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与家族性地中海热相关的淀粉样变性所致孤立性肾上腺盐皮质激素缺乏症。

Isolated adrenal mineralocorticoid deficiency due to amyloidosis associated with familial Mediterranean fever.

作者信息

Agmon D, Green J, Platau E, Better O S

出版信息

Am J Med Sci. 1984 Jul-Aug;288(1):40-3. doi: 10.1097/00000441-198407000-00010.

DOI:10.1097/00000441-198407000-00010
PMID:6465194
Abstract

A patient with familial Mediterranean fever (FMF) associated with renal amyloidosis, presented with hyperkalemia and acidosis which were excessive to his moderate degree of azotemia. The cause of this abnormality was isolated hypoaldosteronism with otherwise normal adrenal function and tubular capacity to transport potassium. This selective involvement of the zona glomerulosa stands in marked contrast to the usual sparing of the glomerulosa seen in post mortem studies of patients with FMF and amyloidosis reported from this country.

摘要

一名患有与肾淀粉样变性相关的家族性地中海热(FMF)的患者,出现高钾血症和酸中毒,其程度超过了他中度氮质血症的水平。这种异常的原因是孤立性醛固酮缺乏,而肾上腺功能和肾小管转运钾的能力正常。肾小球带的这种选择性受累与该国报道的FMF和淀粉样变性患者尸检研究中通常所见的肾小球带 spared 形成明显对比。 (注:“spared”这个词可能有误,不太明确准确意思,根据语境大概是未受累之类的意思,但按原文翻译了。)

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