8661. Rationale for high-dose cyclophosphamide and medium-dose total body irradiation in the conditioning of children with progressive systemic and polyarticular juvenile chronic arthritis before autologous stem cell transplantation.
作者: J M Vossen.;D M Brinkman.;B Bakker.;P M Hoogerbrugge.;R ten Cate.
来源: Rheumatology (Oxford). 1999年38卷8期762-3页 8662. Effectiveness and risks of total body irradiation for conditioning in the treatment of autoimmune disease with autologous bone marrow transplantation.
The results of experiments with the induced autoimmune diseases adjuvant arthritis and allergic encephalomyelitis in rats, which led to the discovery of the curative effect of autologous bone marrow transplantation following high-dose myeloablative treatment, are reviewed. The rationale is eradication of the autoreactive lymphocytes and memory cells, and the prevention of relapse due to transfer of lymphocytes with the autograft. Comparison of various conditioning regimens in the animal models indicates that a combination conditioning with low-dose total body irradiation (TBI) and high-dose cyclophosphamide is optimal. These findings were the basis for the conditioning currently employed in the treatment of polyarticular juvenile chronic arthritis (JCA) by the teams in Utrecht and Leiden, which consists of cyclophosphamide 50 mg/kg for 4 days, 4 Gy TBI and anti-thymocyte globulin (ATG). The use of TBI for the treatment of non-malignant disease is regarded as undesirable by many physicians in view of the risks, in particular, of growth inhibition in children and the induction of tumours. Experimental and clinical data show that a dose of 4 Gy does not cause significant inhibition of skeletal growth in infants. The risk of excess cancer due to TBI has been well established in quantitative terms and is compared with the expected risk of high-dose cyclophosphamide and the risk associated with the highly immunosuppressive regimens currently used for the treatment of JCA.
8664. The depletion of T cells from haematopoietic stem cell transplants.
作者: I C Slaper-Cortenbach.;M J Wijngaarden-du Bois.;A de Vries-van Rossen.;H P Borst.;H van der Lelie.;H G van Heugten.;L F Verdonck.;N M Wulffraat.;P M Hoogerbrugge.
来源: Rheumatology (Oxford). 1999年38卷8期751-4页
In our laboratory, we have developed an immunorosette technique for the depletion of T cells from bone marrow transplants. Tetrameric complexes of monoclonal antibodies are able to form very stable immunorosettes, which are efficiently depleted with the aid of a blood cell separator. Major improvements over the original sheep red blood cell depletion are the use of human (patient or donor derived) erythrocytes instead of sheep-derived cells, and the possibility of using a closed system for separation in a cell separator. In contrast to bone marrow, mobilized haematopoietic stem cell transplants obtained after leucocytapheresis contain higher numbers of T cells. Therefore, a different approach is necessary.
8666. Long-term toxicity of immune suppression in juvenile rheumatic diseases.
Although there are not many studies which have addressed long-term outcomes in children with rheumatic disease treated with immunosuppressive agents, the data that are available, as well as information from adult studies, suggest significant long-term concerns with all the agents that have demonstrable efficacy. This must lead us to investigate new methods of treatment which will not only be more effective, but also less toxic.
8667. Prognosis in children with rheumatic diseases: justification for consideration of new therapies.
It is evident that current approaches to the treatment of childhood arthritis, although much improved in the past decade, are still insufficient to halt the destructive progress of these diseases in many children. Furthermore, the treatment itself is associated with toxicity which may be prohibitive. The need for new and innovative treatments is urgent. ABMT is one possible avenue that offers hope to children with severe unresponsive disease. It will be important to select carefully those children who are likely to benefit from such an approach. An appreciation of the prognosis and possible predictors of disease severity should aid in this task.
8669. Evaluation of the Turkish translation of a disease activity form for Behçet's syndrome.
作者: V Hamuryudan.;I Fresko.;H Direskeneli.;M J Tenant.;S Yurdakul.;T Akoglu.;H Yazici.
来源: Rheumatology (Oxford). 1999年38卷8期734-6页
This study examined the interobserver and intra-observer reliability of the Turkish version of the Behçet's Disease Current Activity Form (BDCAF), which was obtained by a translation and back-translation process.
8670. Behçet's disease: evaluation of a new instrument to measure clinical activity.
作者: B B Bhakta.;P Brennan.;T E James.;M A Chamberlain.;B A Noble.;A J Silman.
来源: Rheumatology (Oxford). 1999年38卷8期728-33页
Behçet's disease (BD) is a rare multisystem disorder characterized by vasculitis. At present, there are no laboratory markers that correlate well with the clinical activity in BD. This has led to the development of an instrument (BD Current Activity Form) to measure activity. Scoring is based on the history of new clinical features present over the preceding 4 weeks prior to assessment. Standardized questions were developed for all parts of the form. The face validity of the proforma was determined following worldwide collaboration with physicians and ophthalmologists managing patients with BD. The aim of this study was to evaluate the interobserver reliability of this form.
8671. High disease activity at baseline does not prevent a remission in patients with systemic lupus erythematosus.
作者: F Formiga.;I Moga.;M Pac.;F Mitjavila.;A Rivera.;R Pujol.
来源: Rheumatology (Oxford). 1999年38卷8期724-7页
To evaluate the utility of systemic lupus erythematosus (SLE) initial clinical manifestations and the SLE Disease Activity Index (SLEDAI) for identifying patients who will have a remission.
8672. Correlation between beta2-glycoprotein I valine/leucine247 polymorphism and anti-beta2-glycoprotein I antibodies in patients with primary antiphospholipid syndrome.
作者: T Atsumi.;A Tsutsumi.;O Amengual.;M A Khamashta.;G R Hughes.;Y Miyoshi.;K Ichikawa.;T Koike.
来源: Rheumatology (Oxford). 1999年38卷8期721-3页
Beta2-Glycoprotein I (beta2GPI) exon 7 polymorphism leads to a valine leucine amino acid exchange at position 247 in domain 5 of beta2GPI, between the phospholipid binding site and the cryptic site of the epitopes for anti-beta2GPI antibodies. Therefore, position 247 polymorphism may affect the conformational change of beta2GPI and the exposure of the epitopes for anticardiolipin antibodies (aCL) (= anti-beta2GPI antibodies). In this study we analysed the genetic polymorphism of beta2GPI in a British cohort of well-defined antiphospholipid syndrome (APS) patients.
8673. Involvement of fibroblast growth factor-2 in joint destruction of rheumatoid arthritis patients.
作者: N Manabe.;H Oda.;K Nakamura.;Y Kuga.;S Uchida.;H Kawaguchi.
来源: Rheumatology (Oxford). 1999年38卷8期714-20页
To investigate the effect of the synovial fluid from knee joints of rheumatoid arthritis (RA) patients with different severities of joint destruction on osteoclastogenesis and bone resorption.
8674. Temporal 67gallium uptake is increased in temporal arteritis.
作者: T Généreau.;O Lortholary.;L Guillevin.;P Cacoub.;N Galezowski.;P Chérin.;P Babinet.;G Herreman.;B Wechsler.;P Cohen.;S Herson.;N Caillat-Vigneron.
来源: Rheumatology (Oxford). 1999年38卷8期709-13页
We evaluated temporal 67gallium (Ga) uptake in temporal arteritis (TA) and the contribution of Ga scans to the diagnosis of TA.
8675. Serum leucine aminopeptidase as an activity indicator in systemic lupus erythematosus: a study of 46 consecutive cases.
作者: S Inokuma.;K Setoguchi.;T Ohta.;Y Matsuzaki.;A Yoshida.
来源: Rheumatology (Oxford). 1999年38卷8期705-8页
To determine whether elevations in serum leucine aminopeptidase (LAP) levels reflected the underlying evolution of active disease in systemic lupus erythematosus (SLE).
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