gamma-sarcoglycan deficiency muscular dystrophy in two adults.

All dystrophin-associated proteins contain sarcoglycan complex. Different forms of muscular dystrophy are caused by defective expression of different proteins of this structure. gamma-Sarcoglycan deficiency muscular dystrophy, so-called severe childhood autosomal recessive muscular dystrophy (SCARMD), is a rare disease that has not been previously reported in Taiwan. This paper describes two Taiwanese adults with this disease: a 26-year-old man with calf pseudohypertrophy who had weakness in both legs for 1 year; and a 43-year-old woman who had progressive weakness in all four limbs, with the initial symptom of gait disturbance at the age of 32 years. Analysis of muscle biopsy specimens, which showed total deficiency of gamma-sarcoglycan protein on immunostaining, confirmed the diagnosis of SCARMD in both cases. However, the clinical manifestations in these two patients, including lower proximal limb weakness initially developing in adulthood with a slow progressive course, are different from previously reported cases of SCARMD. The literature on this disease is reviewed and possible mechanisms of these distinct clinical presentations are discussed.     

铅镉联合暴露对大鼠肾脏功能损伤的研究

目的 探讨铅、镉对大鼠肾脏功能损伤的联合毒性作用.方法 对大鼠进行铅(300 mgPb/L)、镉(50 mgCd/L)单独和联合(300 mgPb/L 50 mgCA/L)饮水染毒8周.检测尿液中碱性磷酸酶(UALP)、N-乙酰-β-D-氨基葡萄糖苷酶(UNAG)、γ-谷氨酰基转移酶(UGGT)活力和总蛋白(utp)、α-微球蛋白(Ua<,1>-MG)、微量白蛋白(UmAlb)含量的动态变化.结果 单独染毒除2周时Pb、Cd组Ua<,1>-MG含量与Pb组UALP活力外,其余所测指标均从染毒2周开始明显高于对照组(P＜0.05;P＜0.01),且升高幅度与染毒时间呈正相关;联合染毒组从2周开始,所测指标均明显高于对照组(P＜0.01).在整个试验过程中,联合染毒组尿酶活力和尿蛋白含量均高于单独染毒组.结论 Pb、Cd联合暴露对大鼠肾脏功能的损伤呈协同毒性效应.     

Structure-activity relationship of 6-methylidene penems bearing 6,5 bicyclic heterocycles as broad-spectrum beta-lactamase inhibitors: evidence for 1,4-thiazepine intermediates with C7 R stereochemistry by computational methods

The design and synthesis of a series of 6-methylidene penems containing [6,5]-fused bicycles (thiophene, imidazole, or pyrazle-fused system) as novel class A, B, and C beta-lactamase inhibitors is described. These penems proved to be potent inhibitors of the TEM-1 (class A) and AmpC (class C) beta-lactamases and less so against the class B metallo-beta-lactamase CcrA. Their in vitro and in vivo activities in combination with piperacillin are discussed. On the basis of the crystallographic structures of a serine-bound reaction intermediate of 2 with SHV-1 (class A) and GC1 (class C) enzymes, compounds 14a-l were designed and synthesized. Penems are proposed to form a seven-membered 1,4 thiazepine ring in both class A and C beta-lactamases. The interaction energy calculation for the enzyme-bound intermediates favor the formation of the C7 R enantiomer over the S enantiomer of the 1,4-thiazepine in both beta-lactamases, which is consistent with those obtained from the crystal structure of 2 with SHV-1 and GC1.     

Activities of antioxidant enzymes in three bacteria exposed to bensulfuron-methyl

Oxidative stress enzymes, superoxide dismutase (SOD), catalase (CAT), and ATPase, from two Gram-positive bacteria and one Gram-negative bacterium, respectively, were tested for response to the oxidative stress caused by bensulfuron-methyl (BSM). Native PAGE was used to detect the SOD isoenzyme profiles of these bacteria. All three bacteria possessed a basal level of SOD, CAT, and ATPase activity prior to being exposed to BSM. Enzyme activities changed in a BSM-concentration-dependent manner after exposure to BSM for 24 h. Activity of all the enzymes was increased and reached the first activity peak after being exposed to BSM at 1 or 1.5 h, respectively, then a decline occurred, and after that another simulation appeared at 9 h or 14 h. Only one and three detectable SOD isoenzyme bands were observed in Gram-positive strains and the Gram-negative strain, respectively. BSM could bring short-term ecotoxicity to three bacteria, but the effect of BSM was not lethal.     

人胃癌组织WAF1基因的缺失与不表达

探讨ＷＡＦ１与胃癌的关系，为胃癌基因治疗提供有价值的依据。方法３２例胃癌和３０例正常胃粘膜标本提取细胞ＤＮＡ、总ＲＮＡ，ＭＴＳ１ｃＤＮＡ探针采用随机引物法进行３２Ｐ－ｄＡＴＰ掺入标记，ＷＡＦ１与所提ＤＮＡ、ＲＮＡ进行Ｓｏｕｔｈｅｒｎ和Ｎｏｒｔｈｅｒｎ杂交分析，检测标本中有无ＷＡＦ１的缺失和ｍＲＮＡ的不表达。ｐ５３抗体与提取的蛋白质进行Ｗｅｓｔｅｒｎ印迹分析，检测标本中有无ｐ５３的表达。结果ＷＡＦ１Ｓｏｕｔｈｅｒｎ和Ｎｏｒｔｈｅｒｎ杂交分析结果显示，３２例胃癌标本ＷＡＦ１基因缺失率及ｍＲＮＡ不表达率分别为３４．４％和４０．６％，明显高于正常胃粘膜组。ＷＡＦ１基因缺失率及ｍＲＮＡ不表达与胃癌浸润的深度及分化程度有关，胃癌侵及肌层组ＷＡＦ１基因缺失及ｍＲＮＡ不表达率分别为５２．６％和５７．９％，胃癌未侵及肌层组分别为７．７％和１５．４％，高、中分化胃癌组ＷＡＦ１基因缺失及ｍＲＮＡ不表达率分别为７．１％和１４．３％，而低分化及印戒细胞胃癌组分别为５５．８％和６１．１％。结论胃癌的发生与ｐ５３失活及ＷＡＦ１ｍＲＮＡ不表达有关，胃癌组织中ｐ５３失活与ＷＡＦ１ｍＲＮＡ不表达关系密切。     

Surgical management for thoracic spinal tuberculosis in the elderly: posterior only versus combined posterior and anterior approaches

Purpose

To compare the clinical outcomes of surgical management by posterior only and combined posterior and anterior approaches for thoracic spinal tuberculosis in the elderly.

Materials and methods

This was a retrospective cohort study. Thirty-six cases of thoracic spinal tuberculosis treated by two different surgical procedures in our center from January 2004 to June 2009 were studied. All the cases were divided into two groups: 20 cases in Group A underwent single-stage posterior debridement, transforaminal fusion and instrumentation, and 16 cases in Group B underwent posterior instrumentation, anterior debridement and bone graft in a single- or two-stage procedure. The operation time, blood loss, correction rate, recovery of neurological function, fusion time and complications were, respectively, compared between Group A and Group B.

Results

All patients were followed up for an average of 35.1?±?5.8?months (range 26?C45?months). It was obviously that the average operative duration, blood loss, hospitalization and complication rate of Group A was less than those of Group B. Spinal tuberculosis was completely cured and the grafted bones were fused in 10?months in all patients. There was no persistence or recurrence of infection and no differences in the radiological results in both groups. The kyphosis was significantly corrected after surgical management. However, loss of correction also occurred in both groups.

Conclusion

Our study showed that the posterior approach only procedure obtained better clinical outcomes than combined posterior and anterior surgeries. It might be a better surgical treatment for thoracic spinal tuberculosis in aged patients with poor health status, especially for cases in early phase of bone destruction and/or mild and moderate kyphosis.     

Differentiation of prostate cancer and benign prostatic hyperplasia: the clinical value of<Superscript>201</Superscript>Tl SPECT—A pilot study

PURPOSE: Thallium-201 (201Tl) is a recognized tumor-imaging agent; however, the usefulness of 201Tl in prostate cancer has not been studied. The purpose of this preliminary study was to evaluate the efficacy of 201Tl single-photon emission computed tomography (SPECT) imaging for differentiating prostate cancer from benign prostatic hyperplasia (BPH). METHODS: 201Tl pelvic SPECT was performed in 10 patients (aged 64-78 years) with biopsy-proven BPH before transurethral resection of the prostate and 15 patients (aged 65-81 years) with biopsy-proven prostate cancer prior to any therapeutic modality or invasive surgical procedures for treatment of their prostate cancer. RESULTS: From the 15 patients with prostate cancer, 201Tl pelvic SPECT detected prostate cancer in 13 (86.7%) but not in 2 (13.3%) patients with Gleason scores of 5 (2 + 3). In contrast, all 10 patients with BPH (100.0%) had negative results of 201Tl pelvic SPECT. CONCLUSION: Our study showed that 201Tl pelvic SPECT scan is very helpful in distinguishing between prostate cancer and BPH.     

亲体部分肝移植治疗肝豆状核变性

目的通过对儿童期肝豆状核变性(WD)1例的亲体部分肝移植术(LRLT)后的肝脏病理学研究,探讨WD患儿LRLT的手术适应证。方法采集WD患儿LRLT后的受体和供体的肝脏标本,进行HE、MASSON、Timm′s、Rubeanic染色。结果WD患儿肝脏HE、MASSON染色表现为不同程度的退行性变,胶原纤维增生明显及典型假小叶形成;Timm′s、Rubeanic染色发现不均匀分布黑色颗粒或团块样物质沉积,处于典型肝硬化阶段,与临床Child Pugh分级存在差异。结论肝脏病理对WD的手术时机选择有重要价值,在肝脏病理支持的基础上,手术指征可适当放宽。     

Neurotropic viruses and Alzheimer''s disease: a search for varicella zoster virus DNA by the polymerase chain reaction.

BACKGROUND: In studies on the possible role of viruses in the aetiopathogenesis of Alzheimer's disease, herpes simplex virus type 1 (HSV1) DNA was detected by the polymerase chain reaction (PCR) in a high proportion of normal elderly people and of patients with Alzheimer's disease. The combination of HSV1 and a host factor, the type 4 allele of the gene for apolipoprotein E, is a strong risk factor for the disease. METHODS: Brain specimens were examined for another herpes virus, varicella zoster (VZV), which, like HSV1, is neurotropic, has a predilection for residing latently in the peripheral nervous system, and can reactivate. RESULTS: Using primers for sequences in the VZV origin of replication gene or thymidine kinase gene, VZV DNA was not found in any of 24 samples (18 HSV1 positive), from 17 patients with Alzheimer's disease, nor in 20 samples (12 HSV1 positive from 12 aged normal people. Hybridisation of the PCR products with a radiolabelled oligonucleotide probe capable of detecting less than 10 copies of the target sequence, confirmed the absence of VZV DNA. CONCLUSION: The presence of one neurotropic virus--HSV1--and the absence of another--VZV--in aged human brains is consistent with a role for HSV1 in the aetiology of Alzheimer's disease.     

Identification and classification in le fort type fractures by using 2D and 3D computed tomography

In1901,basedontheexperimentationwithbrittlewandstrokingcadaverskulls,LeFortfirstlyclassifiesmaxillofacialfracturesintothreetypes.Atpresent,theclassificationisstillusedtodistinguish clinicalmaxillofacialfracturesintheworld.1,2The diagnosisofLeForttypefracturereliesonthephysical andradiologicalexamination,butthephysicalfindings maynotalwaysbepresent.35Conventionalradiographs areroutinelyusedforLeForttypefracture,however,theoverlapofstructuremayimpairproper interpretationofimages.35Routinecomp…