遗传性神经肿瘤综合征的影像学表现

遗传性神经肿瘤综合征是一组以伴发神经系统肿瘤为特征．累及多胚层、多器官、多系统且组织病理学类型复杂多样的家族遗传性疾病．主要包括神经纤维瘤病、结节性硬化复征、yon Hippel—Lindau病、Li—Franmeni综合征、Cowden病、Turcot综合征以及Gorlin综合征等。近年来，随着分子遗传学和医学影像学的进展对此类疾病的认识更加深入和全面，特别是CT和MRI诊断技术的出现对此类疾病所致中枢神经系统疾患的检出率大大提高。[第一段]     

再次肝移植治疗移植肝失功能22例报告

目的 总结再次肝移植治疗移植肝失功能的临床经验。方法 回顾分析2004年1月至2006年6月期间中山大学附属第三医院施行22例再次肝移植受者的临床资料，结合文献加以讨论。再次肝移植的原因分别为移植术后胆道并发症（12例）、移植术后肝癌复发（4例）、肝动脉栓塞（2例）、肝动脉狭窄（2例）以及乙肝复发（2例）。再次移植率为3．62％，供肝植入均采用改良背驮式肝移植技术。结果 全组无手术死亡，8例随访至今分别存活21、14、8、3个月各1例，12、1个月各2例；14例存活2周到28个月不等。首次肝移植术后8～30d行再次肝移植病人围手术期病死率最高，为66．7％；1年内死亡10例，主要死亡原因为感染（60％）。结论 再次肝移植是移植肝失功能的惟一有效的治疗方法，正确掌握手术时机及适应证，钻研手术技巧，合理的个体化免疫抑制方案以及围手术期有效的抗感染治疗是提高再次肝移植病人存活率的关键。     

Foot Polydactyly and Polysyndactyly: Genetic Implications in Two Families

The purpose of this study was to determine the genetic characteristics of foot polydactyly and identify its inheritance pattern by analyzing familial pedigree. Five cases from 2 Korean families were studied: 1 is a family whose members have been affected for 4 generations and the other for 2 generations. Using peripheral blood samples, we performed chromosomal analysis using the banding technique with Giemsa stain and karyotyping. We investigated the shape and structure of 46 chromosomes, looking for translation, deletion, inversion, ring chromosome, and isochromosome abnormalities. All peripheral blood samples demonstrated no chromosomal abnormalities, though the genetic nature of foot polydactyly and a new genetic locus was identified recently by other studies. Familial pedigree analysis suggested that polydactyly was inherited as an autosomal dominant trait in the first family. The mode of inheritance for the second family could not be determined due to an insufficient number of family members. The result of this study brought us to the conclusion that, while genetic factors play a major role in polydactyly, other factors may contribute to its occurrence.     

宜昌市20年结肠镜检查大肠癌患病率回顾性分析

目的探讨该市大肠癌流行病学和临床特点。方法以所有结肠镜受检对象为研究群体,对大肠癌患病率、大肠癌患者性别、年龄、肿瘤部位进行统计分析;比较大肠癌临床症状与大肠癌患者年龄、肿瘤发生部位的关系。结果自1980年以来,大肠癌年度患病率呈非线性缓慢上升,男性大肠癌患病率7.1%;女性大肠癌患病率5.5%;在3个年限段(80年代、90年代和21世纪5年)大肠癌中位患病年龄分别为男53.5、56.7和59.0岁;女51.9、53.7和55.2岁。986例大肠癌中直肠癌691例,乙状结肠癌82例,降结肠癌18例,脾曲癌4例,横结肠癌37例,肝曲癌30例,升结肠、回盲部癌79例,大肠多发性癌45例。临床症状以便血为主,其次为腹痛、腹部包块和肠梗阻发生率较低,半数以上患者有不同程度贫血。结论该市大肠癌患病率和中位发病年龄呈缓慢上升,低于国内大肠癌高发区。大肠癌肿瘤发生部位有近移和多发趋势。便血是直肠癌主要症状,中老年患者便血发生率高于青年患者,但腹痛发生率低于青年患者。     

Differentiation of pancreatic epithelial progenitor cells into hepatocytes following transplantation into rat liver

The ability to identify, isolate, and transplant progenitor cells from solid tissues would greatly facilitate the treatment of diseases currently requiring whole organ transplantation. In this study, cell fractions enriched in candidate epithelial progenitor cells from the rat pancreas were isolated and transplanted into the liver of an inbred strain of Fischer rats. Using a dipeptidyl dipeptidase IV genetic marker system to follow the fate of transplanted cells in conjunction with albumin gene expression, we provide conclusive evidence that, after transplantation to the liver, epithelial progenitor cells from the pancreas differentiate into hepatocytes, express liver-specific proteins, and become fully integrated into the liver parenchymal structure. These studies demonstrate the presence of multipotent progenitor cells in the adult pancreas and establish a role for the liver microenvironment in the terminal differentiation of epithelial cells of foregut origin. They further suggest that such progenitor cells might be useful in studies of organ repopulation following acute or chronic liver injury.     

Low sodium content formula for infants with congestive heart failure

Twenty-two infants of isolated ventricular septal defect with congestive heart failure were fed with lower-sodium content formula-Lonalac (Mead-Johnson) to study the clinical response of treatment for congestive heart failure. There were no significant changes of intake, urinary output, serum sodium, potassium and osmolality before, 2 days and 6 days after Lonalac feeding. The low sodium content formula may feed the infants with congestive heart failure in addition to the traditional anticongestive therapy.     

大鼠头颅瞬间侧向旋转后脑干水肿的形成及其超微形态学基础

目的：探讨头颅瞬间旋转后脑干水肿的形成及超微形态学基础。     

SODIUM EFFECTS ON THE FIBRILLATORYPROPENSITY OF NON-IONIC CONTRAST MEDIA

Removing sodium from standard ionic contrastmedia markedly increases the incidence of ventricu-lar fibrillation in patients undergoing coronary arvgiography. Newer nonionic contrast media, Iopa-midol (IOP), Iohexol (IOH) and Ioversol (IOV),contain only trace amounts of sodium. To determinewhether sodium influences the fibrillatory propen-sity of nonionic contrast media, we measured theprolongation in QT interval and performed program-med electrical stimulation with l,2 and 3 ventricularextra stimuli in 40 dogs during 4 ml intracoronaryinjections of IOP, IOH and IOV. Solutions of eachcontrast media with added NaCI at concentrations of0.225To, 0.45% and 0.970 were compared to stockcontrast media. The addition of NaCI markedlyincreased the amount of QT interval prolongationproduced by each contrast media. With IOP, theamount of QT interval prolongation was 40+11msec with standard IOP but was 58+11 msec with0.225'70 NaCI/IOP, 84+17 msec with 0.45'To NaCI/IOP, and 132+42 msec with 0.970 NaCl/IOP(P相似文献