Sutherland D J, Ruse J L, Laidlaw J C
Can Med Assoc J. 1966 Nov 26;95(22):1109-19.
A father and son are described with a condition characterized by benign hypertension, potassium deficiency, increased aldosterone secretion rate (ASR), raised plasma volume and suppressed plasma renin activity (PRA). There were intermittent elevations of urine 17-ketosteroids and 17-hydroxycorticoids (17-OHCS) but no increase in urine THS, normal circadian rhythm of plasma 17-OHCS, and normal urine 17-OHCS response to dexamethasone and intravenous ACTH. Plasma ACTH and corticosterone secretion were not elevated. Pregnanetriol excretion was normal but urine pregnanediol was increased. At operation on the father no adrenal tumour was found; the excised left adrenal weighed 7 g. and showed nodular cortical hyperplasia; juxtaglomerular cells showed only occasional granules. Following operation hypertension persisted and ASR was half the preoperative value. All abnormalities in father and son were relieved by dexamethasone (DM) 2 mg. daily. The condition recurred following cessation of DM but was relieved by a second course of treatment. No such response to DM was seen in a normal subject or in a patient with Conn's syndrome. For a number of reasons it is suggested that patients with hypertension, increased ASR and low PRA be given a trial of dexamethasone treatment before undergoing adrenal surgery.
本文描述了一对父子患有某种病症，其特征为良性高血压、低钾血症、醛固酮分泌率（ASR）升高、血浆容量增加以及血浆肾素活性（PRA）受抑制。尿17 - 酮类固醇和17 - 羟皮质类固醇（17 - OHCS）有间歇性升高，但尿促甲状腺激素无增加，血浆17 - OHCS昼夜节律正常，尿17 - OHCS对地塞米松和静脉注射促肾上腺皮质激素（ACTH）的反应正常。血浆促肾上腺皮质激素（ACTH）和皮质酮分泌未升高。孕三醇排泄正常，但尿孕二醇增加。对父亲进行手术时未发现肾上腺肿瘤；切除的左肾上腺重7克，显示结节性皮质增生；球旁细胞仅偶尔可见颗粒。术后高血压持续存在，ASR为术前值的一半。父子俩的所有异常情况均通过每日2毫克地塞米松（DM）得到缓解。停用DM后病情复发，但第二疗程治疗使其缓解。正常受试者或原发性醛固酮增多症患者对DM无此反应。基于多种原因，建议高血压、ASR升高且PRA降低的患者在接受肾上腺手术前先试用糖皮质激素治疗。
