ObjectiveTo investigate expression distribution of mutant connexin 32 (Cx32) protein in human endothelial cells in patients with X-linked Charcot-Marie-Tooth disease type 1 ( CMTX1 ) .MethodsNerve biopsies were performed in 3 patients with CMTX1 and in 3 non-CMTX1 controls. Cx32 mutations of c. 379A ＞ T( I127F), c. 533A ＞ G(D178G) and c. 590C ＞ T(A197V) were identified in these 3 patients respectively.Immunofluorescent (IMF) staining of nerve blood vessel was processed with antibodies against Cx32, Yon Willebrand factor and Cx40. The mutant Cx32 was constructed in pEGFP-N plasmid (pEGFP-N1-Cx32) and was transfected in HeLa cells. Cx32 and GRP78, a marker of endoplasmic reticulum ( ER), were stained by IMF in HeLa cells to investigate expression of mutant Cx32. ResultsIn 3 control cases, Cx32 was visualized by IMF staining as dots along gap junction of vascular endothelial cells,and it was coexisted with Cx40.However, immunoreactivity of Cx32 in 3 patients was predominantly decreased and was not located in endothelial gap junction. The transfection of 3 Cx32 mutants into HeLa cells demonstrated thepathogenic changes.The cells withthemutationc. 379A ＞T found Cx32 accumulations in the cytoplasm; the cells with mutation c. 533A ＞G showed few staining positive dots surrounding the nuclear and the cells with c. 590C ＞ T showed dot-like expression of Cx32 both in the cytoplasmicand cell membrane. The mutant Cx32 was not overlapped with expression of the marker of ER.ConclusionsMutant Cx32 might cause dysfunction of endothelial gap-junctions due to the abnormal expression of Cx32 in level and location in the vascular endothelial cells of CMTX1 patients.

OBJECTIVETo investigate the clinicopathological features of pulmonary lymphangioleiomyomatosis (PLAM).

METHODSBy means of HE and immunohistochemistry (SP method) studies, the clinical and pathological features of 5 PLAM cases were analyzed and the related literature reviewed.

RESULTSPLAM was a rare lung disease of unknown etiology and was restricted to females who were generally pre-menopausal. Pathological features showed abnormal smooth muscle cells (LAM cells) line the airways, lymphatics and blood vesssels leading to airflow obstruction and replacement of the lung parenchyma by cysts. LAM cells were positive for HMB45. Clinically the disease was categorized by dyspnoea, haemoptysis, recurrent pneumothoraces and chylous effusions.

CONCLUSIONSPLAM should be considered when recurrent pneumothorax, haemoptysis and dyspnoea occur in females. Pathologic examination of lung tissue biopsy is required for confirmation of PLAM diagnosis.

Actins ; metabolism ; Adult ; Antigens, Neoplasm ; Biopsy ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Lung ; diagnostic imaging ; pathology ; Lung Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Lymphangioleiomyomatosis ; diagnostic imaging ; metabolism ; pathology ; Matrix Metalloproteinase 2 ; metabolism ; Melanoma-Specific Antigens ; Middle Aged ; Neoplasm Proteins ; metabolism ; Tomography, X-Ray Computed

BACKGROUNDControversies on the safety of the cement application between cemented and uncemented total hip arthroplasty (THA) have been existing for decades. The purpose of this study was to observe the changes in mean arterial pressure (MAP), heart rate (HR) and oxygen pressure (PaO(2)) during cemented THA, and to evaluate the intraoperative safety of using the third-generation cementing technique and investigate whether the intraoperative risk is higher in acute femoral neck fracture patients than non-traumatic patients.

METHODSForty-two patients who underwent cemented THA between November 2005 and September 2007 were prospectively included in this study. The third-generation cementing technique as vacuum mixing and pulsatile lavage was used strictly. The MAP and HR were monitored and documented during each operation. Blood gas analysis was performed at exposure, cup implantation, stem implantation and wound closure. MAP, HR and PaO(2) were compared between pre- and post-cement application. Comparisons of MAP, HR and PaO(2) between patients with acute femoral neck fracture and non-traumatic patients were performed as well.

RESULTSNo intraoperative cardiopulmonary complication occurred in these cases. No obvious changes were observed in MAP, HR and PaO(2) after cement application. There was no significant difference in MAP, HR and PaO(2) between acute femoral fracture patients (18 patients) and non-traumatic patients (24 patients).

CONCLUSIONSThe results of this study suggested that the invasive blood pressure monitoring and blood gas analysis are essential for patients undergoing cemented THA, especially for patients with femoral neck fracture. The third-generation cementing technique is safe to use in THA.

Adult ; Aged ; Aged, 80 and over ; Arthroplasty, Replacement, Hip ; adverse effects ; methods ; Cementation ; methods ; Female ; Humans ; Male ; Middle Aged ; Monitoring, Intraoperative ; methods ; Prospective Studies

OBJECTIVETo investigate the changes of CD4(+)CD28(-) T cell and CD4(+)CD25(+) regulatory T cell (Treg) subsets in patients with coronary artery disease (CAD).

METHODSTwenty-eight patients with angiographically established CAD were recruited in this study, including 16 with unstable angina (UA group) and 12 with stable angina (SA group). Eleven patients with chest pain syndrome served as the control group. The proportions of peripheral CD4(+)CD28(-) T cells and CD4(+)CD25(+) Treg subsets were determined with fluorescence-activated cell sorting (FACS).

RESULTSThe proportions of CD4(+)CD25(+) Treg were significantly lower in UA group (6.55-/+2.45%) than in SA (14.01-/+4.92%) and control groups (13.55-/+3.87%). The proportions of CD4(+)CD28(-) T cells were significantly higher in UA group (10.55-/+4.76%) than in SA (2.64-/+1.33%) and control (2.75-/+1.55%) groups.

CONCLUSIONAlterations of circulating T-lymphocyte subsets occur in patients with UA. The changes of Treg and CD4(+)CD28(-) T cells may lead to breakdown of peripheral autoimmune tolerance and play an important role in the development and progression of CHD.

Aged ; Angina, Unstable ; immunology ; CD28 Antigens ; CD4-Positive T-Lymphocytes ; immunology ; Case-Control Studies ; Coronary Disease ; immunology ; Female ; Humans ; Interleukin-2 Receptor alpha Subunit ; Male ; Middle Aged ; T-Lymphocyte Subsets ; immunology ; T-Lymphocytes, Regulatory ; immunology

OBJECTIVETo investigate water exposure modes and times of different populations in mountainous schistosomiasis endemic areas and to inform about the control strategies.

METHODSAll 1054 residents from populations around Qionghai Lake were randomly sampled according to occupation for a retrospective questionnaire survey in November 2001. Each individual was interviewed for his/her mode, frequency, and duration of water exposure occurring between April and October 2001.

RESULTSThe average exposure times and intensity were higher in farmers (median: 16 - 18 min/day and 2.41 - 2.5, respectively) who grow rice, tobacco, and vegetables than others (median: 3.74 - 7.39 min/day and 0.81 - 1.52, respectively); exposure frequency was found highest in farmers (median: 2.04 times/day) in all occupations; schoolchildren had low exposure frequency and times, but very high exposure intensity (median 2.34). Between April and June it is an agriculturally busy season, that is also a peak season of water exposure of adults. Schoolchildren's water exposure peaks on July and August, mainly due to playing water and swimming. Exposure times and intensities were higher in females than in males.

CONCLUSIONWater exposure modes, times, and intensities of different populations were different in mountainous schistosomiasis endemic areas of Xichang. Between April and June should be the peak infection season of adults who are engaging in agricultural activities, while July to August should be the peak infection season for schoolchildren with non-agricultural activities.

Adult ; China ; Environmental Exposure ; analysis ; Female ; Humans ; Male ; Retrospective Studies ; Schistosomiasis ; epidemiology ; Surveys and Questionnaires ; Topography, Medical ; Water ; parasitology

OBJECTIVETo explore the synergetic effect of norcantharidin (NCTD) and adriamycin (ADR) on the proliferation and apoptosis of multiple myeloma (MM) cells.

METHODSHuman MM cell line U266 cells were treated with NCTD alone (10 µmol/L) or in combination with ADR (0.25 µmol/L). MTT and Annexin V/PI staining were used to determine cell viability and apoptosis. The protein expression of nuclear factor-κB P65 (NF-κB P65), phosphorylated NF-κB p65 (p-NF-κB p65), NF-κB P65 inhibitor IκBα, phosphorylated IκBα (p-IκBα), survivin, Bcl-2 and Bax were determined by Western blot. Immunohistochemistry was used to determine the expression of vascular endothelial growth factor (VEGF).

RESULTS(1) NCTD potentiated the cytotoxicity and pro-apoptotic effects induced by ADR. The combination of NCTD and ADR had synergistic anti-proliferation effect. (2) Combination of ADR and NCTD downregulated the expression of nuclear NF-κB P65 and cytoplasm p-IκBα induced by ADR. The expression of nuclear NF-κB P65 and cytoplasm p-IκBα decreased from 2.08 ± 0.29 and 0.39 ± 0.07 to 0.48 ± 0.08 and 0.02 ± 0.01 respectively, while the expression of the cytoplasm NF-κB P65 and IκBα were unchanged in the ADR alone group and the combined group. (3) The expression of survivin and bcl-2 decreased from 0.31 ± 0.05 and 0.23 ± 0.05 to 0.03 ± 0.02 and 0.05 ± 0.02, while the expression of Bax increased from 0.46 ± 0.06 to 0.62 ± 0.08 respectively in ADR alone group and combined group. (4) The positive rate of VEGF in ADR group and combination group were (44.6 ± 4.4)% and (27.0 ± 2.1)% respectively, indicating that NCTD could potentiate the inhibition effect on VEGF induced by ADR.

CONCLUSIONSThe results suggest that NCTD can potentialize the chemosensitivity of multiple myeloma cells to ADR through regulating NF-κB/IκBα signaling pathway and NF-κB-regulated gene products including survivin, Bcl-2, Bax and VEGF.

Bridged Bicyclo Compounds, Heterocyclic ; pharmacology ; Cell Line, Tumor ; Cell Proliferation ; Down-Regulation ; Doxorubicin ; pharmacology ; Humans ; I-kappa B Proteins ; metabolism ; Inhibitor of Apoptosis Proteins ; metabolism ; Multiple Myeloma ; metabolism ; NF-KappaB Inhibitor alpha ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Signal Transduction ; drug effects ; Transcription Factor RelA ; metabolism ; Vascular Endothelial Growth Factor A ; metabolism ; bcl-2-Associated X Protein ; metabolism

Adult ; Aged ; Brugada Syndrome ; diagnosis ; virology ; Humans ; Influenza A Virus, H7N9 Subtype ; pathogenicity ; Influenza, Human ; diagnosis ; virology ; Male ; Middle Aged

OBJECTIVETo evaluate the efficacy and safety of IDA (Haizheng Parmacy, China) in the treatment of acute leukemia.

METHODSA multi-institutional single-blind randomized controlled clinical trial was carried out. A total of 155 newly diagnosed patients with AML and ALL were enrolled. The patients were randomly divided into two groups, one was given IDA (n = 77) and the other given zevodas (Pharnacia & Upjohn, n = 78) for comparison.

RESULTSAll the patients enrolled in this trial were eligible for assessment of side effects, and 129 patients for evaluation of overall response rate. In patients treated with IDA vs zevodas, the overall response rate (OR) was 78.1% vs 76.9%, CR was 68.8% vs 67.7%; in AML patients, OR was 82.4% vs 71.8%, and CR was 76.5% vs 64.1%; in ALL patients, OR was 80.0% vs 81.8%, and CR was 68.0% vs 68.2%. There was no sitatistically significant difference in hematologic and non-hematologic toxicities between the two groups.

CONCLUSIONThe efficacy of IDA in the treatment of acute leukemia is comparable to that of zevodas. Both have similar toxic side effects.

Adolescent ; Adult ; Aged ; Antibiotics, Antineoplastic ; adverse effects ; therapeutic use ; China ; Female ; Humans ; Idarubicin ; adverse effects ; therapeutic use ; Leukemia, Myeloid, Acute ; drug therapy ; Male ; Middle Aged ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; Single-Blind Method

To revise GBZ 188 Technical Specification for Occupational Health Surveillance based on national laws, regulations, standards, specifications and legal documents of occupational disease, and combination with the actual situation in China. The main modifications are as follows: the occupational health surveillance for workers exposed to toluene (xylene may implement by reference), bromopropane, methyl iodide, ethylene oxide, chloroacetic acid, indium and its compounds, coal tar, coal tarasphalt, asphalt, β-naphthylamine, dust of metal and its compounds(tin, iron, antimony, barium and its compounds), hard metal dust, erionite dust, low temperature, laser, tick-borne encephalitis virus, Borrelia burgdorferi, and human immunodeficiency virus, for scraper or grind operators, and underground workers using squatting or kneeling position, crawling position, side-lying position, or shoulder position for a long period of time are included. The emergency health screening for workers exposed to arsenic, fluorine and its inorganic compounds, and acrylamide are included. The occupational medical examination (OME) for workers exposed to amino and nitro compounds of benzene, phosgene, monomethylamine, organic fluorine and dimethyl sulfate has been adjusted and made mandatory, with corresponding assessments required upon leaving the job. The special occupational health surveillance for workers exposed to mycobacterium tuberculosis and hepatitis virus is removed. The OME conclusion of reexamination is removed, and standardize recheck/additional inspection requirements. The optional items in OME performed before, during and after leaving post are removed, but the optional items in emergency medical examination are retained. Additional OME items are added. The Guideline for OME Summary Reports is added as informative appendix, and so on. The revised GBZ 188 Technical Specification for Occupational Health Surveillance is more scientific and practical.

BACKGROUNDCoronary artery disease (CAD) is generally considered as a disease of middle-aged men. It is widely accepted that the risk for CAD of premenopausal women is low because of hormone protection. Based on our clinical experience, more and more premenopausal women suffer from angina and myocardial infarction without adequate concern. Even now, there are still limited detailed data to describe the characteristics, mechanism and prognosis of premenopausal CAD patients. This article aimed to analyze the clinical and angiographic characteristics of premenopausal women with CAD.

METHODSA total of 565 premenopausal women and 721 postmenopausal women (56 - 60 years old) who underwent coronary angiography for the first time from April 2004 to December 2007 were enrolled. The clinical data and coronary angiographic characteristics (presence, localization, length and severity) were compared between the premenopausal and postmenopausal CAD groups.

RESULTSPremenopausal CAD patients presented less frequently with hypertension, diabetes mellitus and dyslipidemia compared with postmenopausal CAD patients (55.0% vs 66.0%, 15.0% vs 31.5%, 23.9% vs 37.4%, respectively; all P < 0.05). Although we found more frequent involvement of single vessel in premenopausal CAD (43.2% vs 26.9%, P = 0), and triple vessels in postmenopausal (56 - 60 years old) CAD patients (33.8% vs 20.4%, P = 0), much more severe lesions (> or = 90%) at left main (2.9% vs 1.1%, P = 0.048) and proximal left anterior descending artery (LAD) (28.2% vs 16.6%, P = 0) in the premenopausal CAD group were found.

CONCLUSIONPremenopausal women with chest discomfort are always found to have obvious atherosclerosis, more inclined to be located at the left main and proximal LAD, which is a strong predictor of an adverse clinical outcome.

Coronary Angiography ; Coronary Artery Disease ; diagnostic imaging ; pathology ; Diabetes Mellitus ; pathology ; Dyslipidemias ; pathology ; Female ; Humans ; Hypertension ; pathology ; Middle Aged ; Postmenopause ; Premenopause