Alpha‐1‐antitrypsin in cell and organ transplantation

Limited availability of donor organs and risk of ischemia‐reperfusion injury (IRI) seriously restrict organ transplantation. Therapeutics that can prevent or reduce IRI could potentially increase the number of transplants by increasing use of borderline organs and decreasing discards. Alpha‐1 antitrypsin (AAT) is an acute phase reactant and serine protease inhibitor that limits inflammatory tissue damage. Purified plasma–derived AAT has been well tolerated in more than 30 years of use to prevent emphysema in AAT‐deficient individuals. Accumulating evidence suggests that AAT has additional anti‐inflammatory and tissue‐protective effects including improving mitochondrial membrane stability, inhibiting apoptosis, inhibiting nuclear factor kappa B activation, modulating pro‐ vs anti‐inflammatory cytokine balance, and promoting immunologic tolerance. Cell culture and animal studies have shown that AAT limits tissue injury and promotes cell and tissue survival. AAT can promote tolerance in animal models by downregulating early inflammation and favoring induction and stabilization of regulatory T cells. The diverse intracellular and immune‐modulatory effects of AAT and its well‐established tolerability in patients suggest that it might be useful in transplantation. Clinical trials, planned and/or in progress, should help determine whether the promise of the animal and cellular studies will be fulfilled by improving outcomes in human organ transplantation.     

Hepatitis C virus genotype 6:Virology,epidemiology,genetic variation and clinical implication

Hepatitis C virus(HCV)is a serious public health problem affecting 170 million carriers worldwide.It is a leading cause of chronic hepatitis,cirrhosis,and liver cancer and is the primary cause for liver transplantation worldwide.HCV genotype 6(HCV-6)is restricted to South China,South-East Asia,and it is also occasionally found in migrant patients from endemic countries.HCV-6 has considerable genetic diversity with23 subtypes(a to w).Although direct sequencing followed by phylogenetic analysis is the gold standard for HCV-6 genotyping and subtyping,there are also now rapid genotyping tests available such as the reverse hybridization line probe assay(INNO-LiPAⅡ;Innogenetics,Zwijnaarde,Belgium).HCV-6 patients present with similar clinical manifestations as patients infected with other genotypes.Based on current evidence,the optimal treatment duration of HCV-6 with pegylated interferon/ribavirin should be 48 wk,although a shortened treatment duration of 24 wk could be sufficient in patients with low pretreatment viral load who achieve rapid virological response.In addition,the development of direct-acting antiviral agents is ongoing,and they give high response rate when combined with standard therapy.Herein,we review the epidemiology,classification,diagnosis and treatment as it pertain to HCV-6.     

《临证指南医案》痹证用药特点分析

目的探讨《临证指南医案》痹证用药特点。方法 运用数据挖掘技术统计药物使用频次、性味归经并进行关联规则、聚类、复杂网络分析等。结果 筛选出83首处方,共涉及中药125味，使用频次前5位依次为桂枝、茯苓、白术、当归、苦杏仁，功效类别以补虚药为主，性味以甘温最多，归经主要涉及肺、脾、肝、心、肾、胃；关联规则得到10对常见组合，高频中药聚为4类，复杂网络分析得到1个核心组合。结论 叶氏常从络病角度治痹，或通、或补、或通补皆施，重视肺脾，注重调和气血营卫，擅长通补奇经治法。     

Intraductal carcinoma of the prostate: What we know and what we do not know

Intraductal carcinoma of the prostate (IDC-P) is a malignant, clonal proliferation of cells growing within the basement membrane-bound structures of the prostate. IDC-P is usually associated with unfavorable clinicopathologic parameters such as large tumor volume, high-grade Gleason score, extra prostatic extension and seminal vesicle invasion. Majority of laboratory and patient data suggest that IDC-P represents intraductal spread of invasive carcinoma, rather than a precursor lesion. Additionally, relationship of IDC-P and adjacent invasive carcinoma has been investigated in a series of molecular studies. The differential diagnosis of IDC-P from other lesions is critical for patient management. In this article, we summarize current literatures regarding what we know about IDC-P, including its pathological morphology, incidence, differential diagnosis, molecular features and clinical significance. In addition, we propose several issues that we currently do not know about IDC-P. Further research is needed to better understand the biological nature of IDC-P.     

Evaluating Patient Education Resources of Popular Clinical Databases

Many library-subscribed clinical databases contain educational materials for physicians and librarians to share with patients. These materials are typically written in layperson language meant to be understood by the average, nonclinician person. However, the materials found in these online databases vary widely, and some are better than others. This article uses the Patient Education Materials Assessment Tool (PEMAT) to evaluate the patient education content of several popular clinical databases, including Access Medicine, Clinical Key, DynaMed Plus, Epocrates, and UpToDate. These databases are also evaluated on other, more basic criteria, including findability, content, printing to PDF, customizability, and available languages.     

1型葡萄糖转运体缺陷综合征一例报告及文献复习

目的总结1型葡萄糖转运体缺陷综合征的临床特点、实验室检查、分子遗传学诊断及生酮饮食治疗疗效。方法报告1例以痫样发作为首发的1型葡萄糖转运体缺陷综合征病例。检索国内外相关文献,共检索出明确诊断的1型葡萄糖转运体缺陷综合征32例,进行文献复习。结果包括作者报告1例患者,共33例,男19例,女14例。临床特点:27/33例有痫样发作,发作年龄2个月至35岁之间。大多发作年龄在6个月以内。25/33例有共济失调,大多数轻中度共济失调,少部分患者共济失调影响行走及日常生活。24/33例肌张力障碍。14/33例有小头畸形。实验室检查:30/33例患者进行了脑脊液糖检查,23-56mg/dl之间,平均34.2±4.7mg/dl。脑脊液糖/血糖0.24-0.57之间,平均0.38±0.07。21/33例患者进行了红细胞摄取3-O-甲基-D-葡萄糖的能力检查,结果显示红细胞摄取3-O-甲基-D-葡萄糖的能力较正常对照下降约50%左右。29/33例进行了脑电图检查,发现痫样放电25例,表现为多灶性棘波、棘慢波。32/33例患者进行了GLUT1-DS基因SLC2A1筛查,发现12例错义突变,2例无义突变,2例插入突变,16例缺失或剪切位点突变。治疗:所有癫痫患者均进行了抗癫痫药物治疗,痫样发作均难以控制。28/33例患者(25例癫痫患者和3例发作性运动障碍)进行了生酮饮食治疗。24例癫痫患者的痫样发作完全控制,1例痫样发作明显缓解。3例发作性运动障碍明显改善。结论 1型葡萄糖转运体缺陷综合征临床以难治性痫样发作、语言智能发育落后、脑脊液糖/血糖明显降低为特点,常规抗癫痫药物难以控制痫样发作,生酮饮食能够控制痫样发作,并对语言、认知、运动障碍均有改善作用。