Potthoff S A, Beuschlein F, Vonend O
Universität Düsseldorf, Medizinische Fakultät, Universitätsklinikum Düsseldorf, Klinik für Nephrologie, Düsseldorf, Germany.
Dtsch Med Wochenschr. 2012 Nov;137(48):2480-4. doi: 10.1055/s-0032-1327318. Epub 2012 Nov 20.
Primary hyperaldosteronism (PHA) is characterized by an increased Aldosterone synthesis which is independent of the Renin-Angiotensin-Aldosterone-System (RAAS). The prevalence of PHA in patients who present in specialized hypertension centers is approx. 10 %. Besides patients with the classical symptoms known as "Conn-Trias" (hypertension, hypokalemia, metabolic alkalosis), the more frequent normokalemic patients with PHA also show a worse outcome compared to patients with essential hypertension. Identifying these patients is an important task in the evaluation of hypertension since targeted treatment options are available. Screening for PHA using the Aldosterone-Renin-Ratio (ARR) should be performed in patients with hypokalemic, severe or resistant hypertension. In addition, young patients with early onset of severe hypertension and/or positive family history should be screened. A positive screening result should be followed by a confirmatory test. The saline infusion test is the preferred clinical test for confirming a suspected PHA since it is accessible and time efficient. Other confirmatory tests are not used on a regular basis. After any confirmatory test, CT- or MRI-imaging and adrenal vein sampling (AVS) is used in order to differentiate between a unilateral adenoma, a bilateral hyperplasia or another cause of PHA. CT or MRI usually cannot discriminate smaller tumors form hyperplasia. Therefore AVS is used to detect lateralization of autonomous aldosterone production. Lateralization of aldosterone production indicates a unilateral adenoma. In these cases, laparoscopic adrenalectomy is the therapeutic option of choice with a hypertension cure rate of up to 60 %. If no lateralization is detectable, bilateral hyperplasia as the underlying cause of PHA is likely. Pharmacological inhibition of the mineralocorticoid receptor is the preferred treatment option in these cases. If Spironolactone is not well tolerated, Eplerenone and potassium-sparing diuretics should be prescribed. Often, however, in order to fully control hypertension, additional antihypertensive therapy is necessary.
原发性醛固酮增多症(PHA)的特征是醛固酮合成增加,且独立于肾素-血管紧张素-醛固酮系统(RAAS)。在专科高血压中心就诊的患者中,PHA的患病率约为10%。除了具有“康恩三联征”(高血压、低钾血症、代谢性碱中毒)这一经典症状的患者外,与原发性高血压患者相比,PHA中更常见的血钾正常患者的预后也更差。识别这些患者是高血压评估中的一项重要任务,因为有针对性的治疗方案可供选择。对于低钾血症、重度或难治性高血压患者,应使用醛固酮-肾素比值(ARR)筛查PHA。此外,对于重度高血压发病早和/或有阳性家族史的年轻患者也应进行筛查。筛查结果为阳性后应进行确诊试验。生理盐水输注试验是确诊疑似PHA的首选临床检查,因为它容易实施且省时。其他确诊试验不常使用。在任何确诊试验后,使用CT或MRI成像以及肾上腺静脉采样(AVS)来区分单侧腺瘤、双侧增生或PHA的其他病因。CT或MRI通常无法区分较小的肿瘤与增生。因此,AVS用于检测自主性醛固酮分泌的侧别。醛固酮分泌的侧别表明为单侧腺瘤。在这些情况下,腹腔镜肾上腺切除术是首选的治疗选择,高血压治愈率高达60%。如果未检测到侧别,PHA的潜在病因可能是双侧增生。在这些情况下,盐皮质激素受体的药理抑制是首选的治疗选择。如果螺内酯耐受性不佳,应开具依普利酮和保钾利尿剂。然而,通常为了完全控制高血压,还需要额外的抗高血压治疗。