The prevalence of 1X type ranks the second among Charcot-Marie-Tooth disease (CMT). Mutations of GJB1 gene causes abnormality of Connexin32's structure and function, which leads to the defect of the intercellular passage, and at last Results in CMT1X. This article reviewed the typical clinical features and the advance of molecular genetics of CMT1X, and summarized the pathogenic researches.

Objective To investigate the protective effect of sodium butyrate on brain injury induced by pneumolysin of infantile rats.Methods Ninety-six normal healthy 1-month-old Spragne-Dawley (SD) rats were randomly divided into three groups,including pneumolysin (PLY) group (n =32),in which rat was injected PLY via external carotid; Normal saline (NS) group (n =32),injected NS via external carotid; sodium butyrate (SB) group (n =32),after injecting PLY,immediately administrated SB via venous.In the injection the 24th h and 48th h,superior vena cava blood was taken,and the animals were sacrificed,and brain tissue samples were prepared.The brain water content (BWC) was recorded by measuring both wet and dry weight,the Evans blue (EB) level was measured by the formamide method.The serum levels of high mobility group protein B1 (HMGB1) and nuclear factor kappa B (NF-κB) were measured by enzyme-linked immunosorbent assay (ELISA).Results In PLY group,brain tissue BWC,EB level,and the blood level of HMGB1 and NF-κB were increased significantly compared with the NS group at each time point,the difference was statistically significant (P ＜ 0.05).These indices were lower in the SB group compared with PLY group,the difference was statistically significant (P ＜ 0.05).The positive correlation was gotten between HMGB1 and NF-κB,BWC,EB levels in the PLY group and SB group (r =0.817 ～0.917,P ＜ 0.05).Conclusions SB has neuroprotective effect in brain injury induced by PLY,which maybe relevant to inhibition of NF-κB activation and suppression of HMGB1 expression.

Objective To investigate the change of serum HMGB1 level and its clinical significance in children with sepsis.Methods Serum HMGB1 and procalcitonin (PCT) levels were determined in 30 healthy individuals and 46 children with sepsis,and the acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) scoring system was evaluated in children with sepsis.These indexes were also collected on the third and fifth days.The correlations between serum HMGB1 level and serum PCT level as well as APACHE Ⅱ score were analyzed.Results Serum HMGB1 levels in children with sepsis on the first day,the third day and the fifth day were significantly higher than those in control group,these differences were statistically significant [(26.28 ± 1.54) ng/ml,(20.32 ± 1.29) ng/ml,(12.84 ± 1.06) ng/ml vs (1.52 ± 0.29) ng/ml,P ＜0.05],HMGB1 levels among three time points were also significantly different(P ＜0.05).The serum HMGB 1 levels were positively correlated with the PCT levels (r =0.931,P ＜ 0.05) and APACHE Ⅱ score (r =0.915,P ＜ 0.05).Conclusion Serum HMGB1 level is obviously elevated in children with sepsis,and the level of HMGB1 can reflect the severity of sepsis.

Objective Frontal resting EEG asymmetry was used to evaluate the gender effect of emotion regulation.Methods The resting EEG was recorded in 30 healthy volunteers (19-26 years old) by the guide 40 amplifier,and off-line analysis on EEG data were conducted by scan4.3 software.The statistical analysis were performed using SPSS 17.0.Results In normal human,frontal resting EEG alpha power was statistically significant(F=4.918,P =0.035).Asymmetry and gender interaction group (F =0.668,P =0.421),electrode point and gender interaction group (F =0.283,P =0.756),electrode point and asymmetry interaction group (F =1.418,P =0.260),electrode point,asymmetry and gender interaction group (F =1.164,P =0.327) had no statistical significance (P ＞ 0.05).The comparison of male and female asymmetry degree of each electrode point (F4/F3 group t =0.465,F8/F7 groupt=0.809,FP2/FP1 group t=1.542) had no statistical significance (P＞0.05).Conclusion Normal human brain hemispheres exist asymmetry.The asymmetry does not exists gender effect.In non-task state,frontal resting EEG alpha power asymmetry can not be recognized as the indicator of emotion regulation ability.

Objective To report clinical and pathological features in a Chinese family with CharcotMarie-Tooth disease type 2A2 (CMT2A2). Methods There were 5 patients (2 male and 3 female) in a family with an autosomal dominant inheritance pattern. The index case was a 36 years old woman. She developed progressive distal limb weakness at the age of 6, with pescavus at the age of 8. Other 4 family members presented with similar symptoms between the age of 3-7. Physical examination showed distal limb weakness and wasting, loss of sensory and contracture in all of them. Nerve conduction velocity revealed non-potential in several motor and sensory nerves in the proband and her son. Sural nerve biopsy was performed on the proband. The sequence of MFN2 gene was analyzed in DNA from 5 patients and 3 asymptomatic members. Results Sural nerve biopsy revealed severe loss of myelinated fibers with few regenerating clusters. Electron microscopy revealed aggregation of mitochondrian in the axons. A R94W mutation in MFN2 gene has been identified in 5 patients, but not in unaffected members. Conclusions We confirmed Chinese CMT2A2. Absent of regenerating cluster in the nerve indicated that MFN2 mutation predominantly resulted in lesions in the neurons.

Three hundred and nine community health service (CHS) practitioners were selected with randomized cluster sampling for survey with the job satisfaction questionnaire-occupational stress indicator (OSI).Degree of the job satisfaction was (68 ± 12)%.Position, work experience, income,feeling of recognition, degree of motivation, the scope for career development, the space for personal ambition and the relationship between salary and work load were potential influencing factors of job satisfaction to CHS practitioners.The authors suggest to establish the dynamic mechanisms of satisfaction study and to formulate comprehensive and effective incentive measures for CHS practitioners.

Objective:To explore the effects of linagliptin on blood glucose, islet function and liver stiffness measurement (LSM) in patients with non-alcoholic fatty liver disease (NAFLD) and type 2 diabetes mellitus (T2DM).Methods:During the period from January 2019 to April 2020, 102 patients with NAFLD and T2DM in the Second Affiliated Hospital of Suzhou University were enrolled and divided into study group and control group according to different treatment methods, with 51 cases in each group. The control group was treated with metformin, while study group was treated with linagliptin and metformin. The clinical curative effect on fatty liver was observed and compared between the two groups. The levels of fasting blood glucose (FBG), glycated hemoglobin (HbA 1c), homeostasis model assessment of insulin resistance (HOMA-IR), homeostasis model assessment of islet insulin cell function index β (HOMA-β), alanine aminotransferase (ALT), serum aspartate aminotransferase (AST), glutamyl transpeptidase (GGT) and LSM before and after treatment were compared between the two groups. The occurrence of adverse reactions during treatment in both groups was recorded. Results:The total response rate of fatty liver treatment in study group was significantly higher than that in control group: 96.1% (49/51) vs. 82.4% (42/51), and there was statistical difference ( P<0.05). After treatment, levels of FBG, HbA 1c, HOMA-IR, serum ALT, AST, GGT and LSM in study group were significantly lower than those in control group: (7.1 ± 1.0) mmol/L vs. (7.9 ± 0.9) mmol/L, (7.5 ± 0.7)% vs. (7.9 ± 1.0)%, 3.2 ± 0.2 vs. 4.7 ± 0.3, (56.7 ± 10.4) U/L vs. (62.8 ± 8.2) U/L, (73.2 ± 6.8) U/L vs. (81.1 ± 6.7) U/L, (56.4 ± 10.2) U/L vs. (62.3 ± 8.1) U/L, (10.5 ± 3.3) kPa vs. (13.4 ± 1.6), the level of HOMA-β was significantly higher than that in control group: 48.5 ± 8.3 vs. 41.2 ± 7.1, and there were statistical differences ( P<0.05). The incidence of adverse reactions during treatment was low in both groups, and the difference was not statistically significant between the two groups ( P>0.05). Conclusions:Linagliptin can improve clinical curative effect on fatty liver in patients with NAFLD and T2DM, control blood glucose level, and improve islet function, liver function and liver fibrosis, with higher medication safety.

Objective To investigate the diffevential diagnostic value of preinvasive and invasive lung adenocarcinoma (including minimally invasive adenocarcinoma and invasive adenocarcinoma) presented as pure ground-glass nodules(pGGN) by CT.Methods One hundred and fifty-six cases of pGGN verified by operative pathology were retrospectively analyzed,including 58 ca ses of preinvasive adenocarcinoma and 98 cases of invasive adenocarcinoma(TNM staging were T1N0M0).The CT features and sex were statistically processed.The difference between the CT features and sex were performed by thex2 test.The ROC curve of lesion focus size was drawn.Results Statistically significant differences were found in the lesion shape,vacuole sign,air bronchogram,blood vessel through,tumor-lung interface and vascular cluster sign between the two groups(all P＜0.05).The ROC curve showed that the accuracy rate of invasive adenocarcinoma was 75.0％ when the size of the pGGN lesions was larger than 15.35 mm.Conclusion The lesion size,shape,vacuole sign,air bronchogram,blood vessels through and vascular cluster sign have some predictive value.

Objective To explore the relationship between job burnout and personality characteristics of prison police,and provide a basis for prevention and intervention of prison police burnout.Methods Using Maslach burnout inventory general survey(MBI-GS) and Catteii the sixteen factor questionnaire(16PF) to investigate 217 prison police in Beijing.Results The overall prevalence of burnout syndrome among prison police was 64.7％.The prevalence of emotional exhaustion(11.09±5.86),depersonalization(6.38±4.81) and diminished personal accomplishment(17.96± 2.07) was 46.5％,25.6％,33％ respectively.Compared with the national norm of 16PF,the 10 factors of warmth(t=-2.01,P=0.007),reasoning(t=9.22,P=0.000),dominance(t=6.46,P=0.000),liveliness(t=4.01,P=0.000),social boldness(t=-2.00,P=0.047),sensitivity(t=-7.80,P=0.000),abstractedness (t=-2.89,P=0.004),privateness (t=4.35,P=0.000),perfectionism(t=3.19,P=0.002) were significant differences.The emotional exhaustion was significantly related to emotional stability,dominance,liveliness,social boldness and tension.The depersonalization was significantly related to emotional stability,abstractedness and tension.The diminished personal accomplishment was significantly related to self-reliance.Conclusion Prison police burnout situation is more serious,and personality characteristics has a significant impact on job burnout.

Objective To explore pre-attentive information processing in undergraduate students with major depressive disorder(MDD) indexed by auditory mismatch negativity (MMN) and provide scientific theory basis for the diagnosis of MDD.Methods In the deviant-standard reverse oddball paradigm with deviant stimulus presentation probability of 20％,duration auditory MMN was obtained in 17 MDD university students and 17 gender-/age-matched healthy undergraduate students.All data were analyzed by statistics means of repeated measurement of F test.Results The amplitudes of 150 ms MMN over the frontal-central area were smaller in MDD undergraduate students((-0.34± 1.24) μV) than those in healthy undergraduate students ((-1.07± 1.55) μV,F=24.318,P=0.000).The 50 ms MMN did not differ between MDD students ((-1.96± 1.14) μV) and healthy undergraduate students ((-1.23 ± 1.97) μV,F =0.089,P =0.767).Conclusion Pre-attentive auditory information processing is impaired in MDD undergraduate students.