摘要:

The molecular mechanisms of the primary immunodeficiencies have been further explored and provide insights into the regulation of the immune response and its role in autoimmune disease, infection, and malignancy. The critical role of CD40-CD40 ligand-mediated effects in T cell-dependent B cell activation, shown through the study of patients with common variable immunodeficiency and hyper IgM syndrome, suggests a potential narrow target for immunomodulatory therapy. A mouse model for chronic granulomatous disease has been developed and promises to be a source of future information. Optimal cost-effective therapy for primary immunodeficiencies continues to be defined, with the results of a large series of patients treated with subcutaneous immunoglobulin infusions reported. Further experience with polyethylene glycol adenosine deaminase and interleukin-2 conjugates is discussed.

ISSN:1040-8711    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

The rheumatologic manifestations of hemophilia and the hemoglobinopathies have been previously reviewed. Recent observations on the management and complications of these disorders are presented. Although rheumatologic manifestations are well recognized in leukemias and lymphomas, most of the observations have been based on small series of patients and case reports. This review focuses on leukemias and lymphomas, and brief reference is made to the myelodysplastic syndromes.

ISSN:1040-8711    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

Developments concerning amyloidosis associated with rheumatic diseases or often causing musculoskeletal symptoms are reviewed. The pathogenesis, clinical manifestations, diagnosis, and therapy of amyloid A, amyloid light-chain, and amyloid β2-microglobulin amyloidosis are discussed from the standpoint of a clinical rheumatologist. The biology of the precursor protein serum amyloid A (SAA) has been extensively studied, and a new assay for SAA has been developed. In amyloid β2-microglobulin amyloidosis, modified β2-microglobulin may function as a pathogenic factor that initiates an inflammatory reaction in which macrophages produce cytokines that induce osteoclastogenesis and bone resorption. Further experience with serum amyloid P component scintigraphy in the diagnosis and monitoring of amyloidosis has accumulated. Guidelines for the dosage of colchicine in the treatment of amyloidosis associated with familial Mediterranean fever have been published. In amyloid light-chain amyloidosis, intensive chemotherapy in combination with bone marrow transplantation or autologous stem-cell infusion has potential therapeutic significance.

ISSN:1040-8711    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

The rheumatologic manifestations of renal disease are commonly encountered and include crystal deposition disease and renal osteodystrophy. In addition, renal transplantation carries important rheumatologic manifestations. Basic calcium phosphate crystal deposition remains an important source of morbidity in renal disease, but it can be largely prevented. Early preventive measures and use of biochemical markers for assessment allow effective management of renal osteodystrophy. Effective treatment is available for the acute bone pain syndrome seen after transplantation, and the natural history of osteonecrosis in such patients may be more benign than previously considered, thus affecting current management schemes for this complication.

ISSN:1040-8711    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

Some clinical features observed in diabetes mellitus suggest secondary Sjögren's syndrome, and the effect of autonomic neuropathy on tear production is analyzed. Limited joint mobility in diabetes mellitus continues to attract attention, and a number of suggestions for further studies are discussed. Neurophysiologic assessment of the median nerve distinguishes diabetic polyneuropathy from carpal tunnel syndrome; however, Martin-Gruber anastomosis may modify nerve conduction measurements. Fracture rate does not appear to be increased in diabetes mellitus. The role of confounding variables and a dose-dependent effect of thyroxine therapy on bone mass is discussed. The possible association of hypothyroidism with gout is debated. Autoimmune thyroid diseases may be added to the list of conditions that produce anticardiolipin antibodies without the clinical manifestations of antiphospholipid syndrome.

ISSN:1040-8711    

出版商:OVID    

年代:1996

数据来源: OVID

摘要:

It is becoming increasingly recognized that a variety of tropical infectious diseases may be associated with a wide range of rheumatic infections. These include viral arthropathies such as O'nyong-nyong, chikungunya, and dengue. Parasitic infections such as filariasis, schistosomiasis, and amoebiasis can be associated with various joint problems. Diseases such as tuberculosis, rheumatic fever, leprosy, and brucellosis, although more common in the tropics, continue to present with rheumatic features in Western countries. Thus, with increasing world travel and migration, there is a need for increasing awareness and further study of tropical diseases and their rheumatic manifestations.

ISSN:1040-8711    

出版商:OVID    

年代:1996

数据来源: OVID