Danby P, Harris K P, Williams B, Feehally J, Walls J
Department of Nephrology, Leicester General Hospital, U.K.
Q J Med. 1990 Sep;76(281):915-22.
Amyloidosis is a multi-system disease. Renal involvement often leads to end-stage renal failure, which carries a poor prognosis. This paper reports the adrenal status of 22 patients with renal amyloid who were considered for or who had been commenced on renal replacement therapy. Twelve patients were considered or found to have AA amyloid and the remaining 10 had AL amyloid. Of 16 patients tested, seven demonstrated an abnormal response to a synacthen test. Four patients died at Addisonian crisis and hypo-adrenalism probably contributed to the deaths of a further two patients. Amyloid deposition was found in the adrenal glands in seven patients who died of systemic amyloidosis and renal failure. It is recommended that all patients with renal amyloid should have an assessment of adrenal function performed and if abnormal replacement steroid therapy should be commenced.
淀粉样变性是一种多系统疾病。肾脏受累常导致终末期肾衰竭，预后较差。本文报告了22例考虑进行或已开始接受肾脏替代治疗的肾淀粉样变性患者的肾上腺状况。12例患者被认为或被发现患有AA型淀粉样变性，其余10例为AL型淀粉样变性。在16例接受检测的患者中，7例对促肾上腺皮质激素试验反应异常。4例患者死于艾迪生病危象，肾上腺功能减退可能是另外2例患者死亡的原因。在7例死于系统性淀粉样变性和肾衰竭的患者肾上腺中发现了淀粉样沉积。建议所有肾淀粉样变性患者都应进行肾上腺功能评估，若结果异常应开始进行类固醇替代治疗。
