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2009 Year 24 Volume 4 Issue
- Abstract：Objective To investigate the clinical and genetic features of a Chinese family with von Hippel-Lindau（VHL） disease revealed by bilateral pheochromocytoma.Methods The proband and other members in a Chinese family with familial pheochromocytoma were clinically evaluated and followed up.Genomic DNA extracted from the peripheral blood of 8 family members（including 3 patients） was amplified by polymerase chain reaction（PCR） and the PCR products were directly sequenced.Results The first presentation in the proband,his mother,and his sister was bilateral pheochromocytoma,and the missense mutation of 695G-A（Arg161Gln） in exon 3 of VHL gene was detected in the three patients.In the follow-up study,the proband and his mother were found to have other VHL tumors,induding retinal and cerebellar hemangioblastomas and pancreatic tumor.Neither clinical presentation of VHL disease nor gene mutation was found in other family members.Conclusion VHL disease should be suspected in some patients with familial pheochromocytoma,and VHL gene screening helps to achieve early diagnosis of the disease.4|34|3Updated：2024-04-10
- Abstract：Objective To assess the spectrum of causes,clinical features,differences between disease phases,and prognosis of extrinsic allergic alveolitis（EAA）.Methods Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively.Their medical records were examined to gather clinical,laboratorial,radiological,and histopathological data.Patients were divided to three phases（acute,subacute,and chronic） according to clinical presentations.Follow-up data regarding treatment response,subsequent radiological and pulmonary function studies,and clinical outcomes were collected.Results A total of 21 cases were enrolled.Among them,11 were subacute,10 were chronic.The most common exposure was pet birds（6 cases,28.6%）.The primary abnormality of pulmonary function was restriction and/or reduction in diffusing capacity（12 cases,63.2%）.The most common findings on high-resolution computed tomography（HRCT） were ground-glass opacities（13 cases,68.4%） and centrilobular nodules（8 cases,42.1%）.Airway obstruction in pulmonary function test,emphysema,lung cysts,and fibrosis on HRCT were more frequently seen in chronic than in subacute patients,though the differences were not statistically significant.Bronchoalveolar lavage fluid（BALF） showed lymphocytosis.The total cell count and the percentage of neutrophils were significantly higher in subacute than in chronic patients（P<0.05）.Nonnecrotizing granulomas were seen in 8（47.1%） cases.Improvement or normalization in symptoms,radiography,and pulmonary function test after treatment were seen in all 18 patients with available follow-up data.Five patients recurred.Conclusions The characteristic abnormalities of pulmonary function,findings on HRCT,and pathology are essential for all phases of EAA,and the atypical manifestations such as obstruction and fibrosis can also be present frequently,particularly in chronic cases.Differential cell counts of BALF are related to the phase of the disease.The treatment response and prognosis of EAA are good.4|16|2Updated：2024-04-10
- Abstract：Objective To explore the molecular mechanism of type 2 diabetes in intrauterine growth restricted adult rats through determination of blood glucose and expression of gluconeogenic enzymes in liver.Methods Male intrauterine growth restriction（IUGR） offspring induced by maternal protein-malnutrition and normal controls were studied.The body weights of offspring rats were weighted from birth to 12 weeks of age.Fasting plasma glucose and insulin levels were determined by glucose oxidase method and enzyme-linked immunosorbent assay（ELISA） respectively at 1 week,8 weeks,and 12 weeks.Peroxisome proliferator-activated receptor-γ coactivator-1α（PGC-1α）,phosphoenolpyruvate carboxykinase（PEPCK）,and glucose-6-phosphatase（G6Pase） mRNA and protein levels in liver were measured by real time RT-PCR and Western blot in newborn rats（Week 1） and adult rats（Week 12）.Results Birth weights of IUGR rats were significantly lower than those of controls until 4 weeks later,when IUGR rats caught up to controls.Between 8 and 12 weeks,the growth of IUGR rats surpassed that of controls.No significant differences were observed in blood glucose and insulin levels at newborn rats between the two groups.However,by the end of 8 weeks IUGR rats developed hyperinsulinemia and high insulin resistance index.At the age of 12 weeks,IUGR rats had mild fasting hyperglycemia.In addition,hepatic PGC-1α mRNA and protein levels as well as hepatic mRNA levels of PEPCK and G6Pase at Week 1 and Week 12 in IUGR rats were all significantly higher than those of controls（P<0.05）.Conclusions As a result of intrauterine malnutrition,the expression of gluconeogenic genes is exaggerated in offspring.This change stays through adulthood and may contribute to the pathogenesis of type 2 diabetes.4|29|2Updated：2024-04-10
- Abstract：Objective To determine the incidence,course,potential risk factors,and outcomes of noninfectious fever developed in patients after aortic surgery.Methods Patients who received operation for aortic aneurysm or dissection in our center from January 2006 to January 2008 were reviewed.Patients who met one of the following criteria were excluded:having a known source of infection during hospitalization;having a preoperative oral temperature greater than or equal to 38.0°C;undertaking emergency surgery;having incomplete data.Univariate analysis was performed in patients with noninfectious postoperative fever and those without,with respect to demographics,intraoperative data,etc.Risk factors for postoperative fever were considered for the multivariate logistic regression model if they had a P value less than 0.10 in the univariate analysis.Results Totally 463 patients undergoing aortic surgery were enrolled for full review.Among them,345（74.5%） patients had noninfectious postoperative fever,the other 118（25.5%） patients didn’t develop postoperative fever.Univariate analysis demonstrated that several risk factors were associated with the development of noninfectious postoperative fever,including weight,surgical procedure,minimum intraoperative bladder temperature,temperature upon intensive care unit（ICU） admission,discharge,and during ICU stay,as well as blood transfusion.In a further multivariate analysis,surgical site of thoracic and thoracoabdominal aorta（odds ratio:4.861;95% confidence interval:3.029-5.801;P=0.004）,lower minimum intraoperative bladder temperature（odds ratio:1.117;95% confidence interval:1.01-1.24;P=0.04）,and higher temperature on ad-mission to the ICU（odds ratio:2.57;95% confidence interval:1.28-5.18;P=0.008） were found to be significant predictors for noninfectious postoperative fever.No difference was found between the febrile and afebrile patients with regard to postoperative hospitalization duration（P=0.558） or total medical costs（P=0.896）.Conclusion Noninfectious postoperative fever following aortic surgery is very common and closely related with perioperative interventions.4|25|0Updated：2024-04-10
- Abstract：Objective To validate the predictive power of the 5th and 6th editions of TNM staging system（TNM-5,TNM-6） in a Chinese patient cohort with hepatocellular carcinoma（HCC） sized > or = 5 cm after radical hepatectomy.Methods Consecutive 121 patients with HCC sized > or = 5 cm undergoing radical hepatectomy between January 1995 and December 2002 were included.The impact of clinicopathological variables on prognosis was determined by univariate and multivariate analyses,after excluding 2 perioperative deaths.Results In univariate analysis,TNM-5 stage did not show prognostic significance for overall or disease-free survival,as opposed to TNM-6 stage,Edmondson-Steiner grade,portal vein tumor thrombosis（PVTT）,vascular invasion,satellite nodule,Child-Pugh grade,and hepatitis B surface antigen（HBsAg） positivity.When these significant variables were entered in multivariate analysis,Edmondson-Steiner grade was the sole independent prognosticator for both overall and disease-free survival,whereas Child-Pugh grade independently influenced disease-free survival.However,TNM-6 stage lost its predictive potential in multivariate analysis.Conclusions Neither TNM-5 nor TNM-6 staging system is revealed to be independently prognostic in patients with HCC sized > or = 5 cm after radical hepatectomy.Therefore,TNM-6 calls for more support in many subsets of HCC patients.4|13|0Updated：2024-04-10
- Abstract：Objective To investigate the prevalence of metabolic syndrome（MS） and its associations with other metabolic disorders and cardiovascular changes in health examination population in Beijing.Methods Totally,10 916 individuals who received health examination in Health Examination Center of Peking Union Medical College Hospital were enrolled.The height,weight,blood pressure,serum levels of triglyceride,high-density lipoprotein cholesterol（HDL-C）,and fasting blood glucose were recorded.MS was diagnosed based on the working criteria of Chinese Diabetes Society 2004（CDS2004）.Meanwhile,other metabolic disorders,including fatty liver and hyperuricemia,were recorded.The cardiovascular changes were reflected by the reports of electrocardiogram（ECG） ST-T changes and atherosclerosis of retinal arteries.Results The overall prevalence rate of MS was 6.1%（666/10 916） in the population.The prevalence rate of MS in male was much higher than that in female（9.0% vs.2.7%,P=0.000）.For individuals with MS,the prevalence rates of fatty liver and hyperuricemia were significantly higher than those without MS,respectively（70.4% vs.35.4%,P=0.000;29.9% vs.17.7%,P=0.000）.As for cardiovascular changes,the prevalence rates of ECG ST-T changes and atherosclerosis of retinal arteries were significantly higher in individuals with MS than those without MS,respectively（13.8% vs.11.7%,P=0.012;12.0% vs.6.8%,P=0.000）.Conclusions The prevalence of MS in Beijing population is high.The individuals with MS have a higher risk for other metabolic disorders and cardiovascular changes.4|36|5Updated：2024-04-10
- Abstract：Objective To explore the mechanisms involved in Staphylococcus aureus（S.aureus） invading human monocytic U937 cells.Methods S.aureus were added to U937 cells at multiplicity of infections（MOI） of 20:1 for 0,15,30,60,and 90 minutes,respectively.Cell apoptosis was analyzed with Hoechst 33258 staining and Annexin V-fluorescein isothiocyanate（FITC）/propidium iodide（PI） flow cytometry analysis.Akt and nuclear factor-κB（NF-κB） activities were detected by Western blotting.Results Infection of U937 cells with S.aureus induced rapid cell death in a time-dependent manner,and the cells displayed characteristic features of apoptosis.S.aureus-induced apoptosis was associated with a prominent downregulation of activated（phosphorylated） Akt and NF-κB.The inhibition of phosphorylated Akt by LY294002 led to the inhibition of NF-κB in a dose-dependent manner.Inhibition of Akt with LY294002 caused further increase in apoptosis of U937 cells.Conclusions S.aureus can stimulate the apoptosis of U937 cells.S.aureus induces apoptosis of U937 cells by inhibiting Akt-regulated NF-κB.4|33|6Updated：2024-04-10
- Abstract：Objective To investigate the effect of emodin on lipopolysaccharides（LPS）-induced corneal injury in rats.Methods Three parallel incisions on the central surface of corneal epithelium were made and LPS was applied on them to induce corneal injury in Wistar rats.All rats were randomly divided into emodin group（n=40） and keratitis group（n=40）.Rats in the emodin group received subconjunctival injection of emodin and rats in the keratitis group received its vehicle 30 minutes before LPS exposure.At different time points-1,3,6,12,and 24 hours after LPS exposure,the symptoms of all rats were observed and the severity of their ocular inflammation was examined with a slit lamp microscope,then 8 rats in each group were killed through cervical dislocation and their eyes were enucleated and prepared to observe pathological changes of corneal tissue under a light microscope.The activation of nuclear factor-κB（NF-κB） under different conditions was determined by Western blot.Immunocytochemistry staining with an antibody against intercellular adhesion molecule-1（ICAM-1） was performed to identify positive cells in corneal tissues.Results The model of acute keratitis was successfully established in Wistar rats.LPS could induce a typical corneal inflammatory response,such as hyperemia,corneal edema and opacity,which were observed in model rats.Compared with keratitis group,both ocular behaviors and damages of the corneal structure were improved in emodin group.Furthermore,the activation of NF-кB and the expression of ICAM-1 induced by LPS were markedly inhibited in emodin group.Conclusion Emodin can inhibit the activation of NF-κB and the expression of ICAM-1 induced by LPS in corneas,protect against acute corneal injury,and improve symptoms in rats.4|20|4Updated：2024-04-10
- Abstract：Objective To investigate the expression of programmed cell death 5（PDCD5） in tissues of normal human prostate（NP）,benign prostatic hyperplasia（BPH）,and prostate cancer（PCa） in order to assess the clinical role of PDCD5 in PCa.Methods PDCD5 expression was determined by EnVision immunohistochemical staining in formalin-fixed and paraffin-embedded specimens obtained from 12 subjects with NP,22 with BPH,and 22 with PCa.In addition,PCa cases were classified as low/middle-risk（Gleason sum≤7） and high-risk（Gleason sum>7） on the basis of Gleason grade.Positive expression rates and intensity of PDCD5 protein were observed under light microscope and analyzed with computer imaging technique.Expression of PDCD5 was compared among different prostatic tissues.Results The expression of PDCD5 was significantly lower in tissue of PCa than in tissues of NP and BPH（P<0.01）.However,there was no significant difference in PDCD5 expression between tissues of NP and BPH.In addition,the expression of PDCD5 was further downregulated with the increase of Gleason sum in PCa.Conclusions By downregulating apoptosis,low PDCD5 expression may play an important role in the occurrence and development of PCa.PDCD5 is supposed to have a potential clinical value to be a new predictor of progression and target of gene therapy in PCa.4|30|23Updated：2024-04-10
- Abstract：<正>HEREDITARY angioedema （HAE） is an autosomal dominant inherited condition which was initially described by Osler in 1888.4|13|1Updated：2024-04-10
- Abstract：<正>INTESTINAL lymphangiectasia （IL） is a rare disease first reported by Waldmanin in 1961.1 Since then, no more than two hundred cases have been reported.4|24|0Updated：2024-04-10
- Abstract：<正>Hereditary angioedema （HAE） is an autosomal dominant condition that affects one in about 50 000 persons,characterized by recurrent episodes of subcutaneous or submucosal swelling4|10|0Updated：2024-04-10
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