双侧岩下窦静脉取血联合去氨加压素刺激试验诊断库欣病的价值

目的 探讨双侧岩下窦静脉取血(BIPSS)过程中联合去氨加压素(DDAVP)刺激试验在库欣病诊断中的意义.方法 16例垂体MRI未见明确肿瘤的ACTH依赖性库欣综合征患者行大剂量地塞米松抑制试验、BIPSS联合DDAVP刺激试验等检查.DDAVP刺激前及刺激后5 min、10 min测定岩下窦静脉与外周静脉血ACTH的比值.比值＞2者考虑库欣病.比较DDAVP刺激的BIPSS及大剂量地塞米松抑制试验和术后诊断的一致性.结果 16例库欣病患者中9例(56.3％)大剂量地塞米松试验可抑制,BIPSS联合DDAVP试验中DDAVP刺激前12例(75.0％)岩下窦静脉与外周静脉血ACTH的比值＞2;刺激后14例(87.5％)患者比值＞2.介入手术过程中未出现明显严重不良反应.结论 BIPSS联合DDAVP刺激试验可用于指导影像学检查无明确结果的库欣病患者的鉴别诊断和治疗.     

肾上腺占位合并垂体病变的库欣综合征九例临床分析并文献复习

目的探讨垂体及。肾上腺均有病变的库欣综合征患者的病因诊断方法。方法回顾性分析9例垂体及肾上腺均病变的患者,行大小剂量地塞米松抑制试验,查血促。肾上腺皮质激素（ACTH）水平,并行鞍区MRI及肾上腺CT。结果（1）血ACTH值在4例库欣病患者中3例高于正常上限,1例正常。5例非ACTH依赖性库欣综合征患者ACTH均〈20ng／L。（2）大剂量地塞米松抑制试验服药第2日24小时尿游离皮质醇在ACTH依赖组均可被抑制到对照值的20％以下,ACTH非依赖组5例患者均未被抑制到对照的50％以下。（3）术后病理支持临床诊断。结论对于兼有肾上腺及垂体两处病变的库欣综合征患者需采用多种方法进行鉴别,最后的判定是术后病理及疗效。     

ACTH非依赖性大结节样肾上腺增生

探讨促肾上腺皮质激素(ACTH)非依赖性大结节样肾上腺增生(AIMAH)的临床和病理特点。AIMAH是不依赖ACTH的库欣综合征,血浆ACTH水平低,大剂量地塞米松抑制试验不被抑制。CT示双侧肾上腺结节样增大,病理改变为双侧肾上腺结节样或腺瘤样增生,双侧肾上腺切除术后不发生Nelson综合征。AIMAH有特殊的临床病理特点,是库欣综合征的一种病因。     

ACTH非依赖性大结节样肾上腺增生

探讨促肾上腺皮质激素（ACTH）非依赖性大结节样肾上腺增生（AIMAH）的临床和病理特点。AIMAH是不依赖ACTH的库欣综合征，血浆ACTH水平低，大剂量地塞米松抑制试验不被抑制。CT示双侧肾上腺结节样增大，病理改变为双侧肾上腺结节样或腺瘤样增生，双侧肾上腺切除术后不发生Nelson综合征，AIMAH有特殊的临床病理特点，是库欣综合征的一种病因。     

促肾上腺皮质激素依赖性库欣综合征的诊断与治疗策略

促肾上腺皮质激素(ACTH)依赖性库欣综合征占库欣综合征病例总数的80%,主要包括垂体性库欣综合征和异位ACTH综合征.ACTH依赖性库欣综合征的诊断和治疗比较复杂,也比较困难.近年来,本病的诊断和治疗有很大的进步,但还存在不少问题.现对目前广泛应用于ACTH依赖性库欣综合征的各种诊断、鉴别诊断及治疗方法进行介绍和评价,并讨论治疗方法的选择以及治疗效果的评估.还对国际内分泌学会于2008年发表的关于库欣综合征的诊断指南及欧洲神经内分泌学会和垂体学会关于ACTH依赖性库欣综合征治疗的共识作简要介绍.     

联合法与经典法地塞米松抑制试验诊断Cushing综合征价值的比较

目的 评价联合法及经典法小及大剂量地塞米松抑制试验（DST）诊断Cushing综合征的价值。方法 回顾性分析北京协和医院1997-2006年152例经手术病理证实的Cushing综合征病例，将经典法与联合法小及大剂量DST结果与病理诊断结果相比较。结果 过夜小剂量DST、经典小剂量DST和联合法之小剂量DST对Cushing综合征定性诊断的符合率分别为97.5％、96．1％和97．6％。3组之间差异无统计学意义（P=0.86）。经典大剂量、联合法之大剂量DST时Cushing病诊断的符合率分别为80．6％、76.4％，2组之间差异无统计学意义（P=0．73）。经典大剂量、联合法之大剂量DST对肾上腺皮质激素（ACTH）非依赖性Cushing综合征诊断的符合率分别为94．2％和95．5％，2组之间差异无统计学意义（P=0．83）。经典法分别行小及大剂量和联合法小及大剂量DST诊断Cushing病的敏感性分别为81．5％和77．8％，特异性分别为92．5％和95．5％。结论 联合法小及大剂量DST比经典法分别行小及大剂量DST操作简便，节省时间，对照值更加准确，2种方法对诊断的符合率差异没有统计学意义，联合法小及大剂量DST可用于Cushing综合征的定性、定位诊断。     

库欣综合征55例临床分析

目的分析库欣综合征患者的病因分类,各种主要临床表现的出现频率以及实验室检查特点,并对各种诊断方法的效率进行评价。方法收集2003～2008年在我院住院经临床和病理确诊为库欣综合征患者的病例资料,对它们进行回顾性分析。结果在本组病例中,库欣病最多见,肾上腺皮质腺瘤次之。临床表现方面,向心性肥胖和高血压出现的频率最高,均在70％以上。血糖异常和血脂异常的发生率分别为51．43％和70．59％,其中糖尿病的比例高达37．14％。四成以上的患者存在低血钾,肾上腺皮质腺癌患者100％有低血钾,且是重度低血钾。在库欣综合征诊断试验中,敏感性从高到低依次为不被小剂量地塞米松抑制,血皮质醇昼夜节律消失,24小时尿游离皮质醇增高,清晨血皮质醇增高。在库欣综合征患者的鉴别诊断中,80％以上的库欣病患者能被大剂量地塞米松抑制,而几乎94％的肾上腺皮质腺瘤患者不能被大剂量地塞米松抑制。有关影像学检查,垂体MRI可以检测出90％以上的库欣病患者存在垂体腺瘤,而肾上腺CT几乎100％可以发现出肾上腺肿瘤。结论本研究中库欣病和肾上腺皮质腺瘤仍然是最常见的病因,但后者的的比例相对较高。而在临床特点方面,高血压和糖尿病的发生率较高。对于库欣综合征的诊断和鉴别诊断,目前没有一种检查方法具有100％的敏感性,我们应对各种结果作出综合判断。     

联合法与经典法地塞米松抑制试验诊断Cushing综合征价值的比较

目的评价联合法及经典法小及大剂量地塞米松抑制试验(DST)诊断Cushing综合征的价值。方法回顾性分析北京协和医院1997—2006年152例经手术病理证实的Cushing综合征病例,将经典法与联合法小及大剂量DST结果与病理诊断结果相比较。结果过夜小剂量DST、经典小剂量DST和联合法之小剂量DST对Cushing综合征定性诊断的符合率分别为97.5%、96.1%和97.6%。3组之间差异无统计学意义(P=0.86)。经典大剂量、联合法之大剂量DST对Cushing病诊断的符合率分别为80.6%、76.4%,2组之间差异无统计学意义(P=0.73)。经典大剂量、联合法之大剂量DST对肾上腺皮质激素(ACTH)非依赖性Cushing综合征诊断的符合率分别为94.2%和95.5%,2组之间差异无统计学意义(P=0.83)。经典法分别行小及大剂量和联合法小及大剂量DST诊断Cushing病的敏感性分别为81.5%和77.8%,特异性分别为92.5%和95.5%。结论联合法小及大剂量DST比经典法分别行小及大剂量DST操作简便,节省时间,对照值更加准确,2种方法对诊断的符合率差异没有统计学意义,联合法小及大剂量DST可用于Cushing综合征的定性、定位诊断。     

论著外周DDAVP兴奋试验在促肾上腺皮质激素依赖性库欣综合征鉴别诊断中的价值

目的 评价外周1-脱氨-8-精氨酸血管加压素(DDAVP)兴奋试验,在促肾上腺皮质激素(ACTH)依赖性库欣综合征鉴别诊断中的价值。方法 回顾性分析2012年1月至2013年6月在北京协和医院诊断明确的ACTH依赖性库欣综合征患者的临床资料,包括外周DDAVP兴奋试验、岩下窦静脉取血、鞍区MRI和手术病理的结果,评价外周DDAVP兴奋试验对诊断垂体ACTH腺瘤的敏感度和特异度。另收集10例文献报告的异位ACTH肿瘤患者资料,纳入统计分析。通过比较岩下窦静脉取血和外周DDAVP兴奋试验结果的差异,分析外周DDAVP兴奋试验中ACTH不升高的原因和此项检查的局限性。结果 (1)共纳入41例ACTH依赖性库欣综合征患者,其中28例为垂体ACTH腺瘤(简称库欣病),13例为异位分泌ACTH肿瘤。(2)如以ACTH升高到基础值1.5倍作为阳性反应标准,82%(23/28)库欣病和38%(5/13)的异位ACTH肿瘤患者可达到此反应。如以ACTH升高到基础值3倍为反应标准,50%(14/28)的库欣病患者达此标准,而异位ACTH肿瘤为0(0/13)。(3)有12例库欣病患者同时进行岩下窦静脉取血。其中7例外周DDAVP试验中ACTH水平未达到基础值的3倍,但岩下窦ACTH水平在DDAVP刺激后明显升高。结论 外周DDAVP兴奋试验有助于ACTH依赖性库欣综合征的鉴别诊断。ACTH升高幅度越大,越支持库欣病的诊断。如ACTH升高到基础值3倍以上,可确诊为库欣病。     

非ACTH依赖性肾上腺皮质大结节增生症

目的 探讨非ACTH依赖性双侧肾上腺皮质大结节增生症(AIMAH)的临床特点。方法 分析4例AIMAH临床和实验室资料，结合有关文献进行讨论。结果 4例AIMAH均有库欣综合征的临床表现及生化异常，实验检查显示血浆AcTH水平低下，皮质醇分泌节律紊乱，小剂量、大剂量地塞米松抑制试验均不被抑制，CT检查示双侧肾上腺明显增大呈结节样改变。结论 AIMAH是库欣综合征中病因独特的临床亚型，有其特有的临床特点及病理改变。     

胰岛素瘤的解剖分布特点分析

目的胰岛素瘤是最常见的胰腺神经内分泌肿瘤，因其临床表现多样，导致诊断困难。影像学诊断尤其是超声内镜(EUS)在胰岛素瘤的诊断中起着重要作用，拥有较高的敏感性和特异性。本研究拟通过明确胰岛素瘤的解剖分布特点，以期有助于提高影像学的诊断准确率和降低漏诊率，尤其是在教育和培训实践中对于EUS的学习者更具有指导价值。 方法回顾性分析解放军总医院第一医学中心病案资料数据库1993年1月至2019年11月经外科手术、病理确诊为胰岛素瘤的患者的临床资料，检索方法采取搜索术后病理诊断为"胰岛素瘤"的病例，通过查阅病例的方法，提取出胰岛素瘤的大小和解剖分布等数据，进一步分析其特点。 结果共检索到确诊为胰岛素瘤的患者116例，其中，男45例、女71例，年龄13～76岁，平均年龄(44.4±14.85)岁。胰岛素瘤单发110例(94.8%)、多发6例(5.2%)。位置分布：头颈部46例(39.7%)，单发45例、多发1例；体尾部68例(58.6%)，单发65例、多发3例；全胰腺多发2例(1.7%)。病变大小特点：最大径0.4～3.4 cm，平均大小(1.53±0.58)cm。≤1 cm 29例、>1 cm而≤1.5 cm41例、>1.5 cm而≤2.0 cm28例，≤3 cm 15例，>3 cm 3例。年龄与肿瘤的大小相关，≤44岁患者肿瘤平均大小为(1.36±0.51)cm、>44岁患者肿瘤平均大小为(1.70±0.60)cm，P<0.05。头颈部的肿瘤大于体尾部的肿瘤，头颈部肿瘤平均大小(1.66±0.63)cm，体尾部(1.42±0.52)cm，P<0.05。 结论胰岛素瘤在胰腺体尾部较头颈部更好发；绝大多数单发，但可以全胰腺多发；多数小于1.5 cm，肿瘤的大小与患者年龄和肿瘤的解剖分布相关。     

Pathogenesis of carcinoma of the papilla of Vater

Most adenomas and carcinomas of the small intestine and extrahepatic bile ducts arise in the region of the papilla of Vater. In familial adenomatous polyposis (FAP) it is the main location for carcinomas after proctocolectomy. In many cases symptoms due to stenosis lead to diagnosis at an early tumor stage. In about 80%, curative intended resection is possible. Operability is the most relevant prognostic factor. Most ampullary carcinomas resp. carcinomas of the papilla of Vater develop from adenomatous or flat dysplastic precursor lesions. They can be sited in the ampulloduodenal part of the papilla of Vater, which is lined by intestinal mucosa. They also can develop in deeper parts of the ampulla, which are lined by pancreaticobiliary duct mucosa. Intestinal-type adenocarcinoma and pancreaticobiliary-type adenocarcinoma represent the main histological types of ampullary carcinoma. Furthermore, there exist unusual types and undifferentiated carcinomas. Many carcinomas of intestinal type express the immunohistochemical marker profile of intestinal mucosa (keratin 7?, keratin 20+, MUC2+). Carcinomas of pancreaticobiliary type usually show the immunohistochemical profile of pancreaticobiliary duct mucosa (keratin 7+, keratin 20?, MUC2?). Even poorly differentiated carcinomas, as well as unusual histological types, may conserve the marker profile of the mucosa they developed from. These findings underline the concept of histogenetically different carcinomas of the papilla of Vater which develop either from intestinal- or from pancreaticobiliary-type mucosa of the papilla of Vater. Molecular alterations in ampullary carcinomas are similar to those of colorectal as well as pancreatic carcinomas, although they appear at different frequencies. In future studies, molecular alterations in ampullary carcinomas should be correlated closely with the different histologic tumor types. Consequently, the histologic classification should reflect the histogenesis of ampullary tumors from the two different types of papillary mucosa.     

Lack of correlation of degree of villous atrophy with severity of clinical presentation of coeliac disease

BACKGROUND AND AIM: Both the clinical presentation and the degree of mucosal damage in coeliac disease vary greatly. In view of conflicting information as to whether the mode of presentation correlates with the degree of villous atrophy, we reviewed a large cohort of patients with coeliac disease. PATIENTS AND METHODS: We correlated mode of presentation (classical, diarrhoea predominant or atypical/silent) with histology of duodenal biopsies and examined their trends over time. RESULTS: The cohort consisted of 499 adults, mean age 44.1 years, 68% females. The majority had silent coeliac disease (56%) and total villous atrophy (65%). There was no correlation of mode of presentation with the degree of villous atrophy (p=0.25). Sixty-eight percent of females and 58% of males had a severe villous atrophy (p=0.052). There was a significant trend over time for a greater proportion of patients presenting as atypical/silent coeliac disease and having partial villous atrophy, though the majority still had total villous atrophy. CONCLUSIONS: Among our patients the degree of villous atrophy in duodenal biopsies did not correlate with the mode of presentation, indicating that factors other than the degree of villous atrophy must account for diarrhoea in coeliac disease.     

Demonstration of the mechanism of action of biguanides

Summary Palmitic acid oxidation in rat diaphragm homogenate is depressed by biguanide concentrations that are still incapable of inhibiting oxidative phosphorylation. Glucose oxidation is not directly effected by the same biguanide concentrations: however, the inhibitory effect of palmitic acid on glucose oxidation is partly removed by biguanides. Inhibition of fatty acid oxidation, which accounts for most of the metabolic effects caused by these drugs, can be regarded as the fundamental mechanism of action of biguanides. There is some evidence suggesting that these drugs might interact with carnitine, thus preventing long-chain fatty acids from being transported across the mitochondrial membrane to the site of oxidation. Traduzione a cura degli AA.     

血吸虫童虫生长发育及其机制的研究进展

血吸虫童虫是宿主免疫系统攻击的重要靶标,包括皮肤型、肺型和肝门型童虫。宿主分子对童虫生长发育具有重要作用。童虫生长发育机制包括免疫调节、信号转导、性别发育及凋亡等。肌动蛋白、组织蛋白酶、烯醇化酶和葡萄糖基转移酶等分子为血吸虫童虫生长发育的重要分子。本文对血吸虫童虫生长发育及其机制的研究进展做一综述。     

124例耐多药结核分枝杆菌基因突变特征分析

目的对临床分离的耐多药结核分枝杆菌相关基因的突变特征进行分析。方法对124例耐多药结核分枝杆菌以及50株敏感株的耐药相关基因(包括异烟肼inh A、kat G、oxyR-ahp C间隔区以及利福平rpo B)进行序列测定,分析其基因突变情况。结果异烟肼耐药inh A基因突变率为14.5%;kat G基因突变率为70.2%(87/124),主要位于315位;oxyR-ahp C间隔区突变率为15.3%;inh A、kat G两种基因同时突变率75.0%,三种基因同时突变率为89.5%。利福平rpo B基因突变的检出率高达95.2%,突变主要发生在531、526、516位点。结论我省耐多药菌异烟肼耐药相关基因最常见突变为kat G 315、inh A C-T(-15)、axyR-ahp C间隔区(-10)C-T,利福平为rpo B531、526、516。结合MDR-TB耐药相关基因的特征分析,可以建立一种快速、准确、特异的适合于我省的检测结核菌耐多药性的新方法。     

Assessment of quality of life of parents of children with juvenile chronic arthritis

The aim of the study was to assess the quality of life (QOL) and the psychological status of parents of children with juvenile chronic arthritis (JCA). The QOL, anxiety and depression of the parents of 28 children with JCA were evaluated and compared to those of the parents of 28 healthy children. Mothers of JCA children and mothers of healthy children reported similar QOL. The reported anxiety and depression levels were similar for mothers and fathers in both groups. The parents of children with pauciarticular-type JCA reported lower QOL and higher levels of anxiety and depression than the parents of children with other types, namely polyarticular and systemic JCA. These findings may be explained by the fact that the pauciarticular patients had shorter disease duration and were less frequently seen in the outpatient clinic. The QOL of mothers of children with JCA was found to be slightly impaired in the group of children with pauciarticular JCA. Future larger studies are needed to confirm these results, as the number of subjects in the three groups was rather low. Received: 26 September 2001 / Accepted: 8 February 2002     

氯硝柳胺悬浮剂的毒性评价

目的评价氯硝柳胺悬浮剂的毒性，为现场大规模应用灭螺提供依据。方法按照中华人民共和国国家标准GB 15670-1995《农药登记毒理学试验方法》和鱼类毒性试验方法进行。结果经口、经皮肤的LDso雌、雄性大鼠均>5 000 mg/kg，经呼吸道的LCso雌、雄性大鼠均>5 000mg/m3，该药经口、经皮肤、经呼吸道毒性均属微毒类药物；兔眼用药后，观察期内无不良反应，对眼无刺激性；皮肤用药后对皮肤无刺激性。与氯硝柳胺原药、氯硝柳胺乙醇胺盐原药和氯硝柳胺乙醇胺盐可湿性粉剂相比，氯硝柳胺悬浮剂对鱼急性毒性最低。结论氯硝柳胺悬浮剂属微毒类药物，对鱼的毒性低于其乙醇胺盐可湿性粉剂，适合于现场应用。     

Numerical Simulation of the Effect of Rate of Change of Glucose on Measurement Error of Continuous Glucose Monitors

Background

A 5-day in-patient study designed to assess the accuracy of the FreeStyle Navigator® Continuous Glucose Monitoring System revealed that the level of accuracy of the continuous sensor measurements was dependent on the rate of glucose change. When the absolute rate of change was less than 1 mg•dl⁻¹•min⁻¹ (75% of the time), the median absolute relative difference (ARD) was 8.5%, with 85% of all points falling within the A zone of the Clarke error grid. When the absolute rate of change was greater than 2 mg•dl⁻¹•min⁻¹ (8% of the time), the median ARD was 17.5%, with 59% of all points falling within the Clarke A zone.

Method

Numerical simulations were performed to investigate effects of the rate of change of glucose on sensor measurement error. This approach enabled physiologically relevant distributions of glucose values to be reordered to explore the effect of different glucose rate-of-change distributions on apparent sensor accuracy.

Results

The physiological lag between blood and interstitial fluid glucose levels is sufficient to account for the observed difference in sensor accuracy between periods of stable glucose and periods of rapidly changing glucose.

Conclusions

The role of physiological lag on the apparent decrease in sensor accuracy at high glucose rates of change has implications for clinical study design, regulatory review of continuous glucose sensors, and development of performance standards for this new technology. This work demonstrates the difficulty in comparing accuracy measures between different clinical studies and highlights the need for studies to include both relevant glucose distributions and relevant glucose rate-of-change distributions.     

治疗高血压药物的经济学评价

重视高血压治疗中的经济学评价,对利用我国有限的卫生资源来遏制高血压对人民群众的危害有着重要的现实意义。药物经济学对于药物治疗的成本和治疗的结果给予同样的关注。因为治疗高血压的费用,不仅涉及药物价格,还包括患者的危险水平,降压疗效和对临床终点事件的影响,以及治疗的依从性和安全性。因此药物经济学更强调整体成本和价-效比。低危病人,若非药价低廉,治疗的价-效比不够理想。而在高危的患者,价-效比越小越经济而不是药费越便宜越好。