[Generalized amyloidosis revealed by vertebral compression].
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Biomedical subjects
Publications and source records attributed to B Delcambre.
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A measurement of the bony density of vertebrae and femur was carried out, in the course of a prospective study, in 99 patients between the ages of 60 and 75 years, living in northern France. 76 patients had no recognized problem of demineralization and 23 others were consulting for an evaluation of vertebral compression. The measurement of the bony density was done by biphotonic absorptiometry with a source of labelled Gadolinium. In women, a significant difference in the values obtained by absorptiometry in terms of the existence of vertebral compressions, symptomatic or not, is noted. A statistical study using the ROC curves method (Receiver Operating System) has enabled us to determine a threshold of high risk of vertebral fracture measured at 31 g or 0.85 g/cm2 in women, and 35 g or 0.85 g/cm2 in men. In addition, there is a significant correlation with the vertebral radiographical index as defined by Meunier. Among the various factors of influence which were studied, only the size and the morphotype seem to play an essential role in our study group. The comparative study of the densities measured in the femoral neck and the lumbar spine enabled us to find a very significant correlation in patients with as well as without compression (r = 0.714, p less than 0.001). Biphotonic absorptiometry proves therefore to be a method which may be used to define a group with a high fracture risk permitting to determine, as early as possible, therapeutic, curative and preventive measures.
Eighty patients studied for several years (average length of time: 76 months, 24 on hemodialysis for over 10 years), were the subject of a prospective study of osteoarticular manifestations. Clinically, these manifestations were found in 58 patients (72 p. cent). Among them, 15 suffered from inflammatory painful episodes (shoulders and fingers) and 10 presented a decreased finger mobility. Radiologically, it is possible to describe 7 types of specific images: erosive arthropathy of the fingers (8 p. cent), multiple carpal cavities (10 p. cent), cavity of the humeral head (9 p. cent), cervical spondylolisthesis (5 p. cent), multiple diaphyseal lacunae (1 p. cent). Some clinical and radiological manifestations are frequently associated and seem to represent a specific pathological group: dialysis in progress for more than ten years, carpal tunnel syndrome, inflammatory-type arthralgias, osseous cavities and spondylo-arthropathy. Biologically, there are numerous disturbances concerning the sedimentation rate, fibrinemia, aluminum blood level, phosphorus blood level, parathyroid hormone and beta-2-microglobulin blood level. The study of bone or synovium biopsies, carried out 20 times, permitted to discover amyloid deposits in 8 instances and microcrystals in 3 instances. The physio-pathogeny of these manifestations leads to evoke the role of many factors (aluminum, microcrystals, silicon, hyperparathyroidism, amylosis). The occurrence of a form of amylosis where a protein homologous to beta-2-microglobulin was isolated, appears to be feasibly the consequence of a bio-incompatibility of the dialysis membranes.
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Eighty patients undergoing longterm hemodialysis were evaluated for joint function and radiographic abnormalities. Mean age of the population was 53 years, and mean duration of dialysis was 76 months (24 patients underwent dialysis for more than 10 years). Fifty-eight patients (72%) had joint symptoms; nonspecific arthralgia was the most frequent. Fifteen patients had morning stiffness and interrupted sleep due to arthralgias (shoulders 9, fingers 3) and 10 patients had diminished finger mobility. Radiographic evaluation showed 7 classes of lesions which are not characteristic of renal osteodystrophy or degenerative lesions, including erosive arthropathies of the fingers (8%), multiple carpal bone cysts (10%), cysts of the humeral head (9%), cysts of the hip (11%), erosive spondyloarthropathies (9%), cervical spondylolisthesis (5%) and multiple diaphyseal lacunae (1%). Overall, 42 distinct radiological lesions were observed in a total population of 23 patients. Twenty-nine of these lesions occurred in patients undergoing dialysis for at least 10 years. Certain clinical and radiological patterns were frequently combined after dialysis for more than 10 years: carpal tunnel syndrome, inflammatory joint pain, bone cysts and spondyloarthropathies.
Clinical and radiological analysis of a population of 80 patients undergoing hemodialysis showed a high incidence of joint symptoms and radiological abnormalities which could not with certainty be attributed to renal osteodystrophy (Part 1). In this same population, laboratory and pathologic studies were performed. Blood chemistry proved abnormal in most cases (fibrinogen, aluminum, beta 2 microglobulin). Histopathologic studies (bone or synovial) were performed in 20 samples. Amyloid deposits were found in 8 samples, and crystal deposits in 3. We consider the idea that amyloid may be responsible for the observed lesions to be an interesting possibility. A better understanding of the behavior of different dialysis membranes may clarify the pathophysiological mechanisms of this disease.
We studied 5 patients (aged 8 to 18) presenting chronic calcipenia with hyperphosphatemia and an increase in C terminal-specific immunoreactive parathyroid hormone. Systematic radiography of the hands revealed 2 cases with signs of subperiosteal resorption characteristic of hyperparathyroidism and 2 cases of cortical fibrillation. These cases were monitored by measuring phosphate levels in the urine after injection of parathyroid hormone (Ellsworth-Howard test), by determining urinary elimination of cyclic AMP after injection of parathyroid hormone (Chase-Aurbach test) and by assaying cyclic AMP levels in the plasma after intravenous administration of parathyroid hormone (Tomlinson-Hendy test). These 3 measurements revealed no changes upon injection of parathyroid hormone, even after administration of vitamin D, thus suggesting renal resistance to parathyroid hormone. The absence of cyclic AMP secretion is suggestive of perturbation of the membrane receptor. Histomorphometric study of the bones showed an increase in resorption and osteoid areas and in the relative osteoid volume without modification of osteoid thickness, which is very suggestive of an effect of parathyroid hormone on the bone. Pseudohypoparathyroidism was, then, characterized by renal resistance to parathyroid hormone and an effect of the latter on bone cells.
The authors report three new cases of erosive disk disease in chronically hemodialysed patients. The cases included one man and two women, who were diagnosed, on the basis of cervical or shoulder pain, as having spinal injuries involving pinched disks, punched-out lesions and incipient condensation. Discal puncture was carried out in only one patient, and proved futile. All three patients were followed-up for periods of at least one year. These three cases raise the problem of erosive disk disease in long-term dialysis patients; several other cases have been reported recently. The etiology remains obscure, but the part played by amylosis, by apatite microcrystals and by the duration of dialysis treatment are all discussed. It would appear that a novel disease entity is indeed involved, one for which, although several etiologic factors can be suggested, it has not been possible to definitely identify any single cause as the exclusive origin of the lesions detected.
Although bone changes in hyperthyroidism are well known, they have been much less studied in hypothyroidism and we set out to establish whether they did occur in the latter condition. We present the results of a prospective study of 20 adult patients, with acquired primary hypothyroidism. No changes in urinary or blood calcium or phosphorus metabolism were observed and serum parathormone, 25-hydro, 125-dihydro and 24-25-dihydroxycholecalciferol concentrations were normal. Histological examination of iliac crest bone biopsy after double labelling with tetracycline showed normal bone trabeculation volume, surface of resorption and rates of calcification. However, the relative osteoid volume was increased and there was a much higher osteoid thickness index. These observations may be explained by a global decrease in the activation and activity of the multicellular units of bone remodeling due to the general slowing of cellular metabolism encountered in that condition.
In a prospective study, 80 patients undergoing long-term hemodialysis, 30% more than ten years, were evaluated for joint function and radiographic abnormalities; 58 patients (72%) had clinical symptoms; non-specific arthralgia was the most frequent; 15 patients had inflammatory pain (shoulders 9, hands 3) and diminution of finger mobility (10). Radiological examinations showed 40 abnormal findings in 23 patients (28%): erosive arthropathies of fingers (4), and multiple geodes of the carpus (8), of the humeral head (7) or of the hip (9). We also detected spondylolisthesis (4), erosive arthropathy of cervical (6) or lumbar (2) spine. Pathological study of synovial biopsies was performed in 16 cases. Amyloidosis was found in 4 samples taken routinely. In 2 other cases the biopsy was done on the basis of lesions seen on x-rays: we found amyloidosis in the synovial membrane in both patients and in the bone in one. In 8 patients we found C3 deposits positive in immunofluorescence. These results underline the role of amyloidosis in the mechanism of some arthropathies in long-term hemodialysis patients.
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