An update on osteonecrosis of the femoral head.
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Biomedical subjects
Publications and source records attributed to B Mazieres.
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OBJECTIVE: To evaluate the performances of 4 sets of criteria proposed to define mixed connective tissue disease (MCTD): the criteria of Sharp, Alarcón-Segovia, Kasukawa, and Kahn. As anti-U1-RNP antibodies appear indispensable to establish the diagnosis of MCTD, we wished to reevaluate these sets of criteria in patients who all had anti-U1-RNP antibodies. METHODS: We analyzed clinical and biological data to find which diagnostic criteria were met by 45 patients with anti-U1-RNP antibodies. We tested criteria for rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, polymyositis, Sjögren's syndrome, and 4 sets of criteria for MCTD. RESULTS: The criteria that best identified patients with MCTD were those proposed by Alarcón-Segovia, with 62.5% sensitivity and 86.2% specificity, comparable to Kahn's criteria. The overlap with other connective tissue diseases was found to be 16%. These results could be improved by using the term "myalgia" instead of "myositis" in the definition. This increased sensitivity to 81.3%, with no decrease in specificity. CONCLUSION: Alarcón-Segovia's and Kahn's criteria are the best classification criteria to define MCTD.
Effective pamidronate dosages in Paget's disease of bone have not yet been determined. We prospectively compared two pamidronate dosages (60 mg and 120 mg) in 26 patients whose baseline alkaline phosphatase level was at least two-fold the upper limit of normal. Pamidronate was given as three-hour infusions of 60 mg each. Follow-up was two years. The two groups were comparable at baseline except for higher alkaline phosphatase and hydroxyproline levels in the group treated with 120 mg pamidronate. Efficacy on laboratory parameters was greater in the higher dosage group. Osteocalcin levels showed little change. In both dosage groups, alkaline phosphatase levels fell significantly versus baseline, and the difference persisted throughout the two-year follow-up. Urinary hydroxyproline excretion remained significantly reduced versus baseline during the first 12 months and rose subsequently. A return to normal of the two study parameters six months after treatment was associated with longer duration of the biologic treatment response. A survival curve analysis confirmed this finding and suggested that a return to normal of laboratory parameters was predictive of clinical remission. The drug was well tolerated. Adverse effects were mild and similar to those recorded during earlier studies.
STUDY DESIGN: This study analyzed computed tomographic scans, magnetic resonance images, and biopsies of the paravertebral muscles of patients with camptocormia and age-matched patients with lumbar interapophyseal osteoarthritis or lumbar vertebral stenosis. OBJECTIVES: To define the muscular lesions and clarify their nature in this particular disorder. SUMMARY OF BACKGROUND DATA: Progressive lumbar kyphosis or camptocormia, a rare disease of the elderly characterized by inability to immobilize the lumbar spine in relation to the pelvis appears to be a result of weakness of the paraspinal muscles. The features presented by these patients do not correspond to any myopathy previously described. METHODS: Twenty-seven patients (5 men and 22 women) mean age 69 years, with camptocormia were compared to fifteen age-matched patients without camptocormia but with posterior interapophyseal osteoarthritis and to nine elderly patients operated for narrowing of lumbar canal. Computed tomographic scans, magnetic resonance images, light microscopy, histochemistry, and electron microscopy of paraspinal muscles were obtained in both groups. RESULTS: In patients with camptocormia, computed tomographic scans and magnetic resonance imaging showed heterogeneous appearance of the spinal muscles with areas of low density. These features were distinct from those of patients with interapophyseal osteoarthritis and were similar to the features described in primary muscular dystrophies. The main microscopic change in camptocormia was the increase of fibrous tissue, frequently with a lobular pattern, not seen in osteoarthritic patients. Familial history of the disorder was frequent (20 out of the 27 patients). CONCLUSION: Camptocormia, disappearing in the recumbent position, is thus very probably linked to muscle involvement. That there is often a family history of such disorder is in favor of a genetically transmitted condition. Magnetic resonance images and computed tomographic scan appearance seems to be in favor of primary muscular disease, restricted to the spinal muscles.
Etiological factors in finger osteoarthritis are both local (single injury or repetitive joint use) and general. Epidemiological studies, twin studies, and studies of the ethnic influence have established that genetic factors play a role. The disease is more common in postmenopausal women, suggesting an influence of hormonal factors. Findings from studies of associations between finger osteoarthritis and osteoarthritis at other sites (spine, hips, knees), hyperostosis, and obesity, as well as the fact that the disease is less common in patients with osteoporosis and absent in hemiplegia, suggest that the concept of "osteoarthritic disease" is relevant.
Three men with osteoporotic fractures were found to have lactase deficiency and low dietary calcium intakes, decreased urinary calcium, and moderately increased serum osteocalcin and parathyroid hormone levels. Histomorphometric studies demonstrated increases in osteoid parameters and resorption surfaces. The few studies of the links between osteoporosis and lactase deficiency have yielded discordant results. A low calcium intake due to aversion to dairy products caused by the lactase deficiency may promote the development of osteoporosis.
In order to clarify the risk-factors for men with vertebral fractures due to osteoporosis, we carried out a study of 51 cases. Twenty-five percent of patients had an endocrine disorder (hyperparathyroidism, hypogonadism, hyperthyroidism) or had received corticosteroids. These patients were compared with 26 age-matched controls. Eleven patients compared with 2 of the 26 control subjects had arteriopathy of the lower limbs; 11 patients had hypercalciuria or hyperphosphaturia compared with 3 of the control subjects. Arteriopathy appears to be associated with osteoporosis in older patients (mean age 71 years), whereas renal tubular disorders were found in younger patients (mean age 45 years).
One case of CRST syndrome with breast granuloma is presented. The presumed diagnosis were infectious or neoplastic diseases. High doses of corticosteroids led to improvement in breast nodules.
In a previous study, the authors demonstrated that in 17 men with ischaemic atherosclerotic disease the bone mineral density (BMD) of the femoral neck was lower than in matched control subjects. The patients with arterial disease were thinner and were heavier smokers than the controls. Osteoporosis and arterial disease of the lower limbs were perhaps due to common risk factors: tobacco consumption and a low body build index. In order to demonstrate the direct effect of atherosclerosis on bone mineral content (BMC), the authors studied by dual-energy X-ray absorptiometry the BMC of both legs in 18 men presenting symptomatic arterial disease of the lower limbs quantified by measurement of distal systolic indexes by doppler ultrasonography. The mean BMC of the leg more severely affected by arterial disease was significantly lower than the mean BMC of the leg less affected by arterial disease (512 +/- 76 g versus 495 +/- 80 g: p = 0.003). In 13 of the 18 patients, the BMC was lower in the leg more severely affected by arterial disease; in 4 of 18 the difference between the BMC of the left and right legs was less than 1%, and in a single patient the BMC was higher in the leg more affected by arterial disease. Arterial disease of the lower limbs could lead to bone mineral loss.
The association of rheumatoid arthritis (RA) with particular MHC class II genes suggests that autoantigen-specific T cell clones present in joints could be central to the pathogenesis of the disease. Previous investigations on the clonal diversity of T cells infiltrating the rheumatoid synovial membrane have yielded conflicting results. With the use of Southern blot analysis, we investigated the clonality of rheumatoid T cell lines expanded from peripheral blood, synovial fluid and synovial tissue. From peripheral blood lymphocyte (PBL) of RA patients and healthy normal controls, we also checked the consequences of two different culture conditions on the clonality of these cell lines. From control PBL, we found that in vitro non-specific expansion of non-clonal T cell populations does not create artefactual clonal selection. However, growing T cells in vitro with IL-2 seems to be able to lead to preferential expansion of cells bearing IL-2 receptor (IL-2R). We identified such in vivo activated IL-2-sensitive T cell clones frequently in RA synovial tissue (8/13) and more rarely in synovial fluid and peripheral blood (3/12). One patient presents the same T cell receptor gene rearrangements in synovial membrane of two affected joints. In RA synovial tissue, the frequency of these IL-2-responsive T cells is most prevalent among actively inflamed membranes removed early in the disease process. The role and the relevance to the disease of these IL-2-responsive T cells remain to be elucidated.
A trial was initiated to determine the feasibility and efficacy of a three-phase treatment including: (1) induction chemotherapy (IC); (2) high-dose melphalan with total body irradiation supported by unpurged autologous bone marrow transplantation (ABMT); and (3) interferon (IFN) alpha maintenance treatment, in previously untreated aggressive myeloma. Thirty-five consecutive patients, ages under 65 years, were enrolled. Initial induction therapy was randomized between the VAD regimen (vincristine, doxorubicin, dexamethasone) or the VMCP regimen (vincristine, melphalan, cyclophosphamide, prednisone) that were found to give similar results as IC. Thirty-one of 35 (89%) patients, with good performance status and normal renal function after IC, received ABMT. IFN alpha was started soon after ABMT and was well tolerated. Fifteen of 35 (43%) patients achieved complete response (CR) and 14 of 35 (40%) achieved partial response (PR). Low pretreatment beta 2 microglobulin was the only predictive factor for accomplishing CR. The duration of response was significantly affected by the magnitude of response. The 33-month, post-ABMT probability of progression-free survival was 85% for patients in CR versus 24% for patients in PR. The 42-month, post-diagnosis probability of survival was 81%. This overall strategy may represent an advance in the management of multiple myeloma. Furthermore, the high rate and long duration of CR that we observed in patients with low beta 2 microglobulin suggest that such patients may preferentially benefit from this strategy.
The authors report a study of 47 patients admitted for cervical myelopathy (N = 17) or symptomatic lumbar spinal stenosis (N = 30). Nine patients had clinical evidence of coexisting cervical myelopathy and lumbar spinal stenosis. Ten out of the 17 patients having cervical myelopathy had lumbar spinal stenosis as evidenced by sagittal tomography and/or computerized tomography. Nine out of the 30 patients admitted for symptomatic lumbar spinal stenosis had coexisting cervical canal stenosis as evidenced by sagittal tomography. Thirteen out of these 19 patients with both cervical and lumbar canal stenosis had also ankylosing spinal hyperostosis.
Certain morphologic features frequently observed in radiography or computed tomography (CT) scan in patients with hyperostosis led us to study the association between a narrowed spinal canal and vertebral hyperostosis. Twenty-eight items were selected and studied by three different investigators (two rheumatologists and one radiologist) in radiographs and CT scans of 100 patients with acquired stenosis of the lumbar canal, with or without hyperostosis (46 and 54 cases, respectively). The most distinctive points that we suggest can be used as diagnostic criteria of the hyperostotic narrowed lumbar canal are anterior or posterior lateral marginal somatic osseous proliferations, proliferations of the nonarticular aspects of the posterior apophyses, and ossifications of the posterior articular capsule and of the ligaments (yellow ligament, posterior longitudinal ligament, and the supraspinal ligament). Four of these six criteria should be present to establish the diagnosis of hyperostotic lumbar stenosis. The appearance of lumbar hyperostosis on X-ray or CT scans differs from that of simple degenerative changes due to arthrosis, and the hyperostosis can be held responsible for dural compression.
Endogenous 13-cis- and all-trans-retinoic acids have been quantitated in human serum using a solvent extraction procedure followed by isocratic reversed phase high performance liquid chromatography and UV detection. In healthy adults, after an overnight fasting period, the concentrations of 13-cis- and all-trans-retinoic acids yielded 5.3 +/- 2.43 nmol/l and 11.8 +/- 3.3 nmol/l, respectively (mean +/- SD). The method has been successfully applied to the analysis of both isomers in serum from patients with idiopathic skeletal hyperostosis in whom, the 13-cis- as well as all-trans-retinoic acid levels were raised as compared to the control group.
Although the link between phosphate diabetes and neoplasm or benign mesenchymal tumors has been well documented, the nature of the phosphaturia factor remains unknown. We describe two cases of phosphate diabetes associated with bronchogenic cancer. In these case reports, we suggest that fibroblasts or osteoblasts synthesize a phosphate eliminating substance.
Twenty patients (median age 57 years) with high tumor mass myeloma in first remission after conventional chemotherapy received a two-phase treatment: autologous bone marrow transplantation (ABMT) using a preparative regimen of high dose melphalan plus total body irradiation followed by maintenance treatment with recombinant alpha interferon. Before ABMT all patients were in partial remission, while after ABMT 10 (50%) achieved complete remission, and 10 remained in partial remission (with a 90% decrease in measurable paraprotein in 7/10). With a median follow-up of 19.8 months (12.2-33.5) after diagnosis and 13 months (4-25) after ABMT, the Kaplan-Meier 2-year post-ABMT probability of progression-free survival was 85% (95% CI = 58.7-95.8%). None of the 10 patients in complete remission has relapsed. No toxic death occurred. Alpha interferon was introduced early after ABMT (2.7 months) and was well tolerated. This strategy may represent an advance in the management of aggressive myeloma.
We describe a case of stress fracture of the roof of the acetabulum, in a woman with osteoporosis. Magnetic resonance imaging gave abnormally low signals in T1 weighted images and high signals in T2 weighted images.
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