Esophagogastric variceal bleeding in cirrhotic portal hypertension： onsensus on prevention and management （2008）

Portal hypertension is a clinical syndrome which is a consequence of a pathological increase in portal vein pressure due to various causes, liver cirrhosis being the most common. The basic pathophysiological characteristic of portal hypertension is resistance to portal vein flow or an increase in portal vein flow, which results in elevation of pressure in the portal vein and its tributaries and the formation of collateral circulation.

急性非静脉曲张性上消化道出血患者早期胃镜最佳时机的探讨

目的探讨急性非静脉曲张性上消化道出血(ANVUGIB)患者早期胃镜检查时间与临床疗效及经济效益的关系,寻找内镜的最佳时机。方法选择2016年7月至2019年9月于惠州市第一人民医院就诊的ANVUGIB患者271例,所有患者均在入院后的24 h内接受内镜检查。依据Glasgow-Blatchford(GBS)评分系统将患者分为中低危组(<12分)及高危组(≥12分);再根据入院后接受胃镜检查的不同时间段分为超早期组(入院后12 h内开始胃镜检查)、早期组(入院后12~24 h内行胃镜检查);分别比较不同时间段各危险亚组之间在输血量、住院时间、住院费用、出血后再干预率和死亡率。结果在高危患者中,超早期组的住院费用、输血量均少于早期组(P<0.05);而在住院时间、止血后再干预率、死亡率指标比较均没有统计学差异(P>0.05)。在中低危患者中,超早期组与早期组的住院费用、住院时间、输血量、死亡率、再干预率指标比较均无统计学意义(P>0.05)。结论对于高危的ANVUGIB患者,12 h内早期胃镜时机对于患者能带来更好的经济效益,但临床预后仍需进一步研究,而对于中低危患者超早期胃镜并未显示出更好的临床结局及经济效益。

排卵障碍性异常子宫出血诊治路径

排卵障碍型异常子宫出血(AUB-O)是临床常见的妇科内分泌疾病,多种近远期危害严重影响女性身心健康。本文以2018年中国AUB-O诊治指南为指导,就临床医生关注的病史采集、体格检查、辅助检查、急慢性AUB-O的治疗等常见问题进行重点阐述,形成基于证据的AUB-O标准化诊疗模式和治疗流程,从而更好地规范医疗行为,贯彻AUB-O指南指导临床实践,提高AUB-O患者的生活质量。

A gist of gastrointestinal stromal tumors: A review

Gastrointestinal stromal tumors (GISTs) have been recognized as a biologically distinctive tumor type, different from smooth muscle and neural tumors of the gastrointestinal tract (GIT). They constitute the majority of gastrointestinal mesenchymal tumors of the GIT and are known to be refractory to conventional chemotherapy or radiation. They are defined and diagnosed by the expression of a proto-oncogene protein detected by immunohistochemistry which serves as a crucial diagnostic and therapeutic target. The identification of these mutations has resulted in a better understanding of their oncogenic mechanisms. The remarkable antitumor effects of the molecular inhibitor imatinib have necessitated accurate diagnosis of GIST and their distinction from other gastrointestinal mes-enchymal tumors. Both traditional and minimally invasive surgery are used to remove these tumors with minimal morbidity and excellent perioperative outcomes. The revolutionary use of specific, molecularlytargeted therapies, such as imatinib mesylate, reduces the frequency of disease recurrence when used as an adjuvant following complete resection. Neoadjuvant treatment with these agents appears to stabilize disease in the majority of patients and may reduce the extent of surgical resection required for subsequent complete tumor removal. The important interplay between the molecular genetics of GIST and responses to targeted therapeutics serves as a model for the study of targeted therapies in other solid tumors. This review summarizes our current knowledge and recent advances regarding the histogenesis, pathology, molecular biology, the basis for the novel targeted cancer therapy and current evidence based management of these unique tumors.

Embolization of liver tumors:Past,present and future

Curative therapies for hepatocellular carcinoma(HCC),such as resection and liver transplantation,can only be applied in selected patients with early tumors.More advanced stages require local or systemic therapies.Resection of HCC offers the only hope for cure.Even in patients undergoing resection,recurrences are common.Chemoembolization,a technique combining intraarterial chemotherapy with selective tumor ischemia,has been shown by randomized controlled trials to be efficacious in the palliative setting.There is now renewed interest in transarterial embolization/transarterial chemoembolization(TACE) with regards to its use as a palliative tool in a combined modality approach,as a neoadjuvant therapy,in bridging therapy before transplantation,for symptomatic indications,and even as an alternative to resection.There have also been rapid advances in the agents being embolized trans-arterially(genes,biological response modifiers,etc.).The current review provides an evidence-based overview of the past,present and future trends of TACE in patients with HCC.

Incidental gall bladder cancers:Are they truly incidental?

AIM: To seek and analyze features suggestive of gallbladder cancer(GBC) on preoperative imaging and intraoperative findings in patients diagnosed as having incidental GBC(IGBC). METHODS: The study was conducted on 79 patients of IGBC managed in our department over a 10-year period(2003-2012). Review of preoperative imaging and operative notes was done to ascertain any suspicion of malignancy-in-retrospect.RESULTS: Of the 79 patients, Ultrasound abdomen showed diffuse thickening, not suspicious of malignancy in 5 patients, and diffuse suspicious thickening was seen in 4 patients. Focal thickening suspicious of malignancy was present in 24 patients. Preoperative computed tomography/magnetic resonance imaging was done in 9 patients for suspicion of malignancy. In 5 patients, dif-ficult Cholecystectomy was encountered due to dense/inflammatory adhesions. Intraoperative findings showed focal thickening of the gallbladder and a gallbladder mass in 9 and 17 patients respectively. On overall analysis, 37 patients had preoperative imaging or intraoperative findings suggestive of malignancy, which was either a missed GBC or an unsuspected/unexpected GBC. In 42(53.2%) patients, there was no evidence suggestive of malignancy and was an unanticipated diagnosis.CONCLUSION: Our study highlights a potential and not-so-rare pitfall of Laparoscopic Cholecystectomy. A greater awareness of this clinical entity along with a high index of suspicion and a low threshold for conversion to open procedure, especially in endemic areas may avert avoidable patient morbidity and mortality.

Implications of the presence of an aberrant right hepatic artery in patients undergoing pancreaticoduodenectomy

AIM:To analyze the differences in outcomes and the clinical impact following pancreatoduodenectomy(PD)in patients with and without aberrant right hepatic artery(aRHA).METHODS:All patients undergoing PD between January 2008 and December 2012 were divided into two groups,one with aRHA and the other without.These groups were compared to identify differences in the intraoperative variables,the oncological clearance and the postoperative morbidity,mortality and hospital stay.RESULTS:A total of 225 patients underwent PD,of which 43(19.1%)patients were found to have eitheraccessory or replaced right hepatic arteries(aRHA group).The aRHA was preserved in 79%of the patients.There was no significant difference in the intraoperative blood loss but operative time was prolonged,reflecting the complexity of the procedure[420±44(240-540)min vs 480±45(300-600)min,P<0.05)].There were no differences in the incidence of postoperative complications(pancreatic leak,pancreatic fistula,delayed gastric emptying and mortality)and hospital stay.Oncological clearance in the form of positive resection margins[13(7.1%)vs 3(6.9%)]and lymph node yield were also similar in the two groups.CONCLUSION:An aRHA is found in approximately one fifth of patients undergoing PD.Preservation is technically possible in most patients and can increase the operative complexity but does not negatively affect the safety or oncological outcomes of the procedure.

Neuroendocrine carcinoma of the ampulla of Vater:a clinicopathologic evaluation

BACKGROUND:Only 105 cases of neuroendocrine tumor(NET)of the ampulla of Vater have been described, mostly as single case reports.The incidence of NET is rising.The changes in incidence may result from changes in detection.This study was to determine the relative incidence and clinicopathological characteristics of high- grade neuroendocrine carcinoma(small cell carcinoma and large cell carcinoma)of the ampulla of Vater at a single institution. METHODS:Sections from paraffin blocks of tumors of the ampulla of Vater taken from 45 patients who underwent Whipple’s procedure and 6 patients who underwent palliative bypass between September 2003 and January 2007 were subjected to immunohistochemical analysis.The clinical and pathological data from 5 patients diagnosed with NET of the ampulla of Vater were analyzed. RESULTS:The patients were 3 men and 2 women,ranging in age from 39 to 47 years(mean 44 years).Operative procedures included Whipple’s procedure in 4 patients and palliative bypass in 1 patient.Histopathological examination revealed large-cell neuroendocrine carcinoma in 2 patients,small cell carcinoma in 2,and carcinoid in 1.Three patients with high-grade neuroendocrine carcinoma who had undergone Whipple’s procedure died at postoperatively 7,11,and 13 months.The patient who had undergone palliative triple bypass died 3 months after surgery.CONCLUSIONS:The relative incidence of high-grade neuroendocrine carcinomas of the ampulla of Vater is higher than that generally expected.The tumors behave aggressively and have a dismal prognosis despite aggressive treatment.

Long-term outcome of gastric access loop in hepaticojejunostomy

BACKGROUND:?The incidence of hepaticojejunostomy stricture is 4%-10% in experienced centres. Many access loops have been designed and used to facilitate endoscopic intervention for this complication of hepaticojejunostomy. In this study, we investigated the effectiveness and safety of gastric access loop. METHODS:A retrospective analysis was conducted on 13 patients who had undergone hepaticojejunostomy with gastric access loop between June 1999 and September 2003. Eleven patients were followed up for a mean period of 51 months (range 20-81 months). Two patients were lost to follow up. RESULTS:?On follow-up, 8 patients had patent jejuno- gastrostomy (end to side anastomosis between Roux loop of jejunum and stomach) and hepaticojejunostomy. Three patients developed stricture of jejunogastrostomy at 41 months, 63 months and 81 months of follow-up. Among these 3 patients, one also had hepaticojejunostomy stricture. In the patient with hepaticojejunostomy stricture, dilatation of jejunogastrostomy stricture was attempted but failed. None of the patients had any evidence of bile gastritis/cholangitis. There was no procedure related morbidity/mortality. CONCLUSIONS:Further studies involving large numbers of patients are required before wide application of gastric access loop in hepaticojejunostomy though it is a safer option than percutaneous transhepatic manipulations or revision surgery.

TET2 Mutations in Ph-Negative-Myeloproliferative Neoplasms: Identification of Three Novel Mutations and Relationship with Clinical and Laboratory Findings

High-throughput DNA sequence analysis was used to screen for TET2 mutations in peripheral blood derived DNA from 97 patients with BCR-ABL-negative-myeloproliferative neoplasms (MPNs). Overall six mutations in the coding region of the gene were identified in 7 patients with an overall mutational frequency of 7.2%. In polycythemia vera patients (n = 25) were identified 2 mutations (8%);in those with essential thrombocythemia (n = 55) 2 mutations (3.6%);in those with unclassifiable MPN (n = 8) 3 mutations (37.5%). No primary myelofibrosis patiens (n = 6) harboured TET2 mutations. Three unreported mutations were identified (p.P177fs, p.C1298del, p.P411del) the first two in patients with unclassifiable MPN, the last in a patient with essential thrombocythemia. On multivariate analysis the diagnosis of an unclassifiable MPN was significantly related to the presence of TET2 mutations (p = 0.02;OR: 2.81;95% CI 1.11 - 7.06). We conclude that TET2 mutations occur in both JAK2V617F-positive and -negative MPN and are more frequent in MPN-U patients. This could represent the biological link between the different classes of myeloid malignancies.

肝硬化病人肝静脉压力梯度与静脉曲张程度、出血、腹水及Child分级之间的关系

肝静脉压力梯度（HVPG）可以清楚地反映肝硬化性门脉高压的门脉压力。本文的目的是研究HVPG与肝硬化静脉曲张程度、Child分级和病因之间的关系。共176例病人参与该项研究。男：女比例=140：36，平均年龄（42．6±13．4）a；病毒性肝病104例、酒精性肝病40例、隐源性肝病26例、多因性肝病6例。本文以HVPG作为预测评估的指标，对肝硬化性门脉高压伴食管静脉曲张的病人进行了HVPG测量。

Large cell neuroendocrine carcinoma of the ampulla of Vater

BACKGROUND: Neuroendocrine tumors of the ampulla of Vater are extremely rare, and few cases of large cell neuroendocrine carcinoma (LCNEC) of the ampulla have been reported. METHODS: A 48-year-old male with obstructive jaundice was admitted to our hospital. On examination the patient was found to have a periampullary growth and subsequently underwent the Whipple's procedure. RESULTS: Histopathological examination and immunohistochemistry revealed features of LCNEC of the ampulla of Vater. The patient developed multiple liver metastases 6 months after Whipple's procedure. CONCLUSION: LCNEC of the ampulla of Vater is rare and highly aggressive, with a dismal prognosis.

青海省野生黑果枸杞种质资源调查

为明确青海省柴达木盆地黑果枸杞种质资源分布区域和生长情况,以更好地保护、开发和利用黑果枸杞资源,对青海省分布的野生黑果枸杞进行调查。结果表明:青海省的野生黑果枸杞自然分布在柴达木盆地腹部,基本以格尔木市为中心,半径200km的区域内,并且其浆果有2种形状,圆球型和蟠桃型。适宜野生黑果枸杞生长的土壤类型为固定或半固定风沙土、草甸盐土和偏碱性土壤,土壤含盐量高达301.6g/kg;伴生植物主要有芦苇、盐爪爪、柽柳和新疆枸杞;在表型和苗木的生长方面,诺木洪地区的黑果枸杞表现较好。

对新近诊断的免疫性血小板减少性紫癜患儿而言,单剂量75μg/kg抗-D免疫球蛋白疗法在快速提高血小板计数方面与静脉注射免疫球蛋白同样有效

Objective: To conduct a randomized prospective trial of immune globulin treatment for 105 Rh+ children with newly diagnosed immune thrombocytopenic purpura and a platelet count < 20,000/μ L, to determine whether anti- D immune globulin (anti- D) is as effective as intravenous immune globulin (IVIg). Study design: Eligible patients received either a single intravenous dose of 50 μ g/kg anti- D (anti- D50), 75 μ g/kg anti- D, (anti- D75), or 0.8 g/kg IVIg,(IVIg). Patients were monitored for response to treatment and adverse events. Results: By 24 hours after treatment 50% , 72% , and 77% of patients in the anti- D50, anti- D75, and IVIg groups, respectively, had achieved a platelet count > 20,000/μ L (P = 0.03). By day 7, hemoglobin concentrations decreased by 1.6 g/dL, 2 g/dL, and 0.3 g/dL in the anti- D50, anti- D75, and IVIg groups, respectively. Headache, fever, or chills occurred least often in the anti- D50 group. Conclusions: A single 75μ g/kg dose of Anti- D raised the platelet count in children with newly diagnosed immune thrombocytopenic purpura more rapidly than standard- dose anti- D and as effectively as IVIg, with an acceptable safety profile.

PRIMARY MALIGNANT MELANOMA OF THE VAGINA:CLINICAL, PATHOLOGICAL AND IMMUNOHISTOCHEMICAL STUDIES OF 9 CASES

Nine cases of vaginal primary malignant melanoma were diagnosed and treaed in Zhejiang Cancer Hospital from 1964 to 1990. The mean age of patients was 48. 6 years. Abnormal vaginal bleeding was the Incipient symptom in 6 patients. Five cases of malignant melanoma located in the lower one-third of the vagina. Five patients had the tumor in size larger than or equal to 4 cm In diameter. All of them were of the cauliflower-like, nodular configuration grossly. The surface of the mass demonstrated in a variable degree of black or grey- blackish in 7 patients. The htstological appearances were similar to those in the skin. Sections of 4 cases Indicated only few or no melanin under microscope. The covering epithelial changes of 3 vaginal primary malignant melanoma resembled that of malignant melanoma in situ. All sections of 9 cases showed positive reaction to S-100 protein and negative to CEA immunohistochemically. This article laid emphasis on the origin, diagnosis and treatment of this tumor, and make discussion in conjunction with the literature.

Use of video-assisted thoracoscope in 121 cases of cardiac surgery

Objective: To sum up 121 cases of heart disease operations with the help of thoracoscope from May to August in 2000. Among these cases, 48 cases were atrial septal defect (ASD); the average age of patients is 20±10 years old; average weight is (43±16) kg. 67 patients with ventricular septal defects (VSD), average age13±9 years old; one male patient, 44 years old with Ebstein malformation; one female patient (21 years old) partial atrioventricular canal combined with cor triatriatum; one male (21 years old) with ruptared aneuryem of aortic sinus. 3 cases(all females ) with mitral stenosis combined with mitral valve incompetence. One male patient(aged 16)with pericardiun effusion after trauma. Methods: During operations, a patient with supine position, and his/her right shoulder was padded 30°higher. Tracheal cannula was inserted and air was piped in with high frequency jet ventilation. Three mini thoracotomies with a diameter of 2 to 3 cm were made in the 4th intercostals space of the right par sternum and the 4th and 7th intercostals spaces of the right middle axillary line respectively. An periphera extra corporeal circulation was made. the aortic clamp was clamped, cannula for cold perfusion. After the heart was sliced and a reformative operation was performed. The process of the operation of defects repairs was finished under the thoracoscope. The other operations were performed with the help of thoracoscope. 3 rheumatic heart disease patients got 25# mechanical mitral valve prosthesis. A 30# tricuspid plasty ring was applied to The Ebstein malformation patient. Results: all 121 patients were successfully operated on without death. Two VSD cases appeared transient third degree atrialventricular block.Because 4 cases had more chest drainage, so they were stanched bleeding twice. After the operation, heart murmur vanished, and ultrasonic inspection showed no diffluence inside the heart. Conclusion: Our experience showed that all atrium, ventricular septal defects can be repaired under the thoracoscope. This scope-assisted technology is more accepted by patients because of tiny incisions and also provides an alternative solution for cardiac surgeons.

Pharmacokinetic Studies of Factor VIII in Chinese Boys with Severe Hemophilia A： A Single-Center Study

Background： Although much attention has been paid to the pharmacokinetics （PKs） of different factor VIII （FVIlI） concentrates in persons with hemophilia A （HA）, limited information is available in young boys with severe HA. In this study, we aimed to assess the PK parameters of FVIII products in boys with severe HA in China. Methods： A total of 36 boys （plasma-derived [pd]-FVIII, n= 15; recombinant [r] FVIII, n = 21） were enrolled between January 2015 and May 2016 in Beijing Children＇s Hospital. PK characteristics of FVIII products were studied according to a reduced 4-sampling time point design （1 h, 9 h, 24 h, and 48 h postinfusion）. Results： The mean FVIII half-life （t1/2） was 10.99 ± 3.45 h （range 5.52-20.02 h）, the mean in vivo recovery （IVR） was 2.01 ± 0.42 IU/dl per IU/kg （range 1.24-3.02 IU/dl per IU/kg） and mean clearance （CL） of FVIII is 4.34 ± 1.58 ml·kg^-1·h ^-1 （range 2.29-7.90 ml·kg^-1·h·^-1）. We also analyzed the influence of several parameters that potentially modulate FVIII PK. The age was closely associated with FVIII half-life （R^2 = 0.32, P 〈 0.01 ）. The t1/2 of FVIII increased by 0.59 h per year. Besides age, yon Willebrand factor antigen （VWF：Ag） also was associated with FVIII half-life （R^2 = 0.52, P 〈 0.01）. Patients with blood Group O had a shorter FVIII halt-life than patients with non-O blood group （9.40 ± 0.68 h vs. 12.3 ± 0.79 h, t = 2.70, P = 0.01）. The FVIII IVR correlated with age （R^2 =0.21, P 〈 0.01） and VWF：Ag level （R^2 = 0.28, P 〈 0.01 ）. CL rates were taster in young patients and in those with low-VWF：Ag levels. CL rates of FVIII are higher in blood Group O versus non-blood Group O persons （5.02 ± 0.38 vs. 4.00 ± 0.32 ml·kg^-1·h^-1 , t = 2.53, P = 0.02）. Conclusions： Chinese boys with severe HA have similar PK values to other ethnic groups and large differences in FVIII PK between individual patients. Age, blood group, and VWF：Ag levels are important determining factors for FVlll CL.

ORIGINAL ARTICLES CALCIUM CHANNEL BLOCKERS IN CIRRHOTIC PATIENTS WITH PORTAL HYPERTENSION

Four calcium channel blockers, i.e. nifedipine, verapamil, cinnarizine and tetrandrine are currently available and used widely in treating cardiovascular diseases. To confirm the effects, if any, of calcium channel blockers on cirrhotic patients with portal hypertension, a study was performed on esophageal variceal pressure and rebleeding rate of esophageal varices after 2 years by using calcium channel blocker in 321 patients from some 23 hospitals. The results demonstrated that the calcium channel blockers could significantly reduce the esophageal variceal pressure and the portal blood flow in cirrhotic patients with portal hypertension. The proportion of patients with no recurrent gastrointestinal bleeding after 2 years medication of tetrandrine was 87.9% in tetrandrine group, significantly higher than those in the other 4 groups (P<0.05). It is suggested that tetrandrine should be more effective for cirrhotic patients with portal hypertension in preventing recurrent variceal bleeding.

新一代斯堪的纳维亚设计

北欧的设计独具一格,不像常见的西方设计那样颜色鲜艳、排列整齐、质感强烈,而是无拘无束,贴近自然,干净简约,脱俗但不乏生活气息,让人看了心神宁静。瑞典、芬兰、挪威、丹麦位于斯堪的纳维亚半岛,因此北欧地设计也称为斯堪的纳维亚(Scandinavia)设计。实际上,斯堪的纳维亚风格形成于20世纪40年代,也不过几十年的功夫,就引领了全球的家居设计风潮。受德国现代主义的影响,包豪斯实验风格在北欧开始传播和流行,再加上瑞典.

TRANSFER AND EXPRESSION OF HUMAN CLOTTING FACTOR IX GENE IN CHINESE HAMSTER OVARY CELLS

Clotting factor Ⅸ is an essential member in the intrinsic dotting pathway and its defect may cause an inherited X-chromosome linked bleeding disorder——hemophilia B, or Christmas disease. Factor Ⅸ is synthesized in liver hepatocytes where it undergoes complex vitamin K-dependent post-translational modifications which are essential for the activation of factor Ⅸ. Recently, several groups have cloned factor Ⅸ cDNA and reported that, in the presence of vitamin K, active factor Ⅸ could be secreted in human and mouse fibroblasts,