以急性左心衰竭为首发表现的巨大嗜铬细胞瘤一例

嗜铬细胞瘤是起源于肾上腺髓质、交感神经节、旁交感神经节或其他部位嗜铬组织的肿瘤。由于瘤组织可阵发或持续性分泌去甲肾上腺素、肾上腺素及微量的多巴胺,临床上常呈阵发性或持续性高血压、头痛、多汗、心悸以及代谢紊乱症候群表现。该文报道了1例以急性左心衰竭为首发症状的嗜铬细胞瘤患者,并通过影像学技术动态观察到嗜铬细胞瘤诱发急性高血压、急性左心衰竭和心肌水肿/顿抑的完整的病理生理过程。     

肝嗜铬细胞瘤1例报告

正嗜铬细胞瘤是一种起源于肾上腺嗜铬细胞的神经内分泌肿瘤,它可以持续或间断分泌儿茶酚胺,包括去甲肾上腺素、多巴胺及肾上腺素。肾上腺外来源的嗜铬细胞瘤称为转移性嗜铬细胞瘤。嗜铬细胞瘤典型的临床表现有血压异常波动、心悸、头疼等,严重者可导致高血压危象,危及生命。本文报道了1例位于肝右后叶的嗜铬细胞瘤的青年患者,并讨论了临床表现、影像学表现、组织学病理和治疗方法。1病例资料     

嗜铬细胞瘤危象

嗜铬细胞瘤起源于交感神经系统的嗜铬细胞，约85％～90％发生在肾上腺髓质，其余在肾上腺外的交感神经组织，其基本病理生理是肿瘤细胞分泌大量的儿茶酚胺（肾上腺素和／或去甲肾上腺素），作用于肾上腺素能受体，引起以高血压及代谢紊乱为主的症候群。严重时可出现嗜铬细胞瘤危象（pheochromocytomacrisis），包括高血压危象、儿茶酚胺心肌病的危重症状，如心律失常、心力衰竭、心源性休克、心肌梗塞等，以及颅内出血、胃肠道出血、摔死等。现将其主要表现及处理分述如下：病血压危象嗜铬细胞瘤患者的高血压可呈阵发性和持续性，约各占半数…     

生化试验诊断嗜铬细胞瘤:尿与血浆对比

血压持续升高的嗜铬细胞瘤通过测定24小时尿VMA(Vanillylmandelate)、甲氧肾上腺素(Metanephrine)及儿茶酚胺即足以肯定或否定诊断。当血压呈间歇性时,诊断较困难,因为在血压正常期间,肿瘤不一定分泌儿茶酚胺。为了判定这些检查的诊断价值,作者测定了15例嗜铬细胞瘤患者(其中4例为间歇型高血压)尿儿茶酚胺代谢产物浓度和安静状态血浆儿茶酚胺浓度。     

嗜铬细胞瘤性儿茶酚胺心肌病

嗜铬细胞瘤性儿茶酚胺心肌病在临床很难诊断,偶于尸检发现。作者用非损伤性方法,即临床表现,心电图和超声心动图对儿茶酚胺心肌病进行诊断。19位嗜铬细胞瘤患者(男性6人,女性13人)。年龄:15～60岁。患者均接受外科治疗。外科治疗前、后分别测定尿中肾上腺素、去甲肾上腺素及多巴胺的含量。急性期和外科治疗后3～6天内检查心电图。     

异位嗜铬细胞瘤

目的了解异位嗜铬细胞瘤患者的临床特点,探讨诊断和治疗方法。方法回顾性分析12例异位嗜铬细胞瘤患者的临床表现、定位和定性检查以及治疗效果的资料。结果11例患者表现为持续性高血压,伴阵发性加重。11例测定了血儿茶酚胺,2例同时测定了24h尿儿茶酚胺。6例进行了腔静脉分段取血。8例患者手术治愈,4例患者药物控制良好。结论异位嗜铬细胞瘤以持续性高血压伴阵发性加重表现为主。血儿茶酚胺检查有助于病例诊断。无禁忌症患者应手术治疗。     

异位嗜铬细胞瘤

目的了解异位嗜铬细胞瘤患者的临床特点,探讨诊断和治疗方法.方法回顾性分析12例异位嗜铬细胞瘤患者的临床表现、定位和定性检查以及治疗效果的资料.结果 11例患者表现为持续性高血压,伴阵发性加重.11例测定了血儿茶酚胺,2例同时测定了24 h尿儿茶酚胺.6例进行了腔静脉分段取血.8例患者手术治愈,4例患者药物控制良好.结论异位嗜铬细胞瘤以持续性高血压伴阵发性加重表现为主.血儿茶酚胺检查有助于病例诊断.无禁忌症患者应手术治疗.     

嗜铬细胞瘤并急性非心源性肺水肿、休克1例报告并文献复习

嗜铬细胞瘤是起源于嗜铬细胞的肿瘤,通过分泌和释放大量的儿茶酚胺引起阵发性或持续性血压升高,其中约25％ ～ 50％合并儿茶酚胺心肌病[1].嗜铬细胞瘤合并急性心力衰竭、肺水肿时预后很差[2],同时合并休克时病情更危重.我院成功抢救嗜铬细胞瘤并急性非心源性肺水肿及休克1例,报道如下.     

嗜铬细胞瘤的心血管表现(待续)

嗜铬细胞瘤心血管的临床表现多样,但通常表现为持续性或阵发性高血压,同时还伴有儿茶酚胺分泌过量的其他相关症状和体征。其中最危及生命的心血管表现,如高血压急症,是由于肿瘤组织突然分泌大量儿茶酚胺所致。嗜铬细胞瘤患者还可能表现为低血压甚至休克,这些表现可能出现在多系统危象之前。嗜铬细胞瘤中最常见的心律失常是窦性心动过速和心悸,也可表现为更严重的室性心律失常或传导障碍。随着对应激性心肌病关注的日益增多,人们发现嗜铬细胞瘤还可表现为可逆性的扩张型或肥厚型心肌病。该文对嗜铬细胞瘤心血管表现的病因、临床表现和治疗进行综述。由于嗜铬细胞瘤的心血管并发症可危及生命,对有临床表现的患者,即使儿茶酚胺分泌正常,也应进行嗜铬细胞瘤的筛查。     

尿液儿茶酚胺水平与高血压控制水平的相关性

目的评价尿儿茶酚胺水平与血压的关系,以及服用不同药物的高血压患者尿儿茶酚胺水平的差异,以明确交感神经系统对血压的影响。方法连续入选本院心血管内科诊治的高血压患者共120例。根据血压情况分为三组:A组:40例(1级高血压组:收缩压140~159mmHg或舒张压90~99mmHg);B组:40例(2级高血压组:收缩压160~179mmHg或舒张压100~109mmHg);C组:40例(3级高血压组:收缩压≥180mmHg或舒张压≥110mmHg),根据服用药物种类不同分为ACEI/ARB组和CCB组两个亚组。选择同期诊治的40例血压正常者为对照组。检测观察组和对照组所有患者的24小时尿液儿茶酚胺水平。结果各组24小时尿液儿茶酚胺水平中,去甲肾上腺素、多巴胺水平比较的差异有统计学意义(P0.01);C组(3级高血压组)患者中服用ACEI/ARB和服用CCB的患者24h尿液儿茶酚胺水平比较的差异无统计学意义(P0.05)。结论高血压患者尿液中去甲肾上腺素水平随着高血压严重程度而升高;服用不同药物种类并不影响尿液儿茶酚胺水平;交感神经过度激活对高血压的发生起着重要作用。     

Increased plasma atrial natriuretic factor in catecholamine-producing tumor patients.

The aim of this study was to evaluate plasma levels of ANF in patients with catecholamine-secreting tumors with and without hypertension and to relate ANF secretion to levels of plasma and urinary catecholamines and blood pressure. Twenty-one pheochromocytoma (15 with sustained, 6 with paroxysmal hypertension), 6 neuroblastoma (1 hypertensive) patients and 28 aged-matched controls were studied in basal conditions. Plasma and urinary norepinephrine (NE),epinephrine (E), dopamine (DA) and DOPA were determined by HPLC-ED and plasma ANF by RIA. Both neuroblastoma and pheochromocytoma patients had significantly higher plasma ANF levels than controls. Neuroblastomas showed higher ANF concentration than pheochromocytomas. No differences were found in plasma ANF between hypertensive and normotensive patients. Pheochromocytomas with ANF levels within the normal range had plasma and urinary NE and urinary DA and DOPA levels significantly higher than patients with high ANF. Plasma ANF levels were unrelated to systolic or diastolic blood pressure or heart rate. A negative correlation between plasma ANF and urinary DA was found only in the patients groups. In conclusion, plasma ANF was increased in pheochromocytoma and neuroblastoma patients. Our data suggest that the excessive catecholamine secretion is not responsible for the increased ANF secretion in these patients. The significance of the relationships among plasma ANF and urinary and plasma catecholamines requires further investigation.     

Increased Plasma Atrial Natriuretic Factor in Catecholamine-Producing Tumor Patients

The aim of this study was to evaluate plasma levels of ANF in patients with catecholamine-secreting tumors with and without hypertension and to relate ANF secretion to levels of plasma and urinary catecholamines and blood pressure. Twenty-one pheochromocytoma (15 with sustained, 6 with paroxysmal hypertension), 6 neuroblastoma (1 hypertensive) patients and 28 aged-matched controls were studied in basal conditions. Plasma and urinary norepinephrine (NE),epinephrine (E), dopamine (DA) and DOPA were determined by HPLC-ED and plasma ANF by RIA. Both neuroblastoma and pheochromocytoma patients had significantly higher plasma ANF levels than controls. Neuroblastomas showed higher ANF concentration than pheochromocytomas. No differences were found in plasma ANF between hypertensive and normotensive patients. Pheochromocytomas with ANF levels within the normal range had plasma and urinary NE and urinary DA and DOPA levels significantly higher than patients with     

坎地沙坦时间治疗学对非杓型高血压的影响

目的观察坎地沙坦不同给药时间对非杓型高血压患者血压水平及血压昼夜节律的影响。方法选择经24h动态血压监测属于非杓型高血压的需要药物治疗的1级高血压患者106例,随机（电脑随机数字表法）分为A组（早晨7：00服药）和B组（晚上7：00服药）。两组均选用坎地沙坦4mg,用药8周后观察用药前后24h动态血压参数,比较两组血压昼夜节律的变化。结果两组治疗前后血压比较,24h收缩压、24h舒张压、白昼收缩压、白昼舒张压、夜问收缩压、夜间舒张压均有明显下降,差异有统计学意义（P〈0．05）。两组治疗后24h舒张压、白昼收缩压、白昼舒张压、夜间舒张压比较,差异无统计学意义（P〉0．05）。B组治疗后24h收缩压、夜间收缩压比A组改善明显,差异有统计学意义（P〈0．05）。结论早晨或夜间服用坎地沙坦均能有效降低非杓型高血压的血压水平及改善血压昼夜节律,但夜间服药组在控制夜间收缩压和纠正血压昼夜节律方面更有优势。     

Effect of removal of phaeochromocytoma on diurnal variability of blood pressure, heart rhythm and excretion of catecholamines

Ambulatory 24 hour blood pressure and ECG monitoring with simultaneous estimation of the urinary excretion of noradrenaline and adrenaline were performed in seven patients with phaeochromocytoma, before and after removal of the tumour. Mean blood pressure during eight 3-hour periods, mean heart rate, and noradrenaline and adrenaline excretion during four 6-hour periods were estimated. Mean blood pressure before surgery did not show any significant circadian changes. After surgery both systolic and diastolic blood pressures were significantly lower at night than in the daytime periods. In contrast, mean heart rate before surgery was significantly lower at night than in the morning, and this relationship was greater after surgery. Ventricular arrhythmias during 24 hour monitoring were noted in three patients before and in six after surgery. These findings suggest that profound disturbances of autonomic blood pressure regulation improve after removal of phaeochromocytoma, and that ventricular arrhythmias, except for paroxysmal symptoms, tend to be less frequent before than after surgery.     

Catecholamines in the cardiovascular expression of pheochromocytoma. II--Study of free urinary catecholamines in 14 pheochromocytomas. Classification of pheochromocytomas according to type of secretion

Studies were conducted in 14 patients with pheochromocytoma over a 3-year period. Circumstances of detection of these tumors varied greatly and were sometimes misleading, hypertension being an inconstant finding in the clinical history and was not always the predominant feature. Biologic exploration involved assay of excretion of free urinary noradrenaline (NA), adrenaline (AD) and dopamine (DA) using a HPLC technique as well as assay of total methoxy derivatives and urinary vanilmandelic acid. Validity of each assay in the diagnosis of pheochromocytoma could be evaluated and only the total free methoxy derivatives gave false negative results. Hormonal secretion of pheochromocytoma is often mixed, but sometimes predominant or exclusive for a single catecholamine. Relative increases of the different catecholamines, evaluated from the ratios DA/NA and DA/NA + AD, are an important factor since a relation exists between blood pressure induced symptomatology and equilibrium between hypotensive hormone (DA) and pressor amines (NA + AD); 3 types of pheochromocytoma can be described: NA-induced with paroxysmal or permanent hypertension but without typical metabolic and cardiac disorders, and with a very reduced DA/NA + AD ratio during hypertensive crises; AD-induced without permanent hypertension but with a mainly orthostatic hypotension and episodes of cardiovascular collapse following hypertensive attacks and with an AD/NA ratio greater than 1; finally the DA-induced lesion in which hypertension is never associated and manifestations are misleading and atypical with an elevated DA/NA + AD ratio.     

轻度妊高征患者动态血压变化与尿微量白蛋白排泄率的关系探讨

目的探讨轻度妊高征患者动态血压变化与尿微量白蛋白排泄率(UMAE)的关系。方法选择轻度妊高征患者(轻度妊高征组)68例和正常晚期妊娠的孕妇(正常妊娠组)76例,动态监测两组孕妇的24 h血压变化,同步监测UMAE变化情况,分析两者之间的相关性。结果轻度妊高征组孕妇24 h收缩压或舒张压平均血压、血压负荷均显著高于正常妊娠组孕妇(P均0.05);昼夜平均血压差低于正常妊娠组(P0.05);轻度妊高征组孕妇UMAE(50,6±32,4)mg/d,显著高于正常妊娠组(15.1±9.5)mg/d(P0.05);轻度妊高征患者24 h动态血压变化与UMAE呈正相关。结论轻度妊高征患者血压昼夜规律性变化消失,与其早期肾脏损害存在密切相关性;妊娠期间24 h监测动态血压与UMAE,有重要的临床指导意义。     

糖耐量低减患者进行动态血压及尿白蛋白检测的临床意义

应用动态血压监测（ＡＢＰＭ）技术进行了５４例血压正常的糖耐量低减（ＩＧＴ）患者动态血压与尿白蛋白检测，并与３０例正常人进行对照，结果显示：ＩＧＴ患者在未出现高血压之前，已有动态血压改变，部分ＩＧＴ存在阵发性高血压，尤其是收缩压（ＳＢＰ）升高显著，舒张压有增高趋势。ＩＧＴ患者尿白蛋白排出率（ＵＡＥＲ）较正常人升高（Ｐ〈０．０１），血压升高者尿白蛋白（ＵＡＥ）增高多见。因此，预防糖尿病并发症，干预应从     

Circadian blood pressure changes and left ventricular hypertrophy in essential hypertension

The effects of circadian blood pressure (BP) changes on the echocardiographic parameters of left ventricular (LV) hypertrophy were investigated in 235 consecutive subjects (137 unselected untreated patients with essential hypertension and 98 healthy normotensive subjects) who underwent 24-hour noninvasive ambulatory blood pressure monitoring (ABPM) and cross-sectional and M-mode echocardiography. In the hypertensive group, LV mass index correlated with nighttime (8:00 PM to 6:00 AM) systolic (r = 0.51) and diastolic (r = 0.35) blood pressure more closely than with daytime (6:00 AM to 8:00 PM) systolic (r = 0.38) and diastolic (r = 0.20) BP, or with casual systolic (r = 0.33) and diastolic (r = 0.27) BP. Hypertensive patients were divided into two groups by presence (group 1) and absence (group 2) of a reduction of both systolic and diastolic BP during the night by an average of more than 10% of the daytime pressure. Casual BP, ambulatory daytime systolic and diastolic BP, sex, body surface area, duration of hypertension, prevalence of diabetes, quantity of sleep during monitoring, funduscopic changes, and serum creatinine did not differ between the two groups. LV mass index, after adjustment for the age, the sex, the height, and the daytime BP differences between the two groups (analysis of covariance) was 82.4 g/m2 in the normotensive patient group, 83.5 g/m2 in hypertensive patients of group 1 and 98.3 g/m2 in hypertensive patients of group 2 (normotensive patients vs. group 1, p = NS; group 1 vs. group 2, p = 0.002).(ABSTRACT TRUNCATED AT 250 WORDS)     

Circadian rhythm and variability of blood pressure and target organ damage in essential hypertension

The aim of this study was to evaluate the influence of blood pressure variability and circadian rhythm on left ventricular mass and urinary albumin excretion rate (UAE) in patients with essential hypertension. 82 untreated patients (35 women and 47 men; mean age 41.1 +/- 13.7) were recruited to this study. Mean office blood pressure at entry was 152/97 mmHg. Ambulatory blood pressure monitoring (ABPM) was performed using an Medilog ABP recorder (Oxford). Blood pressure variability was estimated as the standard deviation (SD) of systolic and diastolic ambulatory blood pressure. Urinary albumin excretion (UAE) was estimated by the radioimmunoassay during two separate days. Echocardiography was used to measure left ventricular mass and left ventricular mass index (LVMI). The median urinary albumin excretion for the whole group was 8.2 mg/day; in 18 patients (21.9%) microalbuminuria was present. Left ventricular mass index in a whole group was 109.1 g/m2; in 23 subjects (28.0%) left ventricular hypertrophy (LVH) was found. Patients with microalbuminuria as well as with left ventricular hypertrophy had higher office and 24 hour ambulatory systolic and diastolic blood pressure and higher systolic blood pressure variability. During ABPM 18 patients with absent nocturnal fall in blood pressure (non-dippers) were found; they did not display more frequent prevalence of target organ damage. Increased 24-hour blood pressure variability present in hypertensive subjects with both microalbuminuria and left ventricular hypertrophy may suggest that this phenomenon plays role in development of target organ damage.