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Biomedical subjects

A Gaucher

Publications and source records attributed to A Gaucher.

At least 163 records · Page 9Linked to original sources
PubMed1977

[HLA-B27 antigen and alkaptonuria].

Study of urinary homogentisic acid and a determinantion of group HLA were carried out for 36 members of a family spread over three generations with three cases of ochronotic rheumatism in the second generation. Alkaptonuria was discovered in seven other subjects, six of them members of the third generation: urinary elimination was poor, less than 0.60 g/24 hours. There is a certain degree of consanguinity in the family studied here and these findings do not therefore rule out a recessive autosomal transmission of the alkaptonuria. They do however lead to the consideration that alkaptonuria may sometimes be found in heterozygotic subjects. A genetic relationship between HLA complex and alkaptonuria can only be claimed with difficulty from this familial study, but the high frequency of B 27 antigen (29 out of 36 members carring it) leaves room for the hypothesis that the B 27 gene, or more precisely a gene associated with the B 27 gene, plays a part in the development of ochronotic rheumatism.

Adult↗
PubMed1977

[Identification of the crystals observed in the destructive arthropathies of chondrocalcinosis].

Study of the synovial membrane and cartilage demonstrating two destructive arthropathies of the knee diagnosed in subjects with articular pseudogout. Scanning electron microscopy reveals the presence of many crystals on the surface of the cartilage and the synovium and in the depth of the cartilage. These can be grouped into two families on the basis of dimensions and morphology. The first consists of those shaped like arrowheads and are large (80-100 microns long.) They were formally identified using Weissenberg's technique; dihydrated calcium hydrogenophosphate (CaHPO4.2H2O) is involved here. In the second family the crystallogenesis is different (prism or lozange-shaped) and the crystals themselves have not been identified with certainty because of their tiny size (20 microns). The hypothesis is proposed that the crystals of dihydrated calcium hydrogenophosphate properly belong to the destructive arthropathies of pseudogout; while not necessarily its cause, they may help explain its development.

Aged↗
PubMed1976

[Gastric mucosa in patients with rheumatoid polyarthritis treated by anti-inflammatory agents. Clinical, radiological, endoscopic, anatomopathological and ultrastructural study].

One hundred patients (50 male, 50 female) suffering from rheumatoid arthritis, treated for more than 6 months with steroid and/or non-steroid anti-inflammatory agents were studied using barium X-rays and gastro-fibroscopy. In 69, one or more gastric biopsies were taken during the endoscopy. The study showed a marked preponderance of ulcerous lesions in males, 9 of the 13 iatrogenic ulcers being found in this sex. It also confirmed the frequency of gastritis and the absence of any correlation between clinical signs and radio-endoscopic findings as well as between the latter and histopathological data. An ultra structural study carried out in 20 cases revealed focal lesions of the clear cells of the neck of the fundus and antral glands and, in 4 cases, marked changes in the gastric endocrine cells.

Anti-Inflammatory Agents↗
PubMed1976

[Diagnostic value of localized hypofixations in the radioisotope scanning of bones].

The scintigraphic appearances of all localized, evolutive bone lesions, whatever their nature, is usually a "hot spot", that is a zone of hyperfixation of the radioactive material. False negatively scintigraphs are, however, noted: the scintigraphic image appears normal, without a zone of hyperfixation, although radiographs of the skeleton are pathological. The 8 cases presented in this article demonstrate that certain bone lesions (aseptic osteonecrosis and malignant destruction of bone) may sometimes be represented as real zones of hypofixation, as veritable "cold spots". These areas of hypofixation result from either a local interruption in the vascularization, which prevents the isotope from reaching the bony structures, or from a quantitative insufficiency of the bony tissue, this being replaced by neoplastic tissue which, in the cases studied, fixed labelled Bleomycin or iodine-131. These areas of hypofixation and any absences of fixation really are the scintigraphic images of areas of osteolysis or of aseptic osteonecrosis but they are generally masked by hyperfixation around the lesion that results in reactional osteogenesis and hypervascularization.

Aged↗
PubMed1976

[Pigmented villonodular synovitits of the hip: ultrastructure and aspects on scanning electron microscopy].

The authors studied one case of pigmented, villonodular synovitis (PVNS) of the hip by means of optical microscopy and by transmission and scanning electron microscopy. Scanning electron microscopy showed that the surface of the PVNS is completely different from that of rheumatic synovitis, in particular that of rheumatoid synovitis. The composition and the cellular morphology of the outer layer of the PVNS appear, however, to be similar to those of normal synovial membrane of arthrosic synovial membrane. Clumps of red corpuscles, enclosed in a fibrin network, were visible on the surface of the PVNS in a way that the authors has never seen previously in the 19 other human synovial membranes, normal and pathological, they had studied in this way. The totality of the microscopic findings confirms the importance of the role played by the intra-articular and intra-synovial haemorrhages, and by the macrophage reactions that follow, in the development of the lesions that characterize PVNS.

Adult↗
PubMed1976

[Contribution of scanning electron microscopy to the study of normal and pathological human synovial membrane].

Scanning electron microscopy permits three-dimensional morphological study of the outer layer of the synovial membrane. This article concerns the study of 15 surgical samples of normal and pathological human synovial membranes: 3 normal synovial membranes, 6 inflammatory synovial membranes, 5 arthrosic synovial membranes, 1 tuberculous tenosynovitis. The samples were washed, fixed in a mixture of glutaraldehyde and iso-osmotic paraformaldehyde, dehydrated in increasing concentrations of alcohol and at the critical point, and then subjected to double metallization (aluminium and gold). Certain portions were treated with hyaluronidase. Hypertrophy of the villi, considerable in inflammatory synovial membranes, minimal in arthrosic synovial membranes, and absent in the normal synovial membrane, was confirmed. The outer layer is composed of cells of different sizes with spaces betwen, in which images indicating intercellular bridges can just be seen. The surface of the cells is irregular and covered with numerous microvilli. The arthrosic synovial membrane is poorly with large cells; in contrast in an inflammatory synovial membrane they are numerous, globular, and project from the surface.

Arthritis, Rheumatoid↗
PubMed1976

[Use of Dodger filters in osteoarticular radiology].

Dodger filters represent considerable progress in the harmonization of the contrast in radiography by modulation of the incident Y-ray beam. The originality of these filters is related to three factors: the mobility of the filter elements, their shape, and in particular the presence of a specific optical system that makes it possible, by demonstrating the filter lines with a luminous ray, to predict and adapt the attenuation effects obtained. Osteo-articular radiology can now be viewed in a new light, as it is possible with Dodger filters to obtain on a single fim, valid images of the whole of the bony structure of a region as well as of the corresponding soft tissues.

Arthrography↗
PubMed1976

[Interaction of salicyl compounds and other non-steroidal anti-inflammatory agents: pharmacokinetic study of phenylbutazone-aspirin combination in man].

The pharmacodynamic interaction between phenybutazone and aspirin was studied in 9 patients. The levels of phenylbutazone were estimated by gas-chromatography and verified by mass-spectrometry. When phenylbutazone was administered together with salicylates a decrease in the bioavailability of phenylbutazone was demonstrated, but there was no significant variation in the absorption and elimination constants or in the half-life. The variations were much less than those observed with other nonsteroid anti-inflammatory drugs (indometacine, naproxen, fenoprofen). These interactions must be taken into account in the long-term treatment of cases of inflammatory rheumatism.

Adult↗
PubMed1975

[Deafness and Paget's disease].

Cases of deafness due to Paget's disease can be separated into two types : (1) deafness of a mainly mixed type in which progressive aggravation occurs particularly in the inner ear, and (2) perceptional deafness which progresses without involvement of the transmission apparatus. Among 35 hospitalized patients with Paget's disease, 21 of whom suffered cranial involvement, 18 cases of deafness related to the bone disease were discovered by means of systematic examinations. The deafness was of the mixed type in 11 cases and of the perceptional type in the other 7. Of the 11 patients with the mixed type of deafness with an ankylosis syndrome of the ossicles, 3 were operated upon : one of them underwent an operation to mobilize the stapes, and the two others underwent total stapedectomy followed by venous interposition and the positioning of a Teflon piston. The long-term results were frankly disappointing and did not suggest that these attempts at surgical treatment should be followed up. Calcitonin was employed in 9 patients (6 with mixed deafness and 3 with perception type deafness), with comparative audiograms in 5 cases, and was also not very effective : this lack of effect was a result of the long duration of the Paget's disease and of the deafness in the patients treated. The value of hormonal treatment in cases of deafness associated with Paget's disease will probably be in the prevention of this complication.

Aged↗