Objective To discuss the meaning of the method of two-step pattern case teaching in image diagnostics. Methods The experiment group was taught by the two-step pattern case teaching,while the control group by the traditional method. Results The experiment group’s image diagnostics exam average score is higher than the control group’s,and the average scores of other major clinical courses are all higher. Conclusion The applications of the two-step pattern case teaching in medicine teaching is the need of developing the medical education business and improving the medical teaching quality.

Objective To discuss the importance of the clinic skill learning center in diagnostics teaching.Methods The experiment group was taught in the clinic skill learning center with the reformed method,while the control group was taught in the traditional laboratories with the traditional one.Results The experiment group's two average scores of diagnostics and skill examine are higher than the control group's,and the difference between the two group's average scores are significant in the statistics analysis.Conclusion The applications of the clinical skill learning center in medicine teaching is the need of developing the medical education business and fostering standardized medical talents.

Objective To explore the intervention effect of emodin on organophosphorus poisoning induced respiratory failure.Methods 60 male Wistar rats of clean grade were randomly divided into:normal control group, model control group, positive drug group and emodin group, with 15 rats in each group.Except the normal control group rats were given intraperitoneal anesthesia, the right common carotid artery intubation, when rats stayed awake began a septic model.Blood gas analysis and serum level of oxygen free radicals and respiratory rate were compared before poisoning, respiratory failure, intervention of 5, 10, 30 min.Results Mouth breathing, slow respiratory frequency and cyanosis, appeared after exposure.Respiratory frequency decreased after exposure , compared with the positive drug group, respiratory frequency of emodin group 10 min and 30 min was higher ( P<0.05), PaO2, SaO2, BE decreased, PaCO2 increased after respiratory failure, Compared with the positive drug group, PaO2, PaCO2, SaO2 and BE of emodin group for the treatment of 10 min, 30 min was higher,(P <0.05).The level of oxygen free radicals in rats of each group had no significant difference before the exposure and the respiratory failure.Compared with the positive drug group, SOD and MDA of emodin group in 30 min after intervention were higher,( P<0.05 ) .Conclusion Emodin can improve the respiratory frequency of organic phosphorus poisoning induced respiratory failure ,improve blood gas analysis of the indicators and the level of oxygen free radicals.

Objective To survey the knowledge requested by patients with coronary artery disease (CAD) after stent implantation.Methods A survey on CAD and stent-related knowledge requested by CAD patients with stent implantation was conducted with a self-designed questionnaire from September 2013 to November 2013.Total 362 patients (235 males and 127 females) aged (67 ± 12) years participated in the survey in Yuetan Community Health Service Center and Fuxing Hospital in Beijing.Open questions and heuristic questions were used together during the survey.Results The knowledge requested by patients were:diet management(255,70.4％),side effects of medication(241,66.6％),management of physical activity (221,61.0％),prevention of CAD(181,50.0％),withdrawal of medication(176,48.6％),the life of stents (168,46.4％),post-operation check-up items (162,44.8％),the causes of CAD (156,43.1％)and drug mechanisms(134,37.0％).Patients with stent implantation less than one year requested more knowledge about the cause of CAD (x2 =37.4,P ＜ 0.001),life-time of stent (x2 =39.9,P ＜0.001),prevention of CAD (x2 =13.4,P =0.001),post-operation check-up items (x2 =21.2,P =0.001) and requested less knowledge about withdrawal of medication (x2 =9.56,P =0.008),diet management (x2 =6.49,P =0.04) than those with more than one year.Patients aged ＜ 70 years requested more knowledge about the cause of CAD (x2 =31.6,P ＜ 0.001),prevention of CAD (x2 =9.99,P =0.007),life-time of stent (x2 =79.1,P ＜ 0.001),side effects of medication (x2 =7.84,P =0.02) than patients aged ＞ 70 years.Patients with education higher than junior high school requested more knowledge about the cause of CAD (x2 =26.6,P ＜ 0.001),prevention of CAD (x2 =14.4,P =0.001) and life-time of stents (x2 =7.82,P =0.02) than those with education lower than junior high school.Patients with education higher than junior college requested more knowledge about the cause of CAD (x2 =26.6,P ＜ 0.001) and post-operation check-up items (x2 =10.7,P =0.005) than those with education lower than junior college.Conclusion Some factors affect the knowledge requested by patients with coronary artery disease (CAD) after stent implantation,which should be considered in health education for CAD patients.

Objective To evaluate the prevention and treatment effect of butylphthalide injection in delayed encephalopathy after severe carbon monoxide poisoning(DESCMP).Methods One hundred and fiftyeight cases with severe carbon monoxide poisoning (SCMP) were randomly assigned to 2 groups.Observation group (n =83), butylphthalide treatment when SCMP was diagnosed;Control group (n =75), with no butylphthalide therapy.Incidence rate in 3 month, drug amount and the clinical effects of butylphthalide were observed.Results Observation group occurred 5 DEACMP, with the incidence rate of 6.58％, and control group occurred 14 cases,with the incidence rate of 18.67％ ,significant difference was observed in two groups(x2 =154.204,P＜0.05).There were also significant differences in the glasgow coma scale(14.00±1.89 vs.13.00 ±3.30,t =1.780, P＜ 0.05) and mini-mental state examination (26.20± 2.89 vs.19.57 ± 7.12, t =2.003, P＜0.05) between the two groups.Conclusion Early treatment with butylphthalide can decrease the incidence rate of DEACMP.

Objective:To evaluate the endothelial functions and autoregulation capacity of cerebral blood flow in patients with Fabry disease .Methods:Brachial artery vasodilation was assessed in 8 pa-tients with Fabry disease and 14 healthy controls by means of flow-mediated dilation ( FMD) and Nitro-glycerin-mediated dilation ( NMD) .Cerebrovascular reactivity was calculated in terms of breath-holding index ( BHI) and vascular motor reactivity ( VMR) by TCD-CO2 test in 4 patients and 14 healthy con-trols.Results:Compared with the controls , brachial artery vasodilation experiment showed no difference (the patients:FMD 15.94%±5.03% and NMD 23.92%±7.23%, the controls: FMD 14.57%± 5 .84% and NMD 22 .64%±6 .96%) , there was no relationship between FMD or NMD and the age , course of disease , MSSI or enzyme activity .In respect of cerebrovascular autoregulation capacity , there was no difference in anterior circulation , while cerebrovascular reactivity tended to be impaired in posteri-or circulation .Conclusion:Endothelial function showed no decline in patients with Fabry disease , but cerebrovascular autoregulation capacity tended to be impaired in posterior circulation .

Objectives To report clinical,electrophysiological and pathological features of myopathy with tubular aggregates.Methods The onset of the disease was between 5-50 years old in the 6 sporadic male cases.Skeletal muscle biopsy was performed for all cases.Specimens were examined histochemically,enzymhistochemically and electromicroscopically.Results Case 1 and 2 presented with limb girdle myasthenic syndrome.The case 1 developed exercise-induced cramps in the late stage.Case 3 complained about persistent weakness and Dopa-responsive dystonia.Case 4 and 5 were characterized by periodic paralysis.Case 6 showed exercise-induced cramps.Serum potassium was normal in all patients. Slight elevation of serum creatine kinase appeared in 3 cases.Electromyography showed neurogenic pattern in case 1 and 6,myogenic changes in case 4 and 5,and no abnormality in other 2 cases.Marked decrement of active potential amplitude was noted with low frequency repetitive nerve stimuli in case 1 and 2.Four percent to forty percent of muscle fibers showed focal material accumulation in the fibers,which involved mainly type 2 fibers in all cases.The material was stained bright red material with modified gomori trichrome,intensive staining with nicotinamide adenine dinucleotide-tetrazolium reductase,lack activity of succinate dehydrogenase and ATPase.Electron microscopy confirmed bundles of parallel micro-tubular structure in the muscle fiber.Conclusions Myopathy with tubular aggregates has various clinical subtypes and electromyographic pattern.Dystonia or other systemic symptoms could be noted in this disease.The limb girdle myasthenic syndrome can also be accompanied with exercise-induced cramps.

Objective To investigate the effect of α-galactosidase A (GLA) gene mutation on cell autophagy and to elucidate its mechanism preliminarily.Methods Two families were diagnosed by ultrastructural pathological examination,GLA gene activity test and GLA gene mutation screening.Mutant type recombinant expression plasmid of two pedigrees (pcDNA3.1-GFP-ex1 (EX1 group),pcDNA3.1-GFP-ex3 (EX3 group)) and wild type recombinant expression plasmid of GLA (pcDNA3.1-GFP-GLA,GLA group) were constructed.Hela cell line (control group) was transiently transfected with recombinant expression plasmid according to lipofectin transfection.The relative gene expression of Beclin-1 was measured with real-time PCR,and protein expression level of LC3-Ⅱ/LC3-Ⅰ,Beclin-1 and P62/SQSTM1 was examined by Western blotting.Results The LC3 protein values of groups EX1,EX3,GLA and control were 1.495 ± 0.064,1.490 ± 0.020,1.285 ± 0.021,1.260 ± 0.042,respectively;P62/ SQSTM1 values were 0.555 ± 0.086,0.480 ± 0.084,0.785 ± 0.439,0.980 ± 0.278,respectively;Beclin-1 mRNA 2-△Ct values were 0.011 ±0.003,0.008 ±0.002,0.005 ±0.001,0.003 ±0.001,respectively;Beclin-1 protein values were 1.178 ±0.098,1.209 ±0.092,0.931 ±0.100,0.796 ±0.184,respectively.Compared with the wide type group,the level of LC3-Ⅱ/LC3-Ⅰ protein was significantly higher in the mutant type groups(t =5.118,4.984;P =0.007,0.008),though no statistically significant difference was found in the expression levels of P62/SQSTM1 (t =1.052,1.400;P =0.323,0.199).Besides,the expression levels of Beclin-1 mRNA (t =3.800,2.445;P =0.005,0.040) and protein (t =2.424,2.729;P =0.042,0.026) were significantly higher in the mutant type groups.Conclusions GLA gene mutation can induce cell autophagic dysfunction,and signaling pathway of autophagic activation may be Beclin-1 dependent.

Objective To report the clinical and peripheral neuropathological findings in two patients with autosomal recessive Charcot-Marie-Tooth disease 2K(AR-CMT2K).Methods Case one was a nine year-old girl.She had distal weakness of lower limbs for six years, with calf atrophy and contracture of Achilles tendon for three years.Case two was an eight year-old boy.He had distal weakness of lower limbs with contracture of Achilles tendon and calf muscle atrophy for three years, and proximal weakness of low limbs for two years.The motor nerve conduction velocities in median nerves were 48.1 m/s in case one and 47.6 m/s in case two.The compound motor action potential amplitude of median nerves decreased by 46% in case one and 69% in case two.Sural nerve biopsies and gene targeted next-generation sequencing were performed in both patients.Results Density of myelinated fibers was 8 407/mm2 in case one and 7 714/mm2 in case two.The ratio of myelinated fibers with diameter over 8 μm was 2.6% in case one and 0 in case two.Both patients had small regenerating cluster of myelinated fibers.Thin myelinated fibers appeared in case one.In case two, atypical onion bulb formations with focal folded myelin appeared, and electromicroscopy revealed mitochondrial aggregate in axons.Compound heterozygous mutations of ganglioside-induced differentiation associated protein 1 gene were detected in both patients, including c.767A>G(p.H256R) and c.466G>A (p.A156T) in case one and c.767A>G and 845G>A(p.R282H) in case two.Conclusions Contracture of Achilles tendon may appear in early childhood of AR-CMT2K patients.The main pathological changes in sural nerve are loss of large myelinated fibers, mitochondrial aggregate in axons and myelin abnormalities.

Objective To analyze the differences of the clinical and neuropathological features among the common Charcot-Marie-Tooth disease (CMT) subtypes.Methods There were 81 CMT patients confirmed by genetic testing from 2005 to 2015 in Department of Neurology,Peking University First Hospital,including 31 cases of CMT1A (38.3％),19 cases of CMTX1 (23.5％),16 cases of CMT2A2 (19.8％) and 15 cases of 9 rare types of CMT (1.2％-4.9％).We compared the onset age,duration,muscles weakness of legs,frequency of pes cavus,and main pathological changes of the sural nerve biopsy in 48 cases of the common CMT subtypes.Results The mean age of the onset was (12.00 ± 6.77) years in CMT1A patients,(11.81 ±4.65) years in CMTX1 patients and (5.00 ±2.68) years in CMT2A2 patients (Brown-Forsythe test,P =0.001).The duration was (12.00 ± 6.75) years in CMT1A patients,(8.50 ± 4.75) years in CMTX1 patients and (5.00 ± 2.73) years in CMT2A2 patients (Brown-Forsythe test,P =0.001).The muscle force of the dorsi flexors was Ⅳ (0,Ⅴ) in CMT1A patients,Ⅲ + (0,Ⅳ) in CMTX1 patients and 0 (0,Ⅳ) in CMT2A2 patients (H =11.359,P =0.020).The pes cavus appeared in 15/23 cases of CMT1A,10/16 cases of CMTX1 and 1/9 cases of CMT2A2 (Fisher test,P=0.017).The leukoencephalopathy appeared only in 3 cases of CMTX1 and the visual loss appeared only in 3 cases of CMT2A2.The onion-bulb formations of myelinated fibers appeared in 23/23 cases of CMT1 A,5/16 cases of CMTX1 and 2/9 cases of CMT2A2(Fisher test,P =0.000).The axonal regeneration appeared in 16/23 cases of CMT1A,16/16 cases of CMTX1 and 9/9 cases of CMT2A2 (x2 =7.666,P =0.016).There were significant differences among the three common CMT subtypes in the above parameters.Conclusions CMT1A,CMT2A2 and CMTX1 are the most common subtypes of CMT in the present study.For the clinical diagnosis,more attention should be paid to the onset of the disease,duration,muscles weakness,pes cavus,cerebral symptoms and visual loss.The present frequency of onion-bulb and the axonal regeneration of myelinated fibers help the different pathological diagnosis among them.