Autosomal dominant undifferentiated spondyloarthropathy not related to the HLA system.
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Biomedical subjects
Publications and source records attributed to A Gaucher.
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The association of rheumatoid arthritis proven by means of synovial biopsy with pulmonary sarcoidosis proven by means of bronchial biopsy, occurred in a 58 year old woman. Corticosteroid therapy resulted in complete resolution of sarcoidosis but only slight improvement of the rheumatoid arthritis, which was secondarily treated with methotrexate with a successful outcome. Only two similar cases have been reported with simultaneous histological proofs of both diseases.
Radiographic, CT, bone scan and MR patterns of the infarcts of the metaphyses and diaphyses of tubular bones and their main complications are presented. Bone marrow infarcts are located in the medullary cavity of long bones. They most often involve the lower limbs. They are commonly associated with necrosis of the epiphyses and have common origin. In contrast to necrosis of the epiphyses, bone infarcts may present specific complications. They are most often detected incidentally, because of their clinical latency. Radiographic and CT changes appear late, showing localized areas of increased density with irregular rims, or sharply demarcated shells of calcification. Earlier changes are demonstrated by bone scan and MRI. MR pattern is characteristic, showing an area of hyposignal on T1- and T2-weighted sequences, with irregular rims, and sometimes small areas of fat signal. Their complications include cystic formation and malignant degeneration (sarcoma and particularly malignant fibrous histocytoma). An unusual complication, infection involving bone infarcts is also presented.
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OBJECTIVE: To investigate the articular toxicity of 2 aluminum derivatives, one insoluble (hydroxide) and/or the other soluble (lactate), after a single administration in rabbits and rats. METHODS: First, aluminum levels in plasma, urine, synovial tissue, liver and kidney were measured in saline treated rabbits and 1 to 2 days after an articular injection of 75 mg of aluminum compounds into their right knee. The methodology used was argon plasma emission spectrometry. Thereafter, the joint toxicity of aluminum lactate at the same dose regimen was evaluated for 2 days by a qualitative histological examination of synovial tissue and articular surfaces and a colorimetric assay (1,9-DMB) of patellar articular cartilage proteoglycan content. Secondly, the single injection of 50 mg of aluminum derivatives as an inducer of inflammation was studied in the rat subcutaneous air pouch, a model for a synovial-like space. Leukocytes and eicosanoids levels were measured in pouch washout fluids from 1 to 72 h after injection. RESULTS: After injection into rabbit knee, aluminum lactate largely distributed within the body while hydroxide remained locally. However, aluminum lactate resulted in perivascular edema, sparse infiltration of inflammatory cells in the synovium and a hemorrhagic effusion. Proliferation of the synovial cell layer coexisted with an apparent loss of proteoglycan in superficial zones of tibial and femoral cartilages when patellar proteoglycan content remained unchanged. Aluminum hydroxide did not affect joint structures. In the air pouch experiment, aluminum lactate increased prostaglandin E2 (PGE2) levels from 3 to 10 h after its injection and less intensively leukotriene B4 (LTB4) levels after 6 h, in the absence of leukocytes migration into the cavity. In contrast, aluminum hydroxide increased leukocytes count in pouch-washout fluid from 3 to 24 h after its injection when PGE2 and LTB4 levels were little modified. CONCLUSION: Although some differences attributable to dissimilarities in the experimental model used, aluminum compounds, even in a soluble form, may damage joint structures either directly or through stimulating the secretion of eicosanoids by synovial-like cells.
Musculoskeletal infections by fungal agents are uncommon conditions. Pseudallescheria boydii is an ubiquitous saprophytic fungus frequently involved in maduromycosis but rarely in septic arthritis. We describe a case of Pseudallescheria boydii destructive suppurative arthritis and osteomyelitis of the knee in an adult Guadeloupean man. Osteoarticular infections due to Pseudallescheria boydii are reviewed.
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The value of scanning electron microscopy (SEM) in the study of crystals in articular diseases is underlined in several cases of examination of joint fluid, or crystal deposits in articular or periarticular tissues obtained by percutaneous or surgical treatment with chemical and crystallographic correlations. Apatite crystals. Two deposits of hydroxyapatite of the rotator cuff were studied by SEM, crystallographic techniques and chemical analysis. SEM study showed spherical aggregates of various size. Urate crystals. Three tophi were observed by SEM, with crystallographic techniques and chemical analysis. Their needle-shape and their great size (20 m) were characteristic. Calcium pyrophosphate crystals. In a case of typical clinical and radiological features, examination of joint fluid, with chemical correlation showed shorter and thicker crystals than those or urate. The precise identification of crystals is based on sophisticated crystallographic techniques such as X-ray diffraction, although SEM allows an accurate and quite simple morphologic study, most often sufficient.
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In order to determine the prevalence of dialysis-associated arthropathy (DAA) and what factors favour its development, we conducted a survey in 19 centres in northeastern France, of all patients receiving haemodialysis for over 10 years (171). A diagnosis of DAA was made in 84 patients (49%) by two investigators, using as criteria single or combined presence of carpal tunnel syndrome (32%), erosions and bone cysts of the large limb joints (33%) and destructive spondylarthropathy (14%). The 84 patients with DAA were compared with the 87 dialysis patients free of these clinical or radiological abnormalities. The affected patients were significantly older at the start of dialysis than unaffected patients. The risk of developing carpal tunnel syndrome increased with the duration of dialysis. Amyloid deposits were found in carpal tunnel tissue obtained from 24 of the 39 operated patients (62%) during surgery. Destructive spondylarthropathy was significantly associated with the presence of disc calcifications and more frequent in AN 69-treated patients in whom secondary hyperparathyroidism appeared to be more severe. The use of an AN 69 membrane for at least 90% of the dialysis period (in 15 patients) was not associated with a lower prevalence of DAA. We found that after 10 years of haemodialysis DAA occurred whatever type of membrane was used and the prevalence increased with the patient's age and the duration of dialysis.
Since the antipyretic and probably the analgesic effects of paracetamol are, at least in part, centrally mediated, its plasma and cerebrospinal fluid (CSF) concentrations were measured in 43 patients with nerve-root compression pain. Each subject was given a short i.v. infusion of 2 g propacetamol, a prodrug which is hydrolysed to paracetamol within 7 min. Single blood and CSF samples were drawn concomitantly in each patient at intervals between 20 min and 12 h. Maximum CSF drug concentrations were observed at the 4th hour, subsequent concentrations exceeding those in plasma. The elimination half-life of paracetamol calculated from pooled data was shorter in plasma (2.4 h) than in CSF (3.2 h). The time-course of paracetamol in CSF may parallel that of analgesic effect.
Multiple calcifications and ossifications of tendons are, as a rule, associated with similar lesions of other articular and/or periarticular structures. The nature and multiplicity of these lesions gives them an unquestionable diagnostic and nosological value. Multiple calcifications are part of the apatite arthritis or of diffuse articular chondrocalcinosis. Multiple ossifications of tendons often suggest Forestier's disease and ankylosing spondylitis.
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