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正常受试者和系统性淀粉样变性患者中放射性碘化血清淀粉样蛋白P成分的代谢研究。

Metabolic studies of radioiodinated serum amyloid P component in normal subjects and patients with systemic amyloidosis.

作者信息

Hawkins P N, Wootton R, Pepys M B

机构信息

Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London, United Kingdom.

出版信息

J Clin Invest. 1990 Dec;86(6):1862-9. doi: 10.1172/JCI114917.

Abstract

125I-Serum amyloid P component (SAP), injected intravenously into 10 normal subjects, remained predominantly intravascular with mean (SD) T1/2 (half time) in plasma of 24.5 (5.9) h. The fractional catabolic rate of 68 (19)% of the plasma pool per day was more rapid than other reported human plasma proteins. All radioactivity was excreted in the urine by 14 d. In 16 patients with monoclonal gammopathy or chronic inflammatory diseases, but without amyloidosis, 125I-SAP metabolism was normal. However, among 45 patients with biopsy-proven systemic amyloidosis (25, amyloid A type; 20, amyloid L type), 125I-SAP was cleared from the plasma more rapidly, accumulated in the amyloid deposits, and persisted there. The T1/2 in amyloid, measured directly with 131I-SAP, was 24 d. Repeat studies after 6-18 mo were notably consistent in normals but changed significantly in amyloid patients, generally correlating with clinical signs of disease progression. Measurements of 125I-SAP turnover may thus be of value for diagnosis and monitoring of amyloidosis. Analysis of SAP metabolism in amyloidosis suggests that plasma SAP is in dynamic equilibrium with a very large amyloid pool, and in two autopsies the total mass of SAP in the amyloid deposits was 2,100 and 21,000 mg, respectively.

摘要

将125I-血清淀粉样蛋白P成分(SAP)静脉注射到10名正常受试者体内后,其主要留存于血管内,血浆中的平均(标准差)半衰期(T1/2)为24.5(5.9)小时。每天血浆池68(19)%的分数分解代谢率比其他已报道的人体血浆蛋白更快。到第14天时,所有放射性均经尿液排出。在16例患有单克隆丙种球蛋白病或慢性炎症性疾病但无淀粉样变性的患者中,125I-SAP的代谢正常。然而,在45例经活检证实为系统性淀粉样变性的患者中(25例为淀粉样蛋白A类型;20例为淀粉样蛋白L类型),125I-SAP从血浆中清除得更快,在淀粉样沉积物中蓄积并持续存在。用131I-SAP直接测得的淀粉样沉积物中的半衰期为24天。6 - 18个月后进行的重复研究在正常受试者中显著一致,但在淀粉样变性患者中变化显著,总体上与疾病进展的临床体征相关。因此,测定125I-SAP的周转率可能对淀粉样变性的诊断和监测有价值。对淀粉样变性中SAP代谢的分析表明,血浆SAP与非常大的淀粉样蛋白池处于动态平衡,在两次尸检中,淀粉样沉积物中SAP的总质量分别为2100毫克和21000毫克。

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