肺动脉瓣缺如综合征的外科治疗

目的:探讨肺动脉瓣缺如综合征(APVS)的外科治疗经验。方法:1994年12月至2006年11月共治疗APVS患儿5例,其中男性4例,女性1例。5例均伴法洛四联症,其中1例伴左肺动脉起源异常,1例伴左肺动脉狭窄,发育不良。全组患儿均在中低温体外循环下行根治手术。其中4例患儿肺动脉瓣区置带瓣补片,1例采用牛心包直接补片。结果:4例存活,1例死亡,术后平均随访(13.8±5.76)个月。超声心动图检查示肺动脉瓣少量返流,右心室至肺动脉平均流速及压差较术前明显下降(P<0.01)。结论:APVS的外科治疗应选用带瓣补片重建右心室流出道。肺动脉严重扩张的患儿应行肺动脉成形术,以解除气道压迫症状。     

右心室微创切口矫治法洛四联症右心功能的中期随访研究

目的:观察右心室微创切口矫治法洛四联症3～5年的手术效果及右心功能情况。方法:入选2010年5月至2017年10月采用经右心室微创切口根治法洛四联症并按期复诊的患儿20例,年龄6.7～29.0个月,平均(14.3±7.1)个月;体质量6.5～14.0 kg,平均(9.3±1.9)kg。3例经右心房及右心室小切口进行矫治(右心室切口长度20 mm),其中2例跨肺动脉瓣环补片。另17例经右心房及肺动脉切口矫治,其中8例跨肺动脉瓣环补片(右心室切口10 mm),9例无跨环补片。结果:术后早期,经右房及肺动脉切口矫治,右室流出道残存压差平均26.3 mmHg,而经右心室小切口矫治压差明显减小(13 mmHg)。中晚期随访,右室流出道压差均明显下降(15.1 mmHg),右心室无明显扩张,三尖瓣及肺动脉瓣反流无加重。结论:右心室微创切口矫治法洛四联症3～5年随访临床效果满意。     

法洛四联症右室流出道疏通的临床经验

目的 探讨法洛四联症（Tetralogy of Fallot,TOF）根治术中右心室流出道疏通的最佳直径。方法 回顾性分析2011年1月至2016年12月我院心脏外科行TOF根治术的儿童病例308例,2011年1月至2013年12月201例患儿（A组）均采取右心室流出道疏通直径等同于标准肺动脉瓣环直径;2014年1月至2016年12月107例患儿（B组）采取右心室流出道疏通直径大于标准肺动脉瓣环直径3 mm。所有出院患者术后随访12~36个月。结果 A组有17例患者术后出现右心室流出道残余梗阻致低心排血量综合征（Low Cardiac Output Syndrome,LCOS）,多普勒测右心室流出道流速3.1~4.8（3.8±0.4）m/s,跨肺动脉瓣压差均大于50 mm Hg,其中6例右心室流出道流速超过4.5 m/s,再次手术行右心室流出道疏通术;术后早期死亡5例,随访余12例未发现右心室流出道残余梗阻进一步发展,且均较术后早期明显改善。B组术后无右心室流出道残余梗阻,跨肺动脉瓣压差均小于50 mm Hg,术后早期死亡2例。随访两组出院患者无三尖瓣、肺动脉瓣大量反流,生长发育良好,心功能均达Ⅰ~Ⅱ级。结论 TOF右心室流出道疏通直径等同于标准肺动脉瓣环直径造成术后残余梗阻发生率较高,大于标准肺动脉瓣环直径3 mm可有更好的疗效。     

双心室矫治完全型房室间隔缺损合并右心室双出口或法洛四联症

目的:总结双心室矫治完全型房室间隔缺损合并右心室双出口或法洛四联症(CAVSDDORV/TOF)的外科经验。方法:全部患者均按常规体外循环方法中低温(28℃~32℃)下手术。11例采用右心房-右心室径路,2例单纯右心房径路。应用逗号状补片修补VSD,其中双片法10例,单片法3例。5例采用单纯补片扩大右心室流出道(RVOT),4例行跨环补片,2例行右心室-肺动脉连接术(Rastelli),1例单纯扩大主肺动脉,1例仅行肺动脉瓣交界切开。结果:2例死亡,均为CAVSD-DORV,VSD远离大动脉,合并镜面右位心。11例存活患者出院前超声心动图检查示左、右心室流出道无狭窄,房室瓣成形满意。10例患者随访3~77个月,心功能均为NYHA I级或II级,无再次手术发生。结论:双心室矫治CAVSD-ROVR/TOF近期及中期效果满意,可作为首选手术策略。采用右心房-右心室径路,应用逗号状补片双片法修补室缺以及精细的房室瓣成形是提高手术成功率,减少并发症的关键。     

法洛四联症18例的外科治疗

目的 总结18例法洛四联症(tetralogy of Fallot,TOF)病人手术治疗的经验,探讨手术时机、危险因素和围术期处理.方法 对18例TOF进行心脏直视根治术,其中单纯右心室流出道漏斗部狭窄4例.漏斗部及肺动脉瓣狭窄9例,主肺动脉及左右肺动脉狭窄5例.全部采用自体心包片加宽右心室流出道及主肺动脉,合并的心脏畸形作相应的矫正.结果 全组手术死亡1例(1/18).死因为严重低心输出量综合征.术后并发症3例,均经治疗后恢复.结论 行TOF根治术病人年龄趋向减少,对横跨右心室流出道的畸形冠状动脉的手术保护处理方法,低心输出量综合征仍是目前导致术后并发症和死亡的主要原因.     

法洛四联症18例的外科治疗

目的 总结18例法洛四联症（tetralogy of Fallot，TOF）病人手术治疗的经验，探讨手术时机、危险因素和围术期处理。方法 对18例TOF进行心脏直视根治术，其中单纯右心室流出道漏斗部狭窄4例，漏斗部及肺动脉瓣狭窄9例，主肺动脉及左右肺动脉狭窄5例。全部采用自体心包片加宽右心室流出道及主肺动脉，合并的心脏畸形作相应的矫正。结果 全组手术死亡1例（1/18）。死因为严重低心输出量综合征。术后并发症3例，均经治疗后恢复。结论 行TOF根治术病人年龄趋向减少，对横跨右心室流出道的畸形冠状动脉的手术保护处理方法，低心输出量综合征仍是目前导致术后并发症和死亡的主要原因。     

聚四氟乙烯人工单叶肺动脉瓣在右心室流出道重建中的应用

目的 评价在右心室流出道重建手术中应用聚四氟乙烯（PTFE）制备单叶肺动脉瓣对防治术后早期肺动脉瓣反流的作用.方法 自2011年10月至2013年10月,随机选择21例具备手术适应证的患者,先用经修剪的PTFE片缝制人工单叶肺动脉瓣,再用经处理的自体心包片行右心室及主肺动脉补片扩大.体外循环转流50～150（83.4±24.1）min,主动脉阻断30～101（58.8±19.1）min.结果 本组21例中死亡1例,病死率4.76％,余20例术后恢复良好.全组病例机械辅助呼吸4～130 h,排除死亡的病例,平均（8.2±3.4）h;ICU滞留时间1～5（1.6±1.0）d;术后住院时间5～14（6.9±2.5）d.出院前及术后3个月行超声心动图检查,除第1例患儿存在中度肺动脉反流外,其余患儿均无反流或仅表现为痕迹量反流;全部患儿均无肺动脉或右心室流出道梗阻.结论 在行跨肺动脉瓣右心室流出道补片扩大术中应用PTFE人工单叶肺动脉瓣,可以有效地改善右心室功能,而体外循环时间、主动脉阻断时间及治疗费用并没有因此而明显增加.     

一期根治术治疗法洛四联症110例

目的总结法洛四联症(TOF)一期根治术的手术经验。方法 TOF患者110例,均施行一期根治术,采用右心房+右心室流出道(ROVT)切口,切除右心室肥厚的肌肉组织,使用自体心包补片重建右心室流出道至远端肺动脉;补片闭合室间隔缺损;带瓣跨瓣环补片重建ROVT 33例。结果 3例因术后出现低心排综合征而死亡,余107例随访1～48个月,均恢复良好,无晚期死亡者。结论加强术前评估、改进手术技术和体外循环管理、完善术后并发症防治措施可以提高TOF患者一期根治术的成功率。     

非常规右心室切口根治法洛四联症

目的:采用非常规右心室切口手术的方法根治法洛四联症(TOF)。方法:2005年5月至2008年2月采用经右心房/肺动脉途径外科根治TOF患者83例。年龄5个月~25岁,平均(22.33±6.41)个月;体质量6.5~51.5 kg,平均(13.18±4.48)kg。28例采用单纯右心房切口;51例右心房加肺动脉联合切口,其中42例跨肺动脉瓣环补片扩大右心室流出道(右心室切口长5~20 mm);另4例经右心房及右心室小切口进行右心室流出道疏通。室间隔缺损经右心房切口连续缝合补片修复81例,另2例经右心房/肺动脉切口2侧修复。结果:术中停止体外循环后,65例采用直接心脏表面穿刺或通过漂浮导管检测,收缩期右心室压/体循环压(RVP/SAP)比值为0.3~0.81,平均(0.41±0.15);收缩期右心流出道残存压差(GRVP-PAP)为8~55 mm Hg(1 mm Hg=0.133 kPa)。术后早期死亡1例。结论:采用右心室微创的手术方法根治法洛四联症在技术上是可行的,操作简单,早期临床效果满意,对中晚期结果可能有潜在的益处。     

姑息性右心室流出道重建术对肺动脉发育的影响

目的:肺动脉发育不良的室间隔缺损肺动脉闭锁患儿的治疗通常采取分期手术,姑息性手术右心室流出道重建术可以促进细小肺动脉的发育。方法:为了评价姑息性右心室流出道重建术对患儿及其肺动脉的影响,分析了2000年～2002年肺动脉闭锁合并室间隔缺损的患儿6例,均行姑息性右心室流出道重建术,间隔9～25个月后再次入院行根治术。术中均在家属知情下取小块肺组织活检,进行病理学的对比分析。结果:经过增加肺血的姑息右心室流出道重建术,患儿经皮血氧饱和度由平均0.71增加到0.83(P<0.05);血红蛋白浓度由平均150.83g/L降至134.83g/L(P>0.05);红细胞压积从47%降至41%(P>0.05);体表面积由平均0.45m2增加到0.57m2(P<0.05);肺动脉指数由平均102.86mm2/m2增加到156.68mm2/m2(P<0.05)。肺动脉指数与肺小动脉平均中膜厚度和面积百分比无显著的相关性。肺小动脉平均中膜厚度百分比在二期根治术前与正常对照中有显著性差异(P<0.05),肺小动脉平均中膜面积百分比在右心室流出道重建术前与正常对照中也有显著性差异(P<0.05)。肺小动脉平均中膜厚度百分比、肺小动脉平均中膜面积百分比二次根治术前与姑息性右心室流出道重建术前相比均有明显的提高(P<0.05)。结论:姑息性右心室流出道重建术对左右肺动脉及肺小动脉发育均有明显的促进作用,同时对患儿的发育也有明显改善。     

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.     

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.     

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.     

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。     

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     

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。