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Biomedical subjects

A Gaucher

Publications and source records attributed to A Gaucher.

At least 19 recordsLinked to original sources

[Ankylosing spondylitis in monozygous twins. Study of HLA complex (author's transl)].

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.

Adolescent

Measurement of bismuth in bone by pulse polarography with anodic stripping voltammetry. Application to two cases of arthropathy.

Pulse polarography with anodic stripping has shown the presence of very high levels (2 005 microgram/kg and 6 760 microgram/kg) of bismuth in bone of two patients treated with bismuth, one parenterally and the other orally. The presence of high levels of bismuth in bone of these patients must be considered in the etiology of their athropathies. Pulse polarography with anodic stripping now permits very precise measurement of bismuth impregnating bones.

Aged

[Study of cellular elements in synovial fluid in scanning electron microscopy].

Scanning electron microscopy allows a tridimensional morphological study of the cellular elements of the pathological human synovial fluid. The cells are grouped into two populations: macrophage cells and round cells, whose differentiation is more difficult: polynuclears, lymphocytes and synovial cells. The combination of scanning electron microscopy and X-ray diffraction makes it possible to describe and indentify crystals of sodium urate and of triclinical, dehydrated calcium pyrophosphate.

Arthritis, Rheumatoid

[HLA DRw in ankylosing spondylitis].

The association of ankylosing spondylarthritis with the B locus and more specifically with the B 27 antigen, is the closet known for any illness. The absence of linkage with the DRw antigens studied during this project, in 50 patients, can give rise to the hypothesis that spondylarthritis is associated with determinants situated on the B lymphocytes, linked to the HLA-B locus.

Adult

[Scintigraphic characteristics of coxopathies: etiologic importance].

Morphological and quantitative studies of the uptake of diphosphonates labelled with technetium 99m were done on 179 pathological hips : 106 aseptic osteonecroses of the femoral head, including 20 at an early stage, 11 algodystrophias (transitory osteoporoses of the hip), 20 coxarthrosis, 16 cases of Paget's disease, 12 isolated malignant lesions of the femoral neck. The location, extent and intensity of the uptake are very variable and depend not only on the etiology of the lesions but also on their stage of evolution. Bone scintigraphy provides, for numerous pathological hips, pieces of information which complete the results of the clinical, radiological and biological study, with which it should always be compared. It can shed light on the etiologic diagnosis of a painful hip at the beginning of its evolution, before the appearance of rare x-ray signs, thanks to certain very precise characteristics of the labelling. This is the case for algdystrophias, osteonecroses, coxitis and strain fractures of the femoral neck.

Femur Head Necrosis

[Scanning electron microscopy study of the synovial membrane in rheumatoid arthritis].

A comparative study under the scanning electron microscope of rheumatoid synovial membranes, 5 arthrosic synovial membranes, one tuberculous membrane, and 3 normal synovial membranes showed the pathological changes in the synovial membrane due to rheumatoid arthritis: Inflammatory aspect of the synovial fringes, surrounded by turgid and proliferative villous processes. Granular appearance of the endo-articular surface like a "pebble beach". Dome-shaped synoviocyte layer, standing out well above the subjacent intimal layer, associated with small round cells, also with a raised margin, covering most of the endo-articular surface. Plasma membrane of the superficial synovial cells, covered with numerous and various differentiations and abundant microvillous processes. Reticular deposits or layers, covering the apical poles of the synoviocytes.

Arthritis, Rheumatoid

[Interpretation of and indications for bone scintiscans].

The interpretatiton of a bone radioisotope scan requires perfect knowledge of normal skeletal appearances, shadows outside the bones and the various methods of expression of bony lesions: generally hyperfixations but sometimes isofixations or even hypofixations. A bone scan permits one to make an overall assessment of multiple bony lesions, determine the extent of a lesion, detect bony complications of a disease or treatment and seek the bony origin of a pain.

Bone Diseases

[Lumbosacral arachnoepiduritis. Clinical and etiologic aspects].

A series of 120 cases of lumbo-sacral arachno-epiduritis was studied from the clinical and etiological points of view. The recruitment of the cases and their classification were based on purely radiological criteria. The clinical symptoms were various, non-specific and without correlation with the radiological type. The course was chronic with a change in the distribution of the symptoms. The influence of the method of examination was discussed in the 9 cases discussed after a first myelography without surgical operation. All the forms suggesting epidural lesions were post-operative and observed mainly in cases of laminectomy.

Arachnoiditis

[Gamma heavy chain disease associated with rheumatoid arthritis. Spontaneous disappearance of the pathologic protein].

The authors report the chance discovery in a man aged 53 years with serum positive rheumatoid arthritis, of gamma 3 heavy chain disease. The originality of this case depended on the absence of any detectable lymphoid proliferation and on the transient character of the pathological protein. Only the course after a long follow up will determine whether there is no incipient lymphoid proliferation and whether the heavy chain disease has disappeared permanently.

Arthritis, Rheumatoid

[Interactions of salicylates and other nonsteroid anti-inflammatory agents].

The salicylates are frequently associated in every day treatment with other non-steroid anti-inflammatory drugs, which justifies a study of their interactions. Animal pharmacodynamics, although interesting, is of little practical interest. Pharmacokinetic research seems to indicate that aspirin reduces the bio-availability of other non steroid anti-inflammatory drugs, which has not yet been confirmed clinically. It is still premature to conclude, but the already established facts suggest the continuation of such studies.

Animals

Hereditary diffuse articular chondrocalcinosis. Dominant manifestation without close linkage with the HLA system in a large pedigree.

Thirty-nine members of one family, covering three generations, were HLA-typed. Twenty-five suffered from primary diffuse articular chondrocalcinosis, and all had the same dominantly transmitted autosomally controlled disease. This was characterized by acute articular attacks, which always started before the age of 35, and radiologically by typical cartilaginous and fibrocartilaginous deposits associated with para-articular calcifications. The lesions were both peripherally and axially generalized. None of the 28 HLA antigens tested seemed related to the disease, nor did the disease segregate with an HLA haplotype.

Adult

Identification of microcrystals in synovial fluids by combined scanning electron microscopy and x-ray diffraction : application to triclinic calcium pyrophosphate dihydrate.

We report a method for studying microcrystals which combines scanning electron microscopy, electron-dispersive X-ray analysis, and X-ray diffraction. Application of all three techniques to the same crystalline bodies permits correlation of their three-dimensional morphology at high magnification with unambiguous identification by means of their crystallographic properties. The method was applied to triclinic calcium pyrophosphate dihydrate.

Calcium Pyrophosphate

[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

[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

[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