Cheng Chung-Yi, Liao Hung-Wei, Peng Kang-Yung, Chen Tso-Hsiao, Lin Yen-Hung, Chueh Jeff S, Wu Vin-Cent
Division of Nephrology, Department of Internal Medicine, School of Medicine, College of Medicine, Taipei Medical University, No. 250 Wu-Hsing Street, Taipei 110, Taiwan.
Division of Nephrology, Department of Internal Medicine, Wan Fang Hospital, Taipei Medical University, No. 111 Section 3, Xinlong Road, Taipei 116, Taiwan.
Biomedicines. 2021 Dec 2;9(12):1816. doi: 10.3390/biomedicines9121816.
The clinical characteristics and surgical prognosis of glucocorticoid-remediable aldosteronism (GRA, also known as familial hyperaldosteronism type 1, FH-I) have not been widely studied. Using data from the Taiwan Primary Aldosteronism Investigation (TAIPAI) registry retrospectively, we describe the associated clinical factors for GRA and clinical predictors of surgical outcomes among identified GRA patients. We found 79 GRA-positive (51.2 ± 13.8 years; women 39 (49.4%)) and 114 GRA-negative primary aldosteronism (PA) patients matched with age, gender, and body mass index. Lower plasma aldosterone concentrations (PACs) and aldosterone-renin ratios were found among GRA-positive individuals. Multivariable logistic regression demonstrated that a PAC ≤ 40 ng/dL could predict concealed GRA individuals (OR 0.523, = 0.037). Low serum potassium (OR 0.285, = 0.008), but not the presence of GRA, was associated with hypertension-remission. Of note, PRA (OR 11.645, = 0.045) and hypokalemia (OR 0.133, = 0.048) were associated with hypertension-remission in GRA patients. Unilateral primary aldosteronism patients harboring concomitant GRA were not associated with inferior hypertension-remission after an adrenalectomy. Low serum potassium and high PRA were positively associated with hypertension-remission in GRA patients.
糖皮质激素可治性醛固酮增多症（GRA，也称为家族性醛固酮增多症1型，FH-I）的临床特征和手术预后尚未得到广泛研究。我们回顾性地利用台湾原发性醛固酮增多症调查（TAIPAI）登记处的数据，描述了GRA的相关临床因素以及已确诊GRA患者手术结果的临床预测因素。我们发现了79例GRA阳性患者（年龄51.2±13.8岁；女性39例（49.4%））和114例年龄、性别和体重指数相匹配的GRA阴性原发性醛固酮增多症（PA）患者。GRA阳性个体的血浆醛固酮浓度（PACs）和醛固酮-肾素比值较低。多变量逻辑回归显示，PAC≤40 ng/dL可预测隐匿性GRA个体（OR 0.523，P = 0.037）。低血钾（OR 0.285，P = 0.008）而非GRA的存在与高血压缓解相关。值得注意的是，PRA（OR 11.645，P = 0.045）和低钾血症（OR 0.133，P = 0.048）与GRA患者的高血压缓解相关。肾上腺切除术后，合并GRA的单侧原发性醛固酮增多症患者与较差的高血压缓解无关。低血钾和高PRA与GRA患者的高血压缓解呈正相关。
