Fatal agranulocytosis in sulfasalazine treated rheumatoid arthritis.
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Biomedical subjects
Publications and source records attributed to J Pourel.
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Extensive immunological investigations were performed in a patient with definite seronegative rheumatoid arthritis who developed hypogammaglobulinemia in the course of gold therapy. The data obtained suggest the presence of acquired maturation abnormalities in the B cell compartment.
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Articular chondrocalcinosis is a well-defined radiological entity with or without clinical manifestations. A familial form was described by Sitaj and Zitnan in 1957. Attention has mostly been paid to chondrocalcinosis associated with metabolic diseases and to "sporadic" chondrocalcinosis, usually due to ageing of the joint. Familial chondrocalcinosis, of which more and more cases are reported, is increasingly under study and is used as a model for pathogenic research. It already appears that familial chondrocalcinosis, of dominant autosomal transmission, is a new metabolic disease.
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Isotope investigation of reflex sympathetic dystrophy is not limited to an evaluation of bony up-take, it also includes examination of the early dynamic scintigraphy of the vessels. The late views of bone scans reflect, above all, the bone's affinity for phosphate complexes i.e. the degree of osteoblast activity. Generally, dystrophies, independent of their site, show increased locoregional uptake, often quite intense, which appears early in the course of the disease. This supports histopathological findings. There are several advantages in using the bone scan in the investigation of reflex dystrophy: early diagnosis before the development of radiological signs, precise evaluation of the local extension of the dystrophic process and detection of the incidence of multifocal forms, follow-up of the course of the disease, definition of new clinical forms: patchy algodystrophy partial, decalcifying dystrophy, sub-radiological dystrophy, the aetiology in certain sites (especially the hip). Early dynamic scans that dystrophy is accompanied by an increase in the vascular compartment and decreased circulatory flow, a sign of the local stage of the disease. There is also an increase of the interstitial compartment, greater than that of the vascular compartment, explaining the presence of edema. The pathophysiological information gained from dynamic studies is matched by the therapeutic information: evaluation, or even prediction, of the effect of a given drug (cortisone, calcitonin).
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Protein binding of salicylates was determined in 16 control subjects and 27 patients suffering from rheumatoid arthritis. Results obtained after separation of the free and bound fractions by dialysis to equilibrium and measured by spectrofluorometry were analyzed using a new mathematical model. In correlation with the decrease in plasma albumin concentrations, a decrease was found in the protein binding of salicylates in patients with rheumatoid arthritis. This phenomenon was less marked in therapeutic zones and was related to the degree of severity of the inflammatory syndrome. The binding capacity per albumin molecule at saturation was decreased in patients suffering from advanced forms, suggesting that the changes seen were not due solely to quantitative variations in serum albumin levels. This study confirms the value of the determination of free salicylate levels in patients suffering from inflammatory rheumatic disorders.
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The uptake of Tc-99m-diphosphonate was studied topographically and quantitatively on 258 diseased hips. For many diseased hips, bone scintigraphy provides items of information that complement the clinical, radiological, and biological data, with which it should always be compared. The localization, extent, and intensity of the uptake differ greatly according to the hip disorder studied, and depend not only on the cause of the lesion but also on its stage of evolution. Bone scintigraphy can shed light on the etiologic diagnosis of a painful hip at the beginning of its evolution, before definite roentgenographic signs appear, thanks to some very precise characteristics of the uptake, this is the case for transient osteoporosis of the hip, aseptic osteonecrosis, Legg-Perthes disease, early arthritis of the hip joint, and stress fractures of the femoral neck or of the pubic rami.
The phenotypes Bf were studied in 50 patients (42 men and 8 women) with definite ankylosing spondylitis and in certain of their relatives. The alleles BfF and BfS were most frequently encountered and the rare allele BfF1 in one case only. There may exist a preferential transmission of the haplotype HLA B27- Bf S. In 11 patients without B27 antigen, the distribution of the phenotypes was similar to that in a control group. It is probable that the locus Bf cannot be used as a preferential marker for ankylosing spondylitis.
Impulsion polarography with anodic redissolution, a very sensitive means of dosage that is easy to repeat and more selective than atomic absorption, makes it possible to affirm the presence of bismuth in the bones after oral or parenteral treatment in 6 patients, 5 of whom suffered from osteoarthropathies that could be linked, given their nature, to the treatment administered, though this could not be proven. The presence of bismuth in the bone tissue of these patients must be considered in the physiopathological discussion of their osteoarthropathies, especially since none of them showed signs of encephalopathies.
A study of HLA complex (HLA A, B, C, DRw, Bf, chiddo) in monozygous twins suffering from ankylosing spondylitis with different clinical courses. All the markers studied were identified. The results would tend to suggest that hereditary factors are not alone responsible in the course of ankylosing spondylitis. Furthermore, they are not the sole causative factor of the disease since the literature contains reports of discordant pairs of twins.