Islet Xenotransplantation: Are We Really Ready for Clinical Trials?

Four clinical trials of porcine islet transplantation have been reported, and there are verbal reports that clinical trials on much larger scales are continuing in centers in China and Russia. The four reported trials are briefly reviewed and, in the light of the present status of experimental islet xenotransplantation, consideration is given to whether such trials are currently justified. The Ethics Committee of the International Xenotransplantation Association has (1) emphasized the need for encouraging studies in non-human primates before clinical trials should be undertaken, (2) mandatory monitoring for the transfer of porcine microorganisms, and (3) careful regulation and oversight by recognized bodies. Other aspects of the topic, such as the need for informed consent, are briefly discussed. We conclude that, at the present time, more data documenting convincing efficacy, focused on clinically applicable immunosuppressive regimens, are needed to justify the initiation of closely monitored clinical trials. A clinical trial may then be justified even though the potential risk to the patients, and possibly for society, will not be zero.     

Coccidioidomycosis After Renal Transplantation in an Endemic Area

Coccidioidomycosis is an endemic fungal infection of the desert southwestern United States of particular concern for immunosuppressed renal transplant recipients. The clinical course of coccidioidomycosis can be severe in immunosuppressed patients, with high rates of dissemination and mortality, and antifungal prophylaxis is routinely administered to high-risk patients. We sought to determine the impact of coccidioidomycosis on patients who received their renal transplant at our hospital in Phoenix, Arizona. A retrospective records review of the first 205 patients who received a renal transplant between June 1999 and December 2003 identified 6 patients (3%) who had contracted coccidioidomycosis after transplantation. All six cases occurred more than 6 months after transplantation. Two of these six patients had disseminated coccidioidomycosis. Two patients, one with pulmonary infection and one with disseminated infection, died. None of the six patients with coccidioidomycosis after transplantation had identified risk factors before transplantation. No high-risk patient who received targeted antifungal prophylaxis had a reactivation of coccidioidomycosis after transplantation. Treatment for acute rejection and induction with antithymocyte globulin did not appear to increase the risk of subsequent coccidioidomycosis.     

Spinocerebellar ataxia types 2 and 3 segregating simultaneously in a single family.

Spinocerebellar ataxia (SCA) types 2 and 3 are autosomal-dominant neurodegenerative disorders caused by mutations in two different genes. We identified mutations for SCA2 and SCA3 segregating simultaneously in a single Brazilian family. The index patient had SCA2, whereas her two second-degree cousins had SCA3. Disease was more rapidly progressive in the SCA2 patient, who presented severe brainstem and pancerebellar atrophy, as opposed to the two SCA3 patients, who had only mild cerebellar vermian atrophy. In such situations, molecular confirmation of all patients may avoid misdiagnosis of SCA subtypes and eventual errors in predictive testing of unaffected family members.     

中原地区遗传性非息肉病性大肠癌表型特征的临床研究

目的探讨中原地区遗传性非息肉病性大肠癌(HNPCC)的表型特征,为制订适合我国国情的HNPCC遴选标准提供依据。方法对符合Am sterdam标准的8个家系37例患者的临床资料(A组),与随机抽调我院40例确切无家族史的大肠癌患者的临床资料(B组)进行对比分析。结果①发病年龄:A组平均46岁,B组62.6岁,平均提前16.6年;<50岁者:A组73%(27/37),B组30%(12/40),两组比较P<0.01;第1、2、3代平均发病年龄分别为69.2、46.9、34.6岁,逐代比较差异有统计学意义(P<0.05或P<0.01)。②发病部位:右半结肠A组73.2%(30/41),B组39.0%(16/41),两组比较P<0.05。③同时、异时多发大肠癌:A组8.1%(3/37),B组2.5%(1/40)。④大肠外癌:A组17.1%(7/41),B组0,两组比较P<0.05。⑤合并腺瘤:A组0,B组22.5%(9/40),两组比较P<0.05。⑥组织学类型:低分化腺癌A组69.7%(23/33),B组40%(16/40),两组比较P<0.05。结论中原地区HNPCC有其独特的临床特征,国际遴选标准不能完全适用于中国人,制定适合我国国情的HNPCC遴选标准确有必要。     

学习困难儿童智力及记忆力特征分析

目的探讨学习困难儿童的智力、记忆力特征。方法学习困难(LD)根据ICD-10五条诊断标准,精神发育迟滞(MR)根据国际疾病分类第10版(ICD-10)诊断标准,分为实验组LD组;对照组a.正常组,b.弱智组。采用中国韦氏儿童智力量表进行智力测验,记忆力测验采用中国科学研究院编制的临床记忆量表。结果LD组与正常对照组在语言智商、操作智商、总智商[(86.23±10.65)分,(87.44±12.59)分,(84.09±13.96)分vs(102.27±10.21)分,(103.22±11.65)分,(103.28±9.88)分]及分测验值差异均有显著性(P<0.01);LD组儿童VIQ与PIQ相距一个标准差以上的比率明显高于对照组(2=9.29,P<0.01),也较MR对照组多(2=4.29,P<0.05)。LD组记忆商(MQ)明显低于正常对照组(P<0.01)。结论LD儿童智商、记忆商水平低于正常儿童,且存在明显智力结构发展不平衡。     

Pathological structure of the kidney from adult mice with mosaic mutation

Summary: The mosaic (Atp7a ^mo-ms ) is an X-linked, lethal mutation in mice. In mosaic mutant males, many clinical features characteristic of defective copper metabolism have been observed and they die at the age of 15 days, exhibiting strongsimilarities to the brindled and macular mutants. About 4% of the mutant males live to sexual maturity and some of them are fertile. In this paper, alterationsin the structure of the kidney from adult mutants are described. Owing to an inherited defect of efflux, copper is accumulated in the kidney of the mutants up to a toxic level and this leads to severe damage of the renal cortex. Pathological changes in the kidney mostly affected the structure of the renal corpuscle and renaltubules.     

过量氟化物导致大鼠腰椎黄韧带退变与骨化

[目的]研究氟化物在黄韧带骨化中可能的作用机理。[方法]36只雄性SD大鼠，实验组饮用含NaF（质量分数为1014）蒸馏水，分别于3个月及6个月时，检测骨密度，血清、骨组织标本中Ca、P^3＋、Mg、Zn、Cu、Fe、F^-含量和血清碱性磷酸酶（ALP）活性；行大鼠腰段标本X线检查后组织病理观察。[结果]3个月实验组10只大鼠中6只出现氟斑牙：6个月实验组均出现氟斑牙，且大鼠中轴骨骨密度显著增加（P〈0．05）。X线检查，6个月实验组中有4只可见黄韧带嵴状骨化影。病理观察，3个月实验组大鼠黄韧带呈退行性改变；6个月实验组部分黄韧带骨化，骨化类型以膜内骨化为主。[结论]过量氟化物可造成SD大鼠腰椎黄韧带的退变、骨化，在黄韧带的骨化中可能起重要作用。