Objective To investigate the features of familiar facial palsy,ophthalmoplegia and dysphagia characterized by autosomal dominant inheritance in a family and to discuss the classification and pathogenesis of the disease.Methods Clinical,electrophysiological,pathological examinations were performed and blood samples were obtained from 5 patients and 26 family members.PCR protocol was used to identify a certain gene. Results In the 5 patients receiving physical examination,all had ptosis,external ophthalmoplegia,facial paralysis,dyphagia,hoarseness,decreased pharyngeal reflex;4 had amyotrophy of muscle of tongue,temporal nuscle,masseter and muscles of distal lower limbs;3 had proximal limb asthenia and distal limbs amyotrophy.Compared to those of oculopharyngeal muscular dystrophy(OPMD)with similar symptoms and signs,both electrophysiological manifestation and pathological findings of the family members supported the diagnosis of muscular dystrophy,but the(GCG)6(GCA)3GCG in the first exon of PABPN1 mutated neither in normal family members nor in patients.Conclusions This family presents clinical manifestations somewhat resembling to those of OPMD and distinctive to other disorders,but has a totally different genetic background from OPMD.It may be a new subtype of muscular dystrophy.

Animals ; Cadmium Chloride ; toxicity ; Dose-Response Relationship, Drug ; Estradiol ; toxicity ; Female ; Organ Size ; drug effects ; Rats ; Rats, Sprague-Dawley ; Uterus ; drug effects ; pathology

OBJECTIVETo investigate the influential factors for job burnout among the managerial staff in a Sino-Japanese joint venture automobile manufacturer in Guangzhou, China.

METHODSA total of 288 managers in a Sino-Japanese joint venture automobile manufacturer were surveyed using the Occupational Stress Indicator, Maslach Burnout Inventory (MBI), Eysenck Personality Questionnaire, Simplified Coping Style Questionnaire, and Social Support Rating Scale.

RESULTSOn the depersonalization dimension, the male managers had significantly higher scores than the female managers. The scores of emotion exhaustion and depersonalization of MBI showed significant differences among the managers with different levels of occupational stress. The path analysis showed that occupational stress, neuroticism, and psychoticism had negative effects on emotion exhaustion, while job satisfaction and utilization of social support had direct positive effects on emotion exhaustion. Occupational stress, psychoticism, and passive coping style had direct negative effects on depersonalization, while job satisfaction, objective support, and utilization of social support had positive effects on depersonalization. Job satisfaction and active coping style had positive effects on sense of personal accomplishment, while passive coping style had a negative effect on sense of personal accomplishment. Personality exerted its effect on social support through coping style and thus on job satisfaction and job burnout.

CONCLUSIONMale managers have a greater propensity to depersonalization than their female counterparts. High occupational stress is a risk factor for job burnout. Personality, social support, and coping style are influential factors for job burnout.

Administrative Personnel ; psychology ; Adult ; Burnout, Professional ; etiology ; psychology ; Fatigue ; etiology ; Female ; Humans ; Job Satisfaction ; Male ; Middle Aged ; Stress, Psychological ; Surveys and Questionnaires ; Young Adult

Objective To establish and evaluate criteria applied to review of complete blood counts (CBC)and differential results from automated hematology analyzers.Methods Temporary criteria were established by using alarm system of XE-2100 automated hematology analyzer and by consulting the 41 suggested rides of international consensus group.2 795 out-and in-patient samples were run as clinical samples.Stained blood films were prepared and manual differential with smear review were performed on all samples.Statistical analysis was done for each temporary rule and instrument flag which indicated abnormal cell quantity and morphology.Results Of all rules,instrument flags of ‘Immature Gran/Left Shift?’, ‘ Atypical Lympho?’and‘NRBC(nucleated red blood cell)?’showed most frequent false positive and false negative instrument flag.Evaluation on rnles about cell quantity change showed false positive and false negative rates were both low.Results of morphology evaluation showed that true positive rate was 17.44%, false positive rate was 15.82%,true negative rate was 63.49%,false negative rate was 3.25%.‘ Atypical Lymphocyte?’,‘Immature Gran?’and‘blast?’were the most frequent false positive flags.According to those results and clinicians opinions,our hematology review criteria for action following automated CBC and leukocytes differential was established.Conclusions The hematology review criteria have high true positive rate and low false negative rate.To clinical hematology laboratory using automated hematology analyzer,new criteria can reduce work load,bring lower false negative rate and higher work efficiency.

OBJECTIVETo establish the method of gas chromatograph-mass spectrometry (GC-MS) for the determination of the cotinine (COT) in human urine.

METHODSThe conjugated trans-3'-hydroxycotinine (THOC) and COT were hydrolyzed in human urine with beta-glucuronidase. The composition of COT was extracted with the mixture of dichloromethane and n-butyl acetate (2:1) and was separated with HP-5MS fused-silica capillary column. The GC-MS was used for determining its content.

RESULTSThe monitoring limit of this method was 0.02 microg/L. Its recovery rate was higher than 90%, Its accuracy rate was 4.30%. It was used for the determination of the cotinine in human urine in Guangzhou Biological Bank the Elderly Cohort.

CONCLUSIONThe GC-MS method is a good microanalysis for monitoring the cotinine in human urine rapidly and accurately with little background disturbance. It has been applied in our Guangzhou Cohort Study for determining cotinine in human urine.

Cotinine ; urine ; Female ; Gas Chromatography-Mass Spectrometry ; methods ; Humans ; Male ; Sensitivity and Specificity ; Smoking ; urine

Objective: To analyze associations of MRI-lesions and clinical features with disability in patients with multiple sclerosis (MS) in Shanghai, China. Methods: We studied patients with MS, identified from a survey in Shanghai, whose sites of lesions in the CNS was based on the MRI examinations. Associations between MRI-lesions, various clinical variables and the severity of disability were analyzed with univariate and multivariate logistic regression analysis. Results: There were 210 patients in this study. The disability of the patients with lesions confined to the spinal cord was significantly more severe than those with lesions in the brain (p < 0.008). Current age (OR: 1.041, 95% CI: 1.007~1.077), MS duration (OR: 1.082, 95% CI: 1.011~1.159) and MRI-lesions in the spinal cord (OR: 2.441, 95% CI: 1.039~5.737) were significantly associated with severity of disability on multivariate logistic regression analysis. Conclusion: MRI-lesions in the spinal cord, older age, a longer MS duration were significantly associated with a more severe disability in this MS study in Shanghai China.

BACKGROUNDThe new Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation was developed to address the systematic underestimation of glomerular filtration rate (GFR) by the Modification of Diet in Renal Disease (MDRD) Study equation in patients with relatively well-preserved kidney function. Performance of the new equation in the Chinese population is unknown. The goal of the present study was to compare performance of these two equations in Chinese patients with chronic kidney disease (CKD).

METHODSWe enrolled 450 Chinese patients (239 women and 211 men) with CKD in the present study. The renal dynamic imaging method was used to measure the referenced standard GFR (rGFR) for comparison with estimations using the two equations. Their overall performance was assessed with the Bland-Altman method and receiver-operating characteristics (ROC) analysis. Performance of the two equations in lower and higher estimated GFR (eGFR) subgroups was further investigated.

RESULTSBoth eGFRs correlated well with rGFR (r = 0.88, 0.81, P < 0.05). In overall performance, the CKD-EPI equation showed less bias, higher precision and improved accuracy, and was better for detecting CKD. In the higher-eGFR subgroup, the CKD-EPI equation corrected the underestimation of GFR by the abbreviated MDRD equation.

CONCLUSIONSThe CKD-EPI equation outperformed the abbreviated MDRD equation not only in overall performance but also in the subgroups studied. For the present, the CKD-EPI equation appears to be the first-choice prediction equation for estimating GFR.

Adult ; Aged ; Asian Continental Ancestry Group ; Female ; Glomerular Filtration Rate ; physiology ; Humans ; Kidney Failure, Chronic ; physiopathology ; Male ; Middle Aged ; Models, Theoretical

Objective To understand the changing trends of non-communicable diseases (NCDs) in Xinjiang Production and Construction Corps from 1998 to 2008.Methods A stratified-cluster random sampling based cross-sectional NCDs survey was carried out in 2008,and using the data of NCDs from the health service surveys in 1998 and 2004,in Xinjiang Production and Construction Corps.The prevalence rate of NCDs was standardization according to age proportion of the population being surveyed in 1998.Results In 1998,2004 and 2008,the prevalence rates of NCDs in Xinjiang Production and Construction Corps were 17.26%,25.61%,24.85% while the Standardized rates of NCDs were 17.26%,23.54% and 20.49% respectively.The prevalence rates of NCDs were statistically significant different in 35-,45-,55- and over 65 age groups in 1998,2004 and 2008 which showed an consecutive upward trend.The prevalence rates of hypertension,diabetes,cerebrovascular disease,coronary heart disease and chronic obstructive pulmonary disease increased significantly from 1998 to 2008.The prevalence rate of hypertensive disease among 25- age group,diabetes among 35- age group,cerebrovascular disease and coronary heart disease among 45- age groups showed an increasing trend.Conclusion Cardiovascular and cerebrovaseular diseases,together with diabetes were the fastest increasing ones over the past 10 years and becoming the major diseases,making the Xinjiang Production and Construction Corps an aging population.NCDs should be prioritized in the health development plan.Targeted health education should be carried out in the whole population,together with other interventions as well as management programs on chronic diseases to reduce the prevalence of NCDs.

Adolescent ; Adult ; Aged ; Female ; Hepatitis B, Chronic ; drug therapy ; Humans ; Male ; Medicine, Chinese Traditional ; Middle Aged

OBJECTIVETo observe the influence on prognosis and possible side-effects of arginine in

METHODSMulti-center clinical trial, randomized double blinded patients with severe trauma and burns. and placebo control methods were employed in the study. Eighty-six patients with severe trauma and burns were randomly divided into control (C, n = 45) and arginine treatment (Arg, n = 41) groups. The patients in Arg group received arginine in dose of 0. 4 g x kg(-1) x d(-1) orally, while those in C group received same dose of placebo (tyrosine) for 7 days. All the patients in both groups were given diet with equal calories and equal nitrogen content. The changes in the wound healing time, hospital stay, and the incidence of side-effects of the medication in both groups of patients were observed and compared before and after the supplementation of arginine.

RESULTSThe wound healing time and hospital stay days of severe trauma patient in Arg group (n = 29) were 11. 1+/-2. 8 d and 19+/-6 d, which were all obviously shorter than those in C group (13. 2+/-5. 5 d, 22 +/-6 d, n =33, P <0.05). On the other hand, in severe burn patients there were no significant difference of the wound healing time (20+/-5 d vs 22+/-8 d, n = 12, P > 0. 05) and hospital stay days (28+/-6 d vs 29+/-8 d, n = 12, P >0. 05) between the Arg and C groups. In addition, in C and Arg groups, the occurrence of the side-effects were seldom (2. 44% vs 2. 22% , P = 1. 000) and it disappeared when the supplementation of drugs was stopped.

CONCLUSIONOral feeding of arginine is beneficial in enhancing wound healing, reduction of hospital stay days in severe trauma patients and with little side-effects, but it is not beneficial to improve the prognosis of severe burn patients. Maybe this is due to inadequate number of case involved in the study.

Administration, Oral ; Adolescent ; Adult ; Aged ; Arginine ; administration & dosage ; adverse effects ; therapeutic use ; Burns ; diagnosis ; drug therapy ; Double-Blind Method ; Female ; Humans ; Length of Stay ; Male ; Middle Aged ; Prognosis ; Wound Healing