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[伴有淀粉样变性的家族性地中海热。近期发病机制及治疗方面]

[Familial Mediterranean fever with amyloidosis. Recent pathogenetic and therapeutic aspects].

作者信息

Löffler H J, Brass H, Thoenes W, Linke R P

出版信息

Dtsch Med Wochenschr. 1983 Feb 11;108(6):210-5. doi: 10.1055/s-2008-1069529.

Abstract

Familial Mediterranean fever was diagnosed in a 34-year-old Turkish patient with severe nephrotic oedema. Immunohistochemical classification of a biopsy specimen showed amyloidosis of the AA-type. There was a definite increase of serum amyloid-A-protein (SAA). The typical recurrent fever, attacks of abdominal pain with symptoms of subileus and joint swelling could be treated successfully with colchicine, the oedema with diuretics. The progression of renal failure and proteinuria as indicator of the degree of amyloid-induced renal damage remained unaffected by this treatment. With dimethyl-sulfoxide (DMSO) a marked improvement in renal function and a lowering of the SAA level could be achieved. Thus this treatment inhibits the progression of amyloidosis of the AA-type in Mediterranean fever and may be considered for other forms of AA-type amyloidoses. It is possible that the lowering of the SAA-serum concentration and the improvement of renal function is due to an antiphlogistic effect of DMSO, the mechanism of action of which is so far unknown.

摘要

一名34岁患有严重肾病性水肿的土耳其患者被诊断为家族性地中海热。活检标本的免疫组织化学分类显示为AA型淀粉样变性。血清淀粉样蛋白A(SAA)明显升高。典型的反复发热、伴有肠梗阻症状的腹痛发作和关节肿胀可用秋水仙碱成功治疗,水肿用利尿剂治疗。这种治疗对肾衰竭的进展以及作为淀粉样变性所致肾损伤程度指标的蛋白尿没有影响。使用二甲基亚砜(DMSO)可使肾功能显著改善,SAA水平降低。因此,这种治疗可抑制地中海热中AA型淀粉样变性的进展,也可考虑用于其他形式的AA型淀粉样变性。SAA血清浓度的降低和肾功能的改善可能是由于DMSO的抗炎作用,其作用机制目前尚不清楚。

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Renal transplantation in amyloid nephropathy.淀粉样肾病中的肾移植
Int Urol Nephrol. 1989;21(3):339-47. doi: 10.1007/BF02559746.

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