嗜铬细胞瘤/副神经节瘤患者血NSE水平与临床特征的关系

目的 研究血清神经元特异性烯醇化酶(NSE)与嗜铬细胞瘤/副神经节瘤(PPGL)临床特征的关系。方法 选取2019年1月至2022年12月诊断的501例PPGL患者,按照NSE正常(≤16.3 ng/mL)和NSE升高(>16.3 ng/mL)进行分组,比较两组临床特征的差别。结果 NSE升高组患者与NSE正常组相比,肿瘤原发灶直径更大(5.00 cm vs 4.60 cm),24 h尿去甲肾上腺素(NE),24 h尿多巴胺(DA)水平更高,出现转移病变的比例更高(31.6%vs 13.7%)(P<0.05)。NSE水平与肿瘤原发灶大小(r=0.131,P<0.05)、24 h尿NE水平(r=0.195,P<0.05)、24 h尿DA水平(r=0.119,P<0.05)成正相关。结论 在PPGL患者中,NSE水平与肿瘤大小、分泌功能以及是否发生转移相关。     

24小时尿香草扁桃酸、肾上腺素、去甲肾上腺素、多巴胺检测在嗜铬细胞瘤临床诊断中应用价值的比较研究

目的探索尿香草扁桃酸(vanillymandelic acid,VMA)和尿肾上腺素(epinephrine,E)、去甲肾上腺素(norepinephrine,NE)、多巴胺(dopamine,DA)等在嗜铬细胞瘤患者尿液中代谢的一般规律及评估其作为嗜铬细胞瘤辅助诊断指标的应用价值。方法收集2017年1～5月由我院收治的214例肾上腺疾病患者的24小时尿液样本,分别检测尿液样本中VMA、E、NE、DA指标的含量。结果嗜铬细胞瘤组尿VMA和尿E、NE、DA水平显著高于其它肾上腺疾病组(P<0.05)。VMA在嗜铬细胞瘤诊断中的ROC曲线下面积为0.857,灵敏度为78.9%,特异性为92.6%,显著优于E、NE、DA这三个指标的诊断效能,与E+DA+VMA联合检测的诊断效能差异无统计学差异。结论 24h尿VMA检测结果可以作为辅助诊断嗜铬细胞瘤的潜在指标。     

尿儿茶酚胺代谢物在嗜铬细胞瘤早期诊断中的临床意义

目的: 通过对尿中儿茶酚胺代谢产物氧甲基去甲肾上腺素(normetaneprine, NMN)及氧甲基肾上腺素(metaneprine, MN)的测定, 评价其在嗜铬细胞瘤(pheochromocytoma, PHEO)早期诊断中的临床意义. 方法: 采用ELISA法, 分别对一般高血压患者及临床诊断为嗜铬细胞瘤及肾上腺占位病变并伴有阵发性高血压患者24 h的NMN/MN进行测定.结果 : 46例疑为嗜铬细胞瘤患者尿中的NMN检测显著升高, 其中32例经本院计算机断层摄影(CT)或磁共振扫描(MR), 确诊为嗜铬细胞瘤, 其24 h 尿中的NMN/ MN检测结果全部升高, 而14例其他肿瘤患者检测仅为尿中的NMN值升高.同时检测排除嗜铬细胞瘤及其他肿瘤患者的50例一般高血压患者尿中的NMN/ MN值结果均显示正常.嗜铬细胞瘤患者尿液中NMN的水平为(1838±167)ìg/L, 对照组为(331±153)ìg/L, MN的水平为(663.2±231.1) ìg/L, 对照组为(135.4±78.5)ìg/L, 病患组NMN/MN测定值明显高于对照组( P＜0.001). 结论: 24 h NMN/MN ELISA检测为患者提供了一种无创性并具有高灵敏度、特异性好的简便方法.对临床从肾上腺占位性病变并伴有阵发性高血压患者中筛查嗜铬细胞瘤具有较高的早期诊断价值.     

肾上腺节细胞神经瘤与嗜铬细胞瘤的临床鉴别诊断及治疗

目的探讨肾上腺节细胞神经瘤与嗜铬细胞瘤的临床特点,诊断、鉴别诊断及治疗。方法回顾性分析1999—2005北京协和医院14例节细胞神经瘤及32例嗜铬细胞瘤患者的临床资料。节细胞神经瘤组男8例,女6例;年龄16～63岁;肿瘤直径2·5～15cm。嗜铬细胞瘤组男14例,女18例;年龄19～74岁;肿瘤直径2～16cm。全部患者均行手术治疗切除肿瘤并经病理证实。结果节细胞神经瘤患者多无临床症状,查体时偶然发现肾上腺区占位;CT多表现为形态规则的圆形或类圆形低密度影,边界光滑,质地均一,增强扫描强化不明显;24h尿儿茶酚胺在正常水平;131I-MIBG检测大部分阴性。嗜铬细胞瘤患者就诊时多有典型的阵发或持续性高血压症状;CT表现多为形态欠规则的实性、囊实性或单纯囊性占位,密度不均,增强后呈不规则明显强化;嗜铬细胞瘤内分泌功能活跃,24h尿儿茶酚胺检查显著升高;131I-MIBG检测大部分呈阳性,浓聚区域与占位病变位置相同。结论肾上腺节细胞神经瘤与嗜铬细胞瘤在临床表现、影像学、内分泌检查和131I-MIBG等均有显著差异。通过仔细询问病史,正确的实验室及辅助检查,术前基本能鉴别诊断。行开放或腹腔镜手术切除可取得良好的治疗效果。     

功能静止型嗜铬细胞瘤29例临床分析

目的回顾性分析29例功能静止型嗜铬细胞瘤的临床资料,以提高其诊治水平。方法分析2010年5月至2013年5月在北京协和医院接受手术并经病理证实的功能静止型嗜铬细胞瘤患者29例,总结患者年龄、肿瘤部位、肿瘤最大径、手术方式、术中血压变化、尿儿茶酚胺以及术前核医学检查等方面的临床特点。结果患者血压正常,尿儿茶酚胺正常或稍高,肿瘤位于双侧肾上腺1例,右侧肾上腺13例,左侧肾上腺15例,直径1.5～14 cm。腹腔镜手术切除25例,开放手术切除4例。23例术前接受药物准备,术中血压平稳,6例术前未行药物准备,其中2例术中出现血压剧烈波动。23例术前行生长抑素受体显像的患者中,有11例未见异常;11例术前行131IMIBG显像,1例未见异常。结论对于功能静止型嗜铬细胞瘤,尿儿茶酚胺及生长抑素受体显像敏感性较低,对于CT表现可疑的,建议术前行131IMIBG显像,一旦确诊,应该在充分药物准备的基础上接受手术治疗。     

首发精神分裂症患者抗N-甲基-D-天冬氨酸受体抗体和内源性皮质醇水平的关系

目的:探讨首发精神分裂症患者血清抗N-甲基-D-天冬氨酸受体抗体(NMDAR-Ab)和尿液内源性皮质醇/肌酐水平的关系。方法:纳入符合美国精神障碍诊断与统计手册第5版诊断标准的首发精神分裂症患者166例，正常对照129例。采用阳性和阴性症状量表(PANSS)评估患者的精神病理症状；血清抗NMDAR-Ab水平应用酶联免疫吸附法检测(ELISA);收集12 h尿液，采用免疫荧光法检测皮质醇和肌酐浓度，计算皮质醇/肌酐比(CCR)。结果:精神分裂症组血清抗NMDAR-Ab水平、12 h尿皮质醇水平、CCR均高于正常对照组(均P<0.001)。精神分裂症组的抗NMDAR-Ab水平与PANSS的阳性症状(r=0.22)、阴性症状(r=0.23)得分和总分(r=0.26)正相关(均P<0.05),与CCR正相关(r=0.30,P<0.001);正常对照组抗NMDAR-Ab水平与CCR之间相关性无统计学意义(P>0.05)。结论:首发精神分裂症患者存在较高的抗NMDAR-Ab水平，并和皮质醇水平升高相关，提示自身免疫系统异常可能参与精神分裂症疾病的发生，而身体通过反馈调节促进...     

功能静止型嗜铬细胞瘤29例临床分析简

 目的 回顾性分析29例功能静止型嗜铬细胞瘤的临床资料，以提高其诊治水平。 方法 分析2010年5月至2013年5月在北京协和医院接受手术并经病理证实的功能静止型嗜铬细胞瘤患者29例，总结患者年龄、肿瘤部位、肿瘤最大径、手术方式、术中血压变化、尿儿茶酚胺以及术前核医学检查等方面的临床特点。结果 患者血压正常，尿儿茶酚胺正常或稍高，肿瘤位于双侧肾上腺1例，右侧肾上腺13例，左侧肾上腺15例，直径1.5~14cm。腹腔镜手术切除25例，开放手术切除4例。23例术前接受药物准备，术中血压平稳，6例术前未行药物准备，其中2例术中出现血压剧烈波动。23例术前行生长抑素受体显像的患者中，有11例未见异常；11例术前行131IMIBG显像，1例未见异常。结论 对于功能静止型嗜铬细胞瘤，尿儿茶酚胺及生长抑素受体显像敏感性较低，对于CT表现可疑的，建议术前行131IMIBG显像，一旦确诊，应该在充分药物准备的基础上接受手术治疗。     

恶性嗜铬细胞瘤,宫内妊娠,产后出血

1病例摘要患者女性,37岁,G2P1,因“恶性嗜铬细胞瘤术后复发,宫内妊娠32 3周”入院。1.1病史19年前因“发作性头痛、高血压”考虑“左肾上腺嗜铬细胞瘤”,于外院手术切除主动脉旁直径3cm的肿瘤。病理:嗜铬细胞瘤,少数瘤细胞有明显异型。术后血压降至正常,后未随诊。4年前因“胸骨柄肿瘤,左锁骨上窝肿物”行胸骨柄切除,胸廓重建,左锁骨后肿瘤切除,颈部清扫术。病理:胸骨柄、左颈淋巴结转移性嗜铬细胞瘤。术中血压最高220/100mmHg,切除瘤体后血压一度降到80/40mmHg,予以多巴胺维持血压100/60mmHg。术后4月复查血压和24h尿儿茶酚胺正常。术后1…     

INSM1在嗜铬细胞瘤/副神经节瘤和肾上腺皮质腺瘤中的表达和病理诊断价值

目的 探讨胰岛素瘤相关蛋白1(insulinoma-associated protein 1, INSM1)在嗜铬细胞瘤/副神经节瘤和肾上腺皮质腺瘤中的表达及其在鉴别诊断中的意义。方法 采用免疫组化EnVision两步法检测INSM1在嗜铬细胞瘤/副神经节瘤和肾上腺皮质腺瘤中的表达。结果 32例嗜铬细胞瘤中31例INSM1阳性(31/32,96.88%),其中高表达20例(20/32,62.50%)。9例肾上腺外副神经节瘤INSM1均阳性,其中高表达8例(8/9,88.89%)。33例肾上腺皮质腺瘤中INSM1均阴性。INSM1在嗜铬细胞瘤/副神经节瘤中的表达显著高于肾上腺皮质腺瘤(P<0.001)。INSM1高表达的嗜铬细胞瘤/副神经节瘤具有更高的Ki67增殖指数(P=0.016),但与患者性别(P=0.190)、年龄(P=0.439)、肿瘤TNM分期(P=0.793)、生长模式(P=0.495)、凝固性坏死(P=0.790)和脉管/包膜侵犯(P=0.790)均无显著相关性。INSM1鉴别嗜铬细胞瘤/副神经节瘤与肾上腺皮质腺瘤的敏感性为97.6%,特异性为100%,ROC曲线下...     

尿儿茶酚胺检测对嗜铬细胞瘤诊断的评价

嗜铬细胞瘤是肾上腺髓质、交感神经节以及其它任何肾上腺素能受体系统的嗜铬组织能产生过多儿茶酚胺的肿瘤[1].临床上主要以心血管系统症状,尤其以不同类型继发高血压为主,兼有其它系统代谢紊乱症群.近年对来本院诊治的继发高血压,影像学定位疑似嗜铬细胞瘤患者进行尿儿茶酚胺检测,其结果报道如下:     

Measurement of norepinephrine and 3,4-dihydroxyphenylglycol in urine and plasma for the diagnosis of pheochromocytoma

We compared the value of plasma samples with that of 24-hour urine samples in identifying patients with pheochromocytoma among those with hypertension. We employed specific gas chromatographic-mass spectrometric analysis of both urine and plasma for simultaneous assay of norepinephrine and its neuronal metabolite 3,4-dihydroxyphenylglycol (DHPG). The study population consisted of 1086 patients with hypertension, among them 25 patients with proved pheochromocytoma. Reference ranges for free norepinephrine and DHPG in plasma and urine were established. Measurement of free norepinephrine in 24-hour urine samples provided the best index of a pheochromocytoma. This technique had 100 percent sensitivity and 98 percent specificity among 1192 urine samples, as compared with 82 percent sensitivity and 95 percent specificity among 358 plasma samples. Simultaneous measurement of norepinephrine and DHPG in urine further improved specificity (to 99 percent), but the use of the ratio of norepinephrine to DHPG reduced sensitivity (to 95 percent), since some patients with pheochromocytoma secrete large amounts of DHPG. We therefore recommend measurement of 24-hour urinary levels of free norepinephrine for the diagnosis of pheochromocytoma and suggest that simultaneous analysis for DHPG may sometimes prove useful in reducing the rate of false positive results.     

产后抑郁症血浆儿茶酚胺浓度对照研究

产后抑郁症是一组严重程度不等的不良情绪或情感障碍 ,一般认为其发病与分娩前后诸多社会 ,心理及生物因素有关。在社会 ,心理方面 ,国内外研究较多[1,2 ] ,但在生物因素方面 ,国内外研究相对较少。众所周知 ,抑郁症发病与儿茶酚胺类递质 (CAs)改变有关 ,那么 ,产后抑郁症血浆CAs浓度情况如何 ?与产前、正常人及非产后抑郁症有否差异 ?本文对此进行了对照研究。1　对象与方法1.1　对象1.1.1　产前组　为 1998年 3月～ 5月在深圳市第一人民医院和深圳市妇儿医院住院待产妇 ,小学以上文化 ,既往无重大伤病史及精神病史 ,经用精神症状自评量…     

Circulating and urinary catecholamines in pheochromocytoma. Diagnostic and pathophysiologic implications.

Three biochemical tests for the diagnosis of pheochromocytoma were evaluated in 24 patients with proved tumors and 40 patients whose clinical picture was suspect but who had no evidence of the disease. Measurement of resting, supine plasma catecholamines (by radioenzymatic assay) was more useful than either 24-hour urinary vanillylmandelic acid (VMA) or metanephrines or both. In only one of 23 patients with pheochromocytoma were plasma catecholamines within the range of those in patients without pheochromocytoma, as compared with urinary VMA in 11 of 22, urinary metanephrines in five of 22 and both metabolites in three of 22. These studies reaffirm the value of plasma catecholamines in the diagnosis of pheochromocytoma and indicate that urinary catecholamine metabolites are less useful. The poor correlation between the height of arterial pressure and circulating levels of catecholamines suggests that the regulation of arterial pressure in pheochromocytoma is complex.     

Association between sympathetic activity and the atherogenic serum cholesterol fraction

Summary A possible modulating influence of nor-adrenergic activity on serum lipoproteins was assessed under placebo conditions and following 4 weeks of sympathetic neurone blockade with debrisoquine in 9 normal subjects, 11 patients with mild essential hypertension, 9 normotensive, and 9 hypertensive hemodialysis patients. Plasma nor-epinephrine (NE) did not differ significantly among groups on placebo and was consistently reduced (P<0.05–0.001) by sympathetic blockade. The latter also decreased (P<0.05–0.001) plasma total cholesterol (C) as well as low and very low density lipoprotein cholesterol (LDL + VLDL-C) in the three patient groups. In the two dialysis groups, basal levels of plasma triglycerides (Tg) were increased and high density lipoprotein cholesterol (HDL-C) was diminished (P<0.01–0.001); sympathetic blockade lowered Tg and raised HDL-C (P<0.01–0.001). In normal subjects, sympathetic blockade did not significantly modify plasma lipoproteins. In the three patient groups, significant correlations (r=0.62–0.88;P<0.05–< 0.001) existed between (a) basal plasma NE and total C or LDL + VLDL-C and (b) debrisoquineinduced changes in NE and changes in total LDL + VLDL-C. These findings suggest that in essential hypertension as well as in hemodialysis patients, the atherogenic C fraction, represented by LDL + VLDL-C, may be modulated by the nor-adrenergic activity.Supported by the Schweizerischer Nationalfonds für wissenschaftliche Forschung     

慢性肾功能衰竭患者血浆同型半胱氨酸及血脂水平变化

目的 :了解慢性肾功能衰竭 (CRF)患者血浆中同型半胱氨酸(Hcy)和血脂水平的变化和透析治疗CRF前后血浆中Hcy水平的变化。方法 :以微板底物诱导法测定CRF患者、冠心病 (CHD)患者和健康者血浆中Hcy和其它血脂指标的浓度 ,并讨论与分析。 结果 :CRF患者血浆Hcy水平明显高于正常组 (P <0 .0 5 )。透析治疗观察发现CRF患者血液透析后 2hHcy水平较透析前下降 45 .7% ,透析后 2 4hHcy水平较透析前仅下降 8.9%。CRF患者血清Hcy和TG之间存在正相关性 (r =0 .719)。结论 :CRF患者普遍存在高Hcy血症 ,动态观察患者血脂、Hcy和肾功能水平 ,对了解病情进展和治疗效果有积极作用     

Conjugated catecholamines in birds

Large concentrations of dopamine (DA) glucuronide were measured in the plasma of chronically cannulated adult chickens (Gallus gallus), adult ducks (Anas platyrhynchos) and 1-month-old ducklings. Glucuronides of norepinephrine (NE) and epinephrine (E) also were present but at much lower concentrations. Sulfoconjugated DA, NE and E could be detected only in chickens. There were quantitative differences between chicken and duck catecholamine metabolism: (i) glucuronoconjugates and sulfoconjugates comprised large portions of the total DA, NE and E in chicken plasma; (ii) the predominant form of DA in duck plasma was DA glucuronide while most of the NE and E circulated in unconjugated (i.e. free) form. Catecholamine levels did not differ between adult and immature ducks. Angiotensin II injection into adult ducks elicited simultaneous increases in arterial blood pressure (Pa) and free NE but did not alter conjugated catecholamine levels.     

Pheochromocytoma with negative urinalysis in pregnancy

We present a rare case of adrenal pheochromocytoma in pregnancy, with serial 24-h urine specimen collections showing normal concentrations of catecholamine metabolites. The diagnosis was based on clinical presentation, abdominal ultrasound, and magnetic resonance imaging, and was confirmed on post-operative pathohistological examination. Clinical suspicion of pheochromocytoma in pregnancy should be sufficient to implement adequate therapeutic measures, regardless of urine catecholamine concentrations.     

Evaluation of the relationship between IL-18 levels in urine and parameters of renal pathological changes in patients with lupus nephritis]

AIM: To evaluate the relationship between IL-18 levels in urine and parameters of renal pathological changes in patients with lupus nephritis (LN). METHODS: IL-18 levels in morning free urine and 24-hour's urine in 19 normal persons and 55 patients with LN were measured by ELISA. The correlation between IL-18 levels and parameters of renal pathological changes, namely activity index (AI) and chronicity index (CI), were analyzed by liner correlation analysis method. RESULTS: IL-18 levels in morning free urine and 24-hour's urine in LN group were elevated significantly compared with control group. In both groups IL-18 levels in morning free urine were (247.1+/-317.5) ng/L and (20.3+/-14.5) ng/L, respectively, P<0.001; those in 24-hour's urine were (192.1+/-170.1) ng/d and (21.0+/-3.8) ng/d, respectively, (P<0.001). There was close positive correlation between IL-18 levels in morning free urine and 24-hour's urine and LN patient's AI (for morning free urine: r=0.602, P<0.001; for 24-hour's urine: r=0.461, P<0.005) but there was no correlation between IL-18 levels in morning urine and 24-hour's urine and CI (P>0.05). Patients with LN were divided into three groups (high, moderate and low) according to AI value. There was distinct difference of IL-18 levels in urine among the three groups: IL-18 levels in morning urine were (69.2+/-82.7) ng/L, (193.5+/-106.1) ng/L and (580.7+/-453.1) ng/L, respectively, (P<0.001); those in 24-hour's urine were (103.5+/-141.4) ng/d, (188.8+/-124.0) ng/d and (333.1+/-183.2) ng/d, respectively. CONCLUSION: It is very simple and convenient to detect IL-18 levels in morning free urine, so it is a good method for evaluating renal pathological activity of LN.     

The diagnosis of atypical pheochromocytoma: a challenge for the biologist as well

Biological diagnosis of pheochromocytoma is relatively easy in those cases releasing great amounts of catecholamines with strong clinical features; instead, diagnosis could be more problematic in atypical or asymptomatic familial pheochromocytoma with small tumors secreting low catecholamine amounts. Several plasma and urine adrenergic markers must be used to confirm the clinical suspicion. We have discussed the biological data of three totally asymptomatic pheochromocytomas (cases no 2, 3, 4) and one case with a very discrete clinical manifestation (no 1). Three patients had very small tumors (4, 7 and 25 g) secreting preeminently adrenaline, one patient had a 45 g adrenal incidentaloma without clinical expression. Our study shows that, in these special cases, except for an inconstant increase of adrenaline, plasma and urine catecholamines and urine VMA can be normal. The most useful markers are plasma and urine methoxyamines. However, plasma methoxyamines are the most sensitive because their increase over reference values is by far greater than in urines. Several factors may explain these findings: a low tumoral secretion, the nature of the released amine, the short half-life of catecholamines in plasma and, in some cases, the involvement of intratumoral catecholamine metabolism. Analysis of the ratio NMN/MN in plasma provides an additional diagnosis tool to reveal adrenaline secretion abnormalities.     

The biochemical basis of relapse and drug response in schizophrenia: review and hypothesis.

This review of the literature suggests that antipsychotic drug response is determined by dopamine (DA) turnover and norepinephrine (NE) activity prior to treatment. The data suggest that NE modulates the DA system. Drug-free psychotic patients with relatively increased DA and NE activity, including release, are more likely to be treatment responsive, while patients who show evidence of enhanced DA and NE activity during treatment with antipsychotic drugs are likely to relapse soon after neuroleptic withdrawal. Basal release of DA and NE is decreased and associated with residual positive and negative symptoms. Improvement during neuroleptic treatment is associated with decreases in DA and NE phasic or stimulus induced release. The variable response to antipsychotic drugs is most likely to be a result of dysregulated DA and NE release, i.e. under state-dependent control, rather than evidence of a heterogeneous aetiology. Because catecholamines regulate gain, signal-to-noise ratio and gating in the brain, this model allows for environmental factors to interact with biochemical state and drug treatment. The author proposes that impaired homeostasis of NE and DA in schizophrenia causes instability in NE and DA neuronal firing and release, presumably related to mechanisms down-stream from the receptors, such as G proteins. This instability of catecholamine release may explain the observed variability in clinical states and drug response in schizophrenia.