Objective To report the development of clinical symptoms in a Chinese family with autosomal dominant progressive external ophthalmoplegia(adPEO).Methods Electromyologram and muscle biopsy were performed in the proband and 4 family members with the disease.Results The proband was a 57 year-old woman,who developed bilateral ptosis after the age of 30,external ophthalmoplegia after the age of 35 years old,weakness of extremities at the age of 37 years old and bulb palsy with palmus at the age of 47 years old.In the family there were 20 male and female members from five generations.All of them complained about bilateral ptosis between 26—33 years old,external ophthalmoplegia(12/15)and weakness of all extremities(14/15)between 35—45,facial and masticatory weakness(9/9)as well as dysphagia(8/9)between 44—60,accompanied with heart lesions(4/7)after 50 years old.Some patients died due to cardiac impairment.Electromyologram showed myopathic abnormalities in the examined patients. The main myopathological changes were ragged red fibers,cytochrome c oxidase negative fibers and ragged blue fibers in succinate dehydrogenase staining.Conclusions The adPEO started from extra-ocular muscles to limbs,finally facial and bulbar muscles.Heart lesions were presented in late stage and lead to death in some members.The developing process of symptoms suggested that we should pay more attention to cardiac manifestations in this disease.

Objective To investigate the axonal lesion in chronic inflammatory demyelinating polyneuropathy(CIDP).Methods Eighteen patients had undergone sural nerve biopsy.The clinical and electrophysiological distinction based on the different pathological changes were analyzed.Results Five patients with demyelination predominance which presented myelinated fiber with thin myelin.Three of them showed also mild axonal degeneration.Eight patients with axonal lesion predominance which presented Wallerian degeneration and regeneration of myelinated fibers.Three patients with mixed myelin and axon lesion of myelinated fibers and two with mild lesion.There was no significant difference between CIDP predominantly with axonal lesion and demyelination.Electrophysiological examination shows both axonal lesion and demyelination feature in some of the 2 types patients at the same time.Conclusions Axonal lesion is a common pathological change in CIDP and should not be considered as an exclusive criterion in diagnosis of the disease.Infiltration of macrophages is a common change.

Objective To explore the influence of hemodialysis(HD) and henodiafiltation on serum ?_2-microglobulin(?_2-MG) of children with acute renal failure and contrast research on declining rate of serum ?_2-MG before and after(children′s) HD and HDF.(Met-)hods By Branc Dialog HD machine of double-pump,HDF for 18 times and HD for 20 times were given to children with acute renal(fai-)lure.The serum ?_2-MG were observed before and after HD and HDF.Results A great difference was observed in level of serum ?_2-MG between before and after HD and HDF.In HDF group,there was significant difference in level of serum ?_2-MG before and after HDF(P

Objective To discuss the application of the hemodialysis and filtration(HDF) in children with acute renal failure(ARF).Methods By Branc Dialog hemodialysis machine of double-pump,HDF for 19 times and hemodialysis(HD) for 21 times were given to the children with ARF.The serum blood urea nitrogen(BUN),creatinine(Cr),uric acid(UA),?_2 microglobulin(?_2-MG) and the rate of the heart,blood pressure,temperature were observed before and after HD and HDF.Results The clearance rates of BUN,Cr,UA,(?_2-)MG of HDF were markedly higher than those of HD(P

OBJECTIVETo analyze the monitoring results of the occupational hazard factors in workplaces in Zhejiang Province, China in 2006 - 2010.

METHODSDescriptive analysis was performed on the monitoring results of the occupational hazard factors in workplaces in Zhejiang Province from 2006 to 2010.

RESULTSFrom 2006 to 2012, the number of monitored objects for each occupational hazard factor increased gradually, and the qualified rate for each factor remained unchanged or rose slightly. The qualified rates for silica dust and asbestos dust were less than 60%. The qualified rates for benzene, toluene, and xylene, which were always the factors highlighted in the monitoring of toxic chemicals, were all above 85%. The numbers of monitored objects for hexane, hydrogen sulfide, and cyanide grew significantly in recent years. However, the qualified rates for physical factors, which mainly included high temperature and noise, were less than 70%, lower than those for chemical factors.

CONCLUSIONThe qualified rate is as important as the number of monitored objects in the monitoring of the occupational hazard factors in workplaces in Zhejiang Province, China. The acute and chronic factors, physical and chemical factors, and traditional and new factors should be balanced in monitoring.

China ; epidemiology ; Dust ; analysis ; Environmental Monitoring ; Occupational Diseases ; epidemiology ; prevention & control ; Risk Factors ; Workplace

OBJECTIVETo apply theory of reasoned action at survey on welding workers occupational health protection behaviors and explore related influencing factors.

METHODSnine companies were randomly selected from areas with many welding works in Zhejiang Province. All welding workers were surveyed using a questionnaire based on theory of reasoned action.

RESULTS10.06%, 26.80% and 37.50% of the respondents never or seldom used eyeshade, mask and earplug, respectively. After controlling the socio-demographic factors, welding workers' behavioral belief was correlated with the behaviors of eyeshade-mask and earplug use (χ(2) = 31.88, 18.77 and 37.77, P < 0.01). the subjective norm of company was correlated with all protection behaviors (χ(2) = 20.60, 10.98 and 19.86, P < 0.01), the subjective norm of colleague was correlated with mask and earplug use, (χ(2) = 27.43, 36.39, P < 0.01), and the subjective norm of family was correlated with mask use (χ(2) = 5.73, P < 0.05).

CONCLUSIONTheory of reasoned action is suitable for welding worker occupational health related behaviors. It is useful to improve occupational health education, to effectively select health education objective and to tailor health education contents.

Adult ; Health Behavior ; Health Education ; Humans ; Male ; Occupational Diseases ; prevention & control ; Occupational Health ; Protective Devices ; utilization ; Surveys and Questionnaires ; Welding ; Young Adult

OBJECTIVERasmussen syndrome (RS) is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. The present study aimed to analyze the electroclinical characteristics and treatment of RS.

METHODSThe medical records of 16 children with RS were retrospectively reviewed.

RESULTSOf the 16 children, 8 were males and 8 were females. The age of onset was from 1 year and 11 months to 11 years and 6 months. The first symptom was seizure in all patients. The main seizure type was partial motor seizures. In all the patients, seizures gradually became frequent and in the form of epilepsia partialis continua (EPC). Thirteen cases developed hemiparesis. Fixed hemiparesis occurred from 2 months to 3 years after the onset of seizures. The cognitive deterioration was present in 14. The EEG background activity was abnormal in all the cases, asymmetric slow wave disturbances were bilateral but with unilateral predominance in 11, unilateral delta or theta wave in 8. The presence of interictal epileptiform discharges were found in all cases, unilateral in 11 and bilateral in 5. Seizures were recorded in all patients, no electroclinical correlation was found in 5. Serial magnetic resonance imaging (MRI) showed progressive unihemispheric or focal cortical atrophy in all cases. Six cases transiently showed focal cortical swelling or T2/FLAIR hyperintense signal on early scans. Antiepileptic drugs were not effective in any of the patients. Three of 10 patients receiving immunoglobulin, and 4 of 8 receiving corticosteroids, had some reduction of seizure frequency for a short period. Six patients accepted functional hemispherectomy, in 4 of them seizure no longer occurred and cognitive function was improved. The results of multiple subpial transection in 2 cases and focal resection in one patient were disappointing.

CONCLUSIONThe clinical features of RS were refractory partial epilepsy, progressive hemiplegia and cognitive deterioration. The EEG background was asymmetric with slow wave activity, interictal epileptiform discharges were unilateral or bilateral, no electroclinical correlation occurred. Serial MRI showed progressive unihemispheric focal cortical atrophy. Antiepileptic drugs were not effective for RS. In some patients, immunoglobulin or corticosteroids could reduce seizure frequency in the short term. Functional hemispherectomy could lead to seizure control and prevent further development of neurological impairment and cognitive deterioration.

Anticonvulsants ; therapeutic use ; Child ; Child, Preschool ; Cognition ; drug effects ; Electroencephalography ; Encephalitis ; drug therapy ; physiopathology ; prevention & control ; Epilepsia Partialis Continua ; drug therapy ; prevention & control ; Epilepsy ; etiology ; prevention & control ; Female ; Hemispherectomy ; methods ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Treatment Outcome

OBJECTIVETo study comparatively the cytotoxicity induced by acid bentonite and organic bentonite.

METHODSThe cytotoxicity of two kinds of bentonite was detected using CCK8 assay, neutral red uptake (NRU) assay, lactate dehydrogenase (LDH) leakage assay, apoptosis assay and hemolysis assay. In hemolysis assay human erythrocytes served as target cells and were exposed to the two kinds of bentonite at the doses of 0, 0.3125, 0.6250, 1.2500 and 2.5000 mg/ml for ten min. In other four assays, human B lymphoblast cells (HMy2.CIR) served as target cells and were exposed to the two kinds of bentonite at the doses of 0, 10, 20, 30, 60, 120 and 180 microg/ml for four h.

RESULTSIn hemolysis assay, the hemolysis rates induced by two kinds of bentonite at all doses were significantly higher than that of control (P<0.05); in CCK-8 assay, the cellular activities in acid bentonite group at the doses > or =30 microg/ml and in organic bentonite group at the doses > or =20 microg/ml were significantly lower than that of control (P<0.01); the similar results appeared in NRU assay and LDH assay, and the dose-effect relationship was observed in above 4 assays. In apoptosis assay, the early apoptosis cell rates in acid bentonite group at the dose of 180 microg/ml and in organic bentonite group at the doses of 120,180 microg/ml were significantly higher than that of control (P<0.05). Moreover, the results of five in vitro assays indicated the cytotoxicity induced by organic bentonite was higher than that induced by acid bentonite.

CONCLUSIONTwo kinds of bentonite could induce cytotoxicity, such as apoptosis and damage of cell membrane. The cytotoxicity of organic bentonite is higher than that of acid bentonite due to the different industrial treatment and characteristics of two kinds of bentonite particles.

Apoptosis ; drug effects ; Bentonite ; toxicity ; Cell Line ; Cytotoxicity Tests, Immunologic ; Erythrocytes ; drug effects ; pathology ; Hemolysis ; drug effects ; Humans ; Lymphocytes ; drug effects ; pathology

OBJECTIVETo study the genotoxicity induced by organic bentonite particles in vitro.

METHODSHuman B lymphoblast cells (HMy2.CIR) were exposed to organic bentonite particles at the doses of 0, 1.88, 3.75, 7.50 and 15.00 µg/ml for 24, 48 and 72 h, calcium sulfate (30 µg/ml) and SiO2 (30 and 240 µg/ml) served as negative and positive controls, respectively. The genotoxicity of organic bentonite particles and soluble fraction was detected using comet assay and Cytokinesis-block micronucleus (CBMN) assay.

RESULTSThe results of comet assay indicated that % tail DNA increased with the exposure doses and time in organic bentonite group, % tail DNA at the dose of 15.00 µg/ml for 24 h, 48 h and 72 h in organic bentonite group were 3.20 ± 0.19, 4.63 ± 0.88 and 9.49 ± 1.31 respectively which were significantly higher than those in calcium sulfate group (1.40 ± 0.11, 1.37 ± 0.22 and 0.90 ± 0.16) and those in 30 µg/ml SiO2 group (1.83 ± 0.21, 1.41 ± 0.27 and 2.48 ± 0.25) (P < 0.01). The results of CBMN assay showed that micronucleus frequencies (MNF) in organic bentonite group (except for 1.88 µg/ml for 24 h) were significantly higher than those in 30 µg/ml calcium sulfate group (MNF for 24, 48 and 72 h were 1.33‰ ± 0.58‰, 1.33‰ ± 1.15‰ and 1.33‰ ± 0.58‰) and those in 30 µg/ml SiO2 group (2.00‰ ± 0.00‰, 1.68‰ ± 0.58‰ and 2.33‰ ± 0.58‰) (P < 0.01). The results of two assays demonstrated that the soluble fraction of organic bentonite did not induce the genotoxicity.

CONCLUSIONThe organic bentonite dusts can induce the genotoxicity in vitro, which may be from the particle fraction.

Bentonite ; toxicity ; Cells, Cultured ; Comet Assay ; DNA Damage ; Humans ; Lymphocytes ; drug effects ; Micronucleus Tests ; Mutagenicity Tests ; Quartz ; toxicity