患者男,60岁,因情绪激动后出现胸闷、憋气半年余,伴有心慌、头晕、头痛收住我院心血管内科,既往高血压病史13年,规律服用“缬沙坦氨氯地平片”降压,血压控制不佳,最高为200/100 mmHg,“蛛网膜下腔出血颅内动脉瘤栓塞术后”10年,“右眼视网膜脱离”病史半年。否认吸烟饮酒史、心血管疾病及早发性高血压病家族史。入院检查心率65次/分,血压155/99 mmHg,一般情况及常规查体阴性,检验检查结果示血钾1.6 mmol/L,血钠147.8 mmol/L,尿液分析示PH 6.0,血浆醛固酮水平立位511 pg/mL、卧位655 pg/mL均升高,血浆肾素活性立位0 ng/mL/hr、卧位0 ng/mL/hr均降低,醛固酮/肾素活性比值(ARR)立位51.1、卧位65.5均升高,肾上腺CT动态增强扫描示双侧肾上腺见低密度结节影,考虑肾上腺皮质腺瘤所致的原发性醛固酮增多症,然后转泌尿外科行后腹腔镜下左肾上腺切除术,术后病理诊断为肾上腺皮质腺瘤,术后2周随访血浆醛固酮、血钾、血钠等生化指标水平均在正常范围,血压正常。 A 60-year-old male patient was admitted to the Department of Cardiovascular Medicine of our hos-pital because of chest tightness and suffocation for more than half a year after emotional agitation, accompanied by palpitation, dizziness and headache. He has a history of hypertension for 13 years and has been regularly taking “valsartan amlodipine tablets” for hypotensive for many years. His blood pressure has never been well controlled during the medication, with the highest value being 200/100 mmHg, with 10 years after intracranial aneurysm embolization for subarachnoid hemor-rhage, and half a year after retinal detachment in the right eye. The patient denied smoking and drinking history, family history of cardiovascular disease and early onset hypertension. On admis-sion, heart rate was 65 times/min, blood pressure was 155/99 mmHg, general condition and con-ventional physical examination were negative. The results of blood potassium and blood sodium were 1.6 mmol/L and 147.8 mmol/L, respectively. Urine analysis showed PH 6.0, and plasma al-dosterone levels increased in vertical position 511 pg/mL and recubitus position 655 pg/mL re-spectively. The plasma renin activity was decreased by 0 ng/mL/hr in vertical position and 0 ng/mL/hr in recubitus position, and the aldosterone/renin activity ratio (ARR) was increased by 51.1 in vertical position and 65.5 in recubitus position. Dynamic enhanced adrenal CT scan showed low- density nodular opacity in both adrenal glands, which was considered as primary aldosteron-ism caused by adrenal cortical adenoma. Then, the patient was transferred to the urology depart-ment for retroperitoneal laparoscopic left adrenal gland resection, which was pathologically diag-nosed as adrenal cortical adenoma. The plasma aldosterone, blood potassium, blood sodium and other biochemical indexes were all in the normal range and blood pressure was normal 2 weeks af-ter surgery.
患者男,60岁,因情绪激动后出现胸闷、憋气半年余,伴有心慌、头晕、头痛收住我院心血管内科,既往高血压病史13年,规律服用“缬沙坦氨氯地平片”降压,血压控制不佳,最高为200/100 mmHg,“蛛网膜下腔出血颅内动脉瘤栓塞术后”10年,“右眼视网膜脱离”病史半年。否认吸烟饮酒史、心血管疾病及早发性高血压病家族史。入院检查心率65次/分,血压155/99 mmHg,一般情况及常规查体阴性,检验检查结果示血钾1.6 mmol/L,血钠147.8 mmol/L,尿液分析示PH 6.0,血浆醛固酮水平立位511 pg/mL、卧位655 pg/mL均升高,血浆肾素活性立位0 ng/mL/hr、卧位0 ng/mL/hr均降低,醛固酮/肾素活性比值(ARR)立位51.1、卧位65.5均升高,肾上腺CT动态增强扫描示双侧肾上腺见低密度结节影,考虑肾上腺皮质腺瘤所致的原发性醛固酮增多症,然后转泌尿外科行后腹腔镜下左肾上腺切除术,术后病理诊断为肾上腺皮质腺瘤,术后2周随访血浆醛固酮、血钾、血钠等生化指标水平均在正常范围,血压正常。
原发性醛固酮增多症,肾上腺腺瘤,高血压,低血钾
Mengyao Shi, Guirong Sun*
Department of Clinical Laboratory, The Affiliated Hospital of Qingdao University, Qingdao Shandong
Received: Apr. 11th, 2022; accepted: May 6th, 2022; published: May 13th, 2022
A 60-year-old male patient was admitted to the Department of Cardiovascular Medicine of our hospital because of chest tightness and suffocation for more than half a year after emotional agitation, accompanied by palpitation, dizziness and headache. He has a history of hypertension for 13 years and has been regularly taking “valsartan amlodipine tablets” for hypotensive for many years. His blood pressure has never been well controlled during the medication, with the highest value being 200/100 mmHg, with 10 years after intracranial aneurysm embolization for subarachnoid hemorrhage, and half a year after retinal detachment in the right eye. The patient denied smoking and drinking history, family history of cardiovascular disease and early onset hypertension. On admission, heart rate was 65 times/min, blood pressure was 155/99 mmHg, general condition and conventional physical examination were negative. The results of blood potassium and blood sodium were 1.6 mmol/L and 147.8 mmol/L, respectively. Urine analysis showed PH 6.0, and plasma aldosterone levels increased in vertical position 511 pg/mL and recubitus position 655 pg/mL respectively. The plasma renin activity was decreased by 0 ng/mL/hr in vertical position and 0 ng/mL/hr in recubitus position, and the aldosterone/renin activity ratio (ARR) was increased by 51.1 in vertical position and 65.5 in recubitus position. Dynamic enhanced adrenal CT scan showed low-density nodular opacity in both adrenal glands, which was considered as primary aldosteronism caused by adrenal cortical adenoma. Then, the patient was transferred to the urology department for retroperitoneal laparoscopic left adrenal gland resection, which was pathologically diagnosed as adrenal cortical adenoma. The plasma aldosterone, blood potassium, blood sodium and other biochemical indexes were all in the normal range and blood pressure was normal 2 weeks after surgery.
Keywords:Primary Aldosteronism, Adrenal Adenoma, High Blood Pressure, Hypokalemia
Copyright © 2022 by author(s) and Hans Publishers Inc.
This work is licensed under the Creative Commons Attribution International License (CC BY 4.0).
http://creativecommons.org/licenses/by/4.0/
原发性醛固酮增多症(primary aldosteronism, PA)简称原醛症,是指肾上腺皮质发生病变引起的醛固酮分泌增多,导致潴钠排钾、体液容量扩增、肾素–血管紧张素系统受抑制,表现为高血压、低血钾的临床综合征。研究表明,大量醛固酮可导致心脑血管系统、肾功能受损,相对于原发性高血压患者,PA患者心脑血管及肾脏损伤更为严重,过去认为PA患病率较低,仅占高血压病人的0.4%~0.2%,但随着筛查及诊断技术的提升,现有数据表明,我国因高血压就诊的患者中PA检出率高达10.7% [
患者老年男性,因“胸闷、憋气半年”就诊我院心内科门诊,并收住我院心血管内科病房进一步诊断和治疗。患者半年前于情绪激动后反复出现胸闷、憋气,伴心慌、头晕、头痛,情绪平稳及服用“速效救心丸”后约10分钟缓解,期间未就医系统诊治。入院查体:体温36.7℃,脉搏65次/分,呼吸18次/分,血压155/99 mmHg,心肺腹查体阴性。既往“高血压”病史13年,最高为200/100 mmHg,多年来规律服用“缬沙坦氨氯地平片”降压,在服用药物期间血压从未得到很好的控制,“蛛网膜下腔出血颅内动脉瘤栓塞术后”10年,“右眼视网膜脱离”病史半年,否认吸烟饮酒史、心血管疾病及早发性高血压病家族史。
患者入院后完善血、尿、便常规、血糖、血脂、电解质、肝肾功能、甲状腺功能、心衰和心肌损伤指标NT-proBNP、高敏肌钙蛋白等相关检验,结果示血钾1.6 mmol/L,血氯96.7 mmol/L,血钠147.8 mmol/L,高敏肌钙蛋白T 0.061 ug/L,其余指标均未见明显异常。检查结果示心电图一度房室传导阻滞、Q-T延长、多导ST-T改变;心脏超声检查发现:左房扩大、左室心肌肥厚升主动脉扩张(轻度),左室舒张功能减低,EF 60%,考虑高血压性心脏病变,心脏CT冠状动脉血管造影三维成像(CTA)示左右冠状动脉均未见斑块、狭窄及扩张,左冠前降支心肌桥。检验结果示血钾过低,符合低钾血症诊断标准,低钾血症分为缺钾性、转移性及稀释性低钾血症,其中以缺钾性为主要原因,包括摄入不足、排出过多 [
根据2020版原发性醛固酮增多症诊断治疗的专家共识 [
未明确诊断前先给与患者对症药物治疗,静脉、口服补钾,口服枸橼酸钾溶液每日3次,每次20 ml;卡维地洛片每日2次,每次10 mg,患者血钾升高后完善高血压相关动态试验,明确诊断后给与患者口服螺内酯片每隔8小时一次,每次40 mg,并监测生命体征。根据2020最新版指南 [
患者老年男性,根据上述相关检验检查结果,临床诊断为原发性醛固酮增多症,高血压病3级(极高危),电解质紊乱,低钾血症,高血压性心脏病,视网膜脱离(右眼),颅内动脉瘤破裂伴蛛网膜下腔出血栓塞术后,心电图及心脏超声提示心肌肥厚、高血压性心脏病变,冠脉CTA提示心肌桥(左冠前降支),既往视网膜脱离病史,以上考虑为高血压所致心脏、肾脏、眼部等靶器官损害,泌尿系统超声未见异常,可除外肾血管性高血压,患者行手术治疗,术后组织病理结果为肾上腺皮质腺瘤,病理诊断为原发性醛固酮增多症。
内分泌系统是由经典的分泌腺和能产生激素的器官与组织共同组成的,通过激素发挥调节效应,肾上腺是人体重要的内分泌腺,其分为肾上腺皮质和肾上腺髓质,其中盐皮质激素由肾上腺皮质球状带分泌,主要包括醛固酮、11-去氧皮质酮、11-去氧皮质醇,以醛固酮生物活性最强。醛固酮作用的靶器官包括唾液腺、胃肠道外分泌腺、汗腺、肾脏等,以肾脏最重要。肾素–血管紧张素–醛固酮系统(renin-angiotensin-aldosteron system, RAAS)是人体内分布最广的内分泌调节系统,其在血压的调节、心血管功能稳态、电解质以及维持体液的平衡中发挥着至关重要的作用。RAAS系统的主要调控因素是肾素,是由肾脏近球细胞分泌的一种酸性蛋白酶,肝脏或组织中合成和释放的血管紧张素原由肾素水解生产血管紧张素I ( angiotensin-I, Ang-I),血管紧张素转化酶(angiotensin converting enzyme, ACE)水解Ang-I变为血管紧张素II (angiotensin-II, Ang-II),血管紧张素中发挥主要生理作用的是Ang-II,肾上腺皮质球状带经其刺激可合成和分泌醛固酮 [
目前,PA是与心脑血管疾病相关的继发性高血压的常见原因,占所有高血压发病患者的10%,原发性醛固酮增多症的及时诊断和治疗可减轻醛固酮特异性靶器官损害 [
原醛症的发展一般可分为三个阶段:仅有高血压阶段、高血压轻度钾缺乏阶段、高血压严重钾缺乏期。原醛症目前最常用ARR作为筛查指标,已广泛应用于临床,大大提升了原醛症检出率,ARR的测定目前包括醛固酮与血浆肾素浓度比值及其与肾素活性比值,其中,肾素活性检测为通过测定Ang-I的产生速率来间接反映肾素活性水平,Ang-I定量方法主要包括放射免疫法(RIA)和质谱法。目前采用放射免疫法以125I标记Ang-I作为竞争性抗原,通过竞争结合原理定量检测样品中的Ang-I,但此方法受影响因素过多,应用有一定局限性。质谱法主要包括电喷雾质谱(LC/ESI-MS/MS)、免疫基质辅助激光解离质谱(iMALDI-MS)两种,相对于免疫学分析方法,质谱法检测有高特异性、高灵敏度、低检测限、无放射性核苷酸等优势,使其有更广泛的应用,但质谱仪价格昂贵,且在方法标准化方面仍需进一步完善。近年来,全自动化学发光免疫分析法检测肾素浓度逐渐开展,该方法拥有检测速度快、稳定性和重复性好、易于标准化等优点。同时ARR也存在一定的局限性,由于目前诊断流程及检测方法未统一,ARR的切点变化范围过大,影响因素过多,ARR切点需实验室根据相应情况制定 [
通过该病例启示我们对于原醛症患者需明确手术及药物治疗两种方式所适用的疾病分型以及长期药物治疗的副作用、手术治疗的指征等,也使我们对最新版原醛症诊疗专家共识有了全面的了解,并对RAAS系统进行回顾学习,提醒各位医生在临床工作中对早发性和顽固性高血压患者应警惕和注重筛查继发性高血压的可能性和病因,鉴别诊断要更细致和完善,以免将继发性高血压误判为原发性高血压,使病情发展加重甚至出现严重的并发症,延误了有效的治疗。
该病例报道已获得病人知情同意。
石梦瑶,孙桂荣. 误诊多年的原发性醛固酮增多症1例A Case of Primary Aldosteronism Misdiagnosed for Many Years[J]. 临床医学进展, 2022, 12(05): 3855-3860. https://doi.org/10.12677/ACM.2022.125557