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ß2-Microglobulin amyloidosis

Michel fadoul, Charles van, Ypersele de Strihou

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Abstract

Abstract ß2-Microglobulin amyloidosis (ß2mA) is a now well-characterized complication of long-term dialysis. Assenat et al. (1980) first reported the presence of amyloid deposits in carpal tunnel biopsies from hemodialysis (HD) patients.This ‘dialysis-related amyloidosis’ (DRA) was renamed ß2-microglobulin amyloidosis after Gejyo eta!. (1985) demonstrated that ß2m is the major component of DRA deposits. It subsequently became apparent that the prevalence of B2mA increased both with age at the onset of dialysis and with the duration of the treatment (van Yperseleetal., 1991)Our understanding of this crippling complication has improved markedly over the last 10 years. In this chapter, we review the clinical characteristics of ß2mA, the available diagnostic tools, the risk factors for the development of the disease, and briefly summarize current pathophysiological concepts.

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Abstract ß2-Microglobulin amyloidosis (ß2mA) is a now well-characterized complication of long-term dialysis. Assenat et al. (1980) first reported the presence of amyloid deposits in carpal tunnel biopsies from hemodialysis (HD) patients.This ‘dialysis-related amyloidosis’ (DRA) was renamed ß2-microglobulin amyloidosis after Gejyo eta!. (1985) demonstrated that ß2m is the major component of DRA deposits. It subsequently became apparent that the prevalence of B2mA increased both with age at the onset of dialysis and with the duration of the treatment (van Yperseleetal., 1991)Our understanding of this crippling complication has improved markedly over the last 10 years. In this chapter, we review the clinical characteristics of ß2mA, the available diagnostic tools, the risk factors for the development of the disease, and briefly summarize current pathophysiological concepts.

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Available abstract

Abstract ß2-Microglobulin amyloidosis (ß2mA) is a now well-characterized complication of long-term dialysis. Assenat et al. (1980) first reported the presence of amyloid deposits in carpal tunnel biopsies from hemodialysis (HD) patients.This ‘dialysis-related amyloidosis’ (DRA) was renamed ß2-microglobulin amyloidosis after Gejyo eta!. (1985) demonstrated that ß2m is the major component of DRA deposits. It subsequently became apparent that the prevalence of B2mA increased both with age at the onset of dialysis and with the duration of the treatment (van Yperseleetal., 1991)Our understanding of this crippling complication has improved markedly over the last 10 years. In this chapter, we review the clinical characteristics of ß2mA, the available diagnostic tools, the risk factors for the development of the disease, and briefly summarize current pathophysiological concepts.

Key concepts: Amyloidosis, Beta-2 microglobulin, Medicine, Carpal tunnel syndrome, Complication, Hemodialysis, Dialysis, Amyloid (mycology)

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