduction in their tumour mass of between 40 and 80%. There was an incomplete and inconstant effect on pain and functional status. The haematological toxicity was marked and one patient died after 7 months from marrow failure. One of the four remaining patients relapsed after 3 months and the other three are still in remission after 6, 8 and 10 months. In the present state of knowledge, double half-body irradiation should be considered to be a form of salvage treatment for myelomas not responding to chemotherapy.
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A retrospective study allowed us to work out an original method for an essentially clinical approach of the correlation between femoral neuropathy and diabetes mellitus. Nine parameters were chosen for this purpose and a score was developed. From this score, three categories were defined: in each, the existence and etiological probability of diabetes mellitus are discussed. This clinical approach is a simple means of establishing more accurate guidelines for complementary investigations.
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The authors report on four cases of aseptic osteonecrosis of the vertebral body in adults (VON). Among these, one patient, in whom histological data were available, presented with a slow spinal cord compression syndrome (SSCCS). This complication is uncommon since only one other similar case has been reported so far (4). VON thus remains an underestimated etiology of SSCCS by non-tumoral compression fracture of the vertebral body. Diagnosis is based on the roentgenograms which evidence osteocondensation with "gaseous dissection" of the vertebral body, the latter being considered as pathognomonic of this disease. The etiopathogeny is still unknown. Treatment is purely symptomatic and the course has always been favorable.
In both Still disease and Wissler-Fanconi syndrome, onset may occur in adulthood and the clinical picture includes acute, often migratory, polyarthritis, polymorphic exanthema and a major inflammatory syndrome with fever (often intermittent) and high polymorphonuclear leukocyte counts. The grounds for this double designation are not clearly stated in the medical literature: does it refer to two separate entities or not? Some authors plainly stand for a unitary opinion. We report on seven cases in adults, five of which recovered without sequellae, while two developed respectively into rheumatoid arthritis and seronegative polyarthritis. We therefore propound a dualistic concept although, in the present state of our knowledge, there is no means of predicting at onset whether the disease will resolve or progress towards polyarthritis. We suggest that the term Still disease be used only when chronic and/or destructive arthritis develops. Among the forms which are neither chronic nor destructive, there seems to be a number of cases in allergic patients with poor tolerance of chrysotherapy. Some of these patients could recover after the cure of an often latent focal infection, usually of the upper respiratory tract, ear or oral cavity, resistant to antibiotics. In the other cases, corticosteroid therapy is usually very effective in arresting an exacerbation of the disease.
The sub-population of T-lymphocytes which express the DR antigen was studied in 48 patients with rheumatoid arthritis and in 42 controls. The sub-population of T-lymphocytes which express DR was found to be larger in the patients with rheumatoid arthritis. This increase is even more marked in cases of rheumatoid arthritis with high erythrocyte sedimentation rates and with a high level of immune complexes.