Inherited epidermolysis bullosa (EB) is a group of hereditary skin diseases characterized by increases in skin brittleness and being prone to skin blisters or erosions when the skin is exposed to slight friction or injury. At present, it is classified into four types, namely EB simplex, junctional EB, dystrophic EB and Kindler syndrome. An onion skinning approach is recommended for the diagnosis of inherited epedermolysis bullosa, in which, immunofluorescence mapping is performed firstly, and causative genetic loci are then determined through detection of relevant genes. In its treatment, skin care should be intensified, and medical or surgical treatment may be used to alleviate symptoms. Further development of cell?, protein?and gene?based therapies is expected to bring hope to patients via realization of timely prenatal diagnosis and avoidance of adverse consequences.

As one of active immune effector molecules,immunoglobulins play an important role in the pathogenesis,diagnosis and treatment of autoimmune bullous diseases.Human immunoglobulins can be divided into 5 categories,including IgG,IgA,IgM,IgD and IgE,and all of them participate in and play different roles in the pathogenesis of autoimmune bullous diseases except IgD.IgG can induce the formation of blisters by activating complements and leukocytes as well as by releasing proteolytic enzymes,and different subtypes of IgG differ in biological activities.IgA can cause granulocyte migration and lead to the formation of blisters and pustules.IgE is associated with urticaria-like erythema and eosinophil infiltration.IgM is usually seen in Brazilian pemphigus foliaceus.These findings may provide new ideas for the diagnosis and treatment of autoimmune bullous diseases.

Objective To study the risk factors of aortic arch calcification (AoAC) in maintenance hemodialysis (MHD) patients.Methods Retrospective analysis was performed in patients who accepted MHD ≥ 5 years.The clinical data were collected.The images of plan chest radiograph detected during followup period were reviewed by certain imaging physicians,and the AoAC score was calculated.Logistic regression model analysis was proceeded to study the risk factors of AoAC.The patients were divided into AoAC positive group (29 cases) and AoAC negative group (37 cases) respectively.The clinical parameters were compared between the 2 groups.Results Sixty-six patients (21 male,45 female) were selected.The year AoAC scores from the zeroth to fifth year were (0.013 40 ± 0.074 96),(0.018 90 ± 0.078 55),(0.118 50 ± 0.170 06),(0.157 00 ± 0.207 55),(0.166 00 ± 0.205 37),(0.175 50 ± 0.222 29) scores,progressively.There were significant differences in AoAC score from the third year compared with that of the zeroth year (Z =-3.984,-4.021,-4.017;P ＜ 0.01).Logistic regression model analysis result showed that only the age of dialysis beginning was the risk factor for AoAC (relative risk 1.177,regression coefficient 0.163,P =0.002).The age of dialysis beginning,rate of diabetic nephropathy,serum calcium-phosphate product were significantly higher in AoAC positive group than that in AoAC negative group [(59.5 ± 12.6) years vs.(52.9 ± 13.2) years,27.6％(8/29) vs.2.7％(1/37),(5.81 ± 1.63) mmol2/L2 vs.(4.13 ± 0.86) mmol2/L2].Conclusions The AoAC will aggravate with the increased duration of dialysis in MHD patients.The age of dialysis beginning is the risk factor of AoAC.

Objective To evaluate the effect of transcutaneous electrical acupoint stimulation (TEAS) on sedative efficacy during induction of anesthesia with propofol.Methods Patients,of ASA physical status Ⅰ or Ⅱ,aged 21-69 yr,scheduled for elective thyroid surgery,were randomly divided into 2 groups using a random number table:control group (group C) and TEAS group.TEAS was applied to bilateral Hegu and Neiguan acupoints for 30 min.The frequency was 2/100 Hz,wave length was 0.2-0.6 ms and the intensity was maintained at about 8-12 mA according to the current that could be tolerated.Induction of anesthesia was started at the end of TEAS.Sequential method was used to determine the median effective target plasma concentration (EC50) of propofol causing loss of consciousness in each group.The initial target plasma concentration of propofol was 3 μg/ml.BIS value was recorded every 30 s within 5-8 min after loss of consciousness and the average BIS value was calculated.It was considered to be positive response when the average BIS value was below 50.The target concentration of propofol was decreased/increased by 0.3 μg/ml in the next patient.Results EC50 of propofol causing loss of consciousness was 3.08 μg/ml in group TEAS,and 3.70 μg/ml in group C,and there was significant difference between the two groups.Conclusion TEAS can enhance the sedative efficacy during induction of anesthesia with propofol in the patients.

Objective To compare the graft function of 2 methods of kidney harvesting,the modified method of combined liver and kidney procurement and rapid en bloc kidney procurement. Methods The clinical data of 220 cadaveric renal transplantation recipients were collected(12 months follow-up),174 cases from en bloc kidney procurement and 46 from combined liver and kidney procurement.The average harvesting time,the incidence of renal vein injury,1 year kidney survival and incidence of acute rejection were compared between the two methods.Results In average harvesting time and incidence of renal vein injury,the en bloc kidney procurement were better than the method of combined liver and kidney procurement.However,the method of combined liver and kidney procurement was better than the en bloc kidney procurement in 1 year kidney survival,1 year incidence of acute rejection and average warm ischemia time.There was no difference in 1 year survival of patients and 24 h,1 week and 1 year graft function after transplantation. Conclusion Although the method of combined liver and kidney procurement is better than the en bloc kidney procurement in 1 year kidney survival and 1 year incidence of acute rejection,there is no difference between the 2 methods in 1 year survival of patients and graft function after transplantation.

Objective To study the effects of russel viper venom X(RVV X)on blood coagulation protein.Methods We divide diluted protein into control and RVV-X groups,then use chromogenic substract assay to detect the activation effect of RVV-Ⅹ on coagulation factor Ⅶ,Ⅸ,Ⅹ and antithrombin,plasminogen,with or without activator.Results In RVV-Ⅹ group,the coagulation factor Ⅶ, Ⅸ and plasminogen displayed weakly enhanced chromogenesis,all P

Objective To investigate the efficacy of centrally fixed eyeball for assessment of the depth of anesthesia in premature infants undergoing outpatient fundus examination. Methods Fifty eight premature infants undergoing examination of fundus of eyes were enrolled in this study. Their gestational age (from the first day of last menstruation period to birth) + after birth age (from birth to the day when examination of fundus of eyes was performed) = 44-64 weeks. The patients were randomly divided into 2 groups: Ⅰ group body movement (group M, n = 27) and Ⅱ group centrally fixed eyeball (group E, n = 31). Anesthesia was induced and maintained with isoflurane inhalation. The patients were breathing spontaneously. The eyelids were kept open with speculum after induction of anesthesia. The EC50 of sevoflurane concentration which could inhibit body movement or make eyeballs centrally fixed was determined by up-and-down sequential experiment. The initial isoflurane concentration was 3% in both groups. Each time the isoflurane concentration was increased/decreased by 0.5 %. 95 % confidence interval (CI) was calculated. The lowest SpO2, respiratory rate and coughing during maintenance of anesthesia were recorded. Results The EC50 of sevoflurane (95% CI) was 2.9% (2.2%-3.6%) in group M and 3.4%(2.6%-4.6%) in group E. Examination was successfully completed in all patients. No respiratory depression and coughing occurred during examination and no vomiting and coughing were observed during feeding at 1 h after recovery from anesthesia. No body movement occurred in 15 patients whose eyeballs were centrally fixed in group E. Conclusion Centrally fixed eyeball can be used as sign of appropriate depth of anesthesia for fundus examination in premature infants.

Objective To detect the expression of CD133 in hepatic cell lines SMMC7721 and bcl-7402, and to investigate the possibility of CD133 as the surface marker of liver cancer stem cells. Methods The cell cycle and expression of CD133 in hepatic cell lines SMMC7721 and bcl-7402 were detected by flow eytometry. Magnetic cell sorting was used to isolate CD133-positive and CD133-negative cells. The differences in morphology, proliferation and differentiation between CD133-positive and CD133-negative cells were observed and analyzed by one-way ANOVA and u test. Results The percentages of CD133-positive cells in SMMC7721 and bcl-7402 cell lines were 0.7% -1.0% and 1.7% -8.9%, respectively. The percentages of CD133-positive cells in G0>/G1> phase in the 2 cell lines were 85.3% and 89.4%, which were significantly higher than CD133-negative cells and unsorted cells (F = 14.49, 38.84, P <0.05). The in vitro proliferation capability of CD133-positive cells was greater than that of CD133-negative cells and unsorted cells, especially during day 1-3 and day 5-7 (F =49.32,784.04, 89.91, 152.83, P < 0. 05). During the cultivation, the proportion of the CD133-positive cells decreased as time passed by, and the proportion of CD133-positive cells was nearly the same as unsorted cells on day 15 (u =O. 271, P <0.05). Conclusions CD133-positive ceils have strong capability of proliferation and differentiation in SMMC7721 and bcl-7402 cell lines in vitro. CD133 is one of the surface markers of liver cancer stem cells.

Objective To investigate the change and its related risk factors of cognitive function of patients with Parkinson's disease(PD).Methods The cognitive function of 70 idiopathic PD patients were assessed with Montreal cognitive assessment scale (MoCA),and other PD related neuropsychological test batteries were used to evaluate their movement symptoms and non-movement symptoms such as depression and anxiety.Patients' personal information were collected at the same time.They were reassessed after 2 years.Results As the disease progressed,the MoCA score of the PD patients significantly reduced from (24.79±4.07) points to (21.69±5.22) points (P＜0.01).Seven subdomains of MoCA were attenuated,and 5 of them reached statistical significance.Motor subtype was a main predictor of the outcome of the cognitive impairment in PD.Scores of MoCA total score,naming,language,abstract and directional domains reduced more notably in patients with postural instability gait difficulty(PIGD) than those in the tremor dominant(TD) patients.Conclusion Cognitive function of PD patients decreases obviously after 2 years.The patients' cognitive impairment should be identified and intervened as soon as possible,especially the patients with PIGD.

Objective To investigate the effect of dexmedetomidine on minimal induced dose of propofol and cardiovascular responses to tracheal intubation.Methods A double-blind randomized controlled trial was conducted.Sixty patients who underwent elective laparoscopic surgery were randomly divided into the dexmedetomidine group (treatment group) and control group.Patients in treatment group were given dexmedetomidine at dose of 0.1 μg/kg · min for 10 min before anesthesia induction,and then infused with 0.4 μg/kg · h until the end.Sodium chloride injection was infused at the same rate in control group.After 10 minutes from the start of dexmedetomidine or sodium chloride injection,propofol was infused by 0.4 mg/kg · min.The dosage of propofol used was recorded when eyelash reflex disappearing,and BIS value in 40 to 60.Propofol was infusion at the same rate,and laryngoscope was incubated at 2 minutes after fentanyl and rocuronium were given.The alertness/sedation (OAA/S scores),mean arterial pressure (MAP),heart rate,pluse oxygen saturation (SpO2) and BIS values were recorded at baseline (before dexmedetomidine or sodium chloride injection infusion),5 and 10 minutes after dexmedetomidine or sodium chloride injection infusion,at the time of eyelash reflex disappearing,before endotracheal intubation,placing the laryngoscope,1,3 and 5 min after intubation.Results OAA/S scores and BIS values in the treatment group were significantly lower than that of control group at 5 minutes and 10 minutes after the dexmedetomidine or sodium chloride injection infusion(P ＜0.05).MAP in treatment group was higher than that of control group at the time of eyelash reflex disappearing,before endotracheal intubation,placing the laryngoscope,1,3 and 5 minutes after intubation (P ＜0.05).Heart rate in treatment group was lower than that of control group at 5 and 10 minutes after dexmedetomidine or sodium chloride injection infusion and before the endotracheal intubation(P ＜ 0.05).There was no significant difference in terms of SpO2 between two groups (P ＞ 0.05).When patients consciousness disappeared and BIS values were in 40-60,the minimum induced dose of propofol was (88.00 ± 25.91) mg in treatment group and (117.33 ± 25.45) mg in control group.The dosage of propofol treatment group was obviously less than control group (t =4.423,P ＜ 0.05).Conclusion Dexmedetomidine reduces the minimum induced dose of propofol while maintaining more stable hemodynamic changes during anesthesia induction.However,there has no obvious inhibition effect on cardiovascular response to tracheal intubation.