Objective To explore clinic,imageology and pathological characteristics of twin brothers with adrenoleukodystrophy(ADL).Methods Clinical data of twin brothers with ALD and pathological data of one case were analyzed retrospectively.Results Clinical manitestation of elder brother was cerebral ADL,T1-weighted of MRI with low intensity lesion and T2-weighted with high intensity lesion were shown widely in the parietooccipital and postero-corpus callosum white matter.The pathological changes were myelinopothy diffused in the parietooccipital white matter,but U fibber was maintained in the subcortex.Clinical manitestation of the young brother was shown spinal damage.His cerebral MRI was normal.Spinal MRI had shown spinal cord thinning,line-like equal signal was found in the periphery of the lesion.He might be juvenile adrenomyeloneuropathy.Conclusions Although the twin brothers are both suffer from adrenoleukodystrophy,their clinical manifestation,MRI and pathological changes are different.

Objective To observe pathological features of liver injury induced by Helicobacter hepaticus ( H.hepaticus) and the difference between male and female BALB/cCr mice.Methods Fifty SPF-class BALB/cCr mice (25 males and 25 females) were administrated by gavage with 0.2 mL bacterial suspension (1 ×108 CUF/ml) of H.hepaticus standard strain ATCC 51450 for 3 times with 48 h intervals. The control group (25 males and 25 females) received same volume of phosphate buffered saline (PBS). Mice were sacrificed in batches ( n=5) after fasting for 12 h at month 1, 3, 6, 9 and 12.Enzyme linked immunosorbent assay ( ELISA) was used to determine the serum level of H.hepaticus IgG antibodies.Liver tissue samples were taken for histopathology examiantion, micro-aerobic bacteria isolation, culture and identification.t test was used to analyze the differences in serum levels of H.hepaticus IgG antibody and liver histopathologic scores between different time points and groups. Results The seroprevalance of H. hepaticus-IgG antibody in male BALB/cCr mice infected with H.hepaticus were all positive, peaked at 6 month, and then gradually declined.H.hepaticus-IgG antibody levels at 3, 6, 9 and 12 month were higher than that at 1 month (t=2.828, 4.300, 3.536 and 4.500, P<0.05).Only one female BALB/cCr mouse infected with H.hepaticus was positive for H.hepaticus-IgG antibody at 9 and 12 month.H.hepaticus colonization in liver was observed in male BALB/cCr mice infected with H.hepaticus since month 3, while it was not observed in female BALB/cCr mice.Compared with femle mice, the histopathologic scores of liver in male mice infected were much higher at all time points (t=2.598,7.770,7.987,10.850 and 12.260, P<0.05 or P<0.01) .Liver histopathologic scores increased in male mice as infection time extended within 6 months (t=4.949, P<0.01), but not during 6 and 12 month (t=1.052, P>0.05).Conclusion Compared with female mice, H.hepaticus colonization and histopathologic changes in liver are more significant in male BALB/cCr mice infected with H.hepaticus, and the histological scores are increased as infection time extended.

Objective To verify the relationship between qi-yin deficiency syndrome and the etiology and diagnostic information on type 2 diabetes mellitus.Methods Data of 185 cases of qi-yin deficiency syndrome of type 2 diabetes and 351 cases of other syndromes at TCM Hospital of Hongqiao District in Tianjin and Metabolic Disease Hospital of Tianjin Medical University were randomly collected. Etiology and diagnostic information of qi-yin deficiency syndrome were screened by the likelihood radio of forward step method by using Logistic regression analysis. On this basis, the relationship between qi-yin deficiency syndrome and the etiology and diagnostic information was verified by using maximum likelihood estimation method of the structural equation model.Results The goodness of fit index of the model (GFI) was 0.909; root mean square residual (RMR) of that was 0.071; comparative fit index (CFI) of that was 0.942, which suggested that the fitting effect of the model was good. The reasonable interpretation of the relationship between qi-yin deficiency syndrome and the etiology and diagnostic information was given. Conclusion From the aspect of single syndrome factors, the relationship between qi-yin deficiency syndrome and the etiology and diagnostic information on type 2 diabetes mellitus is verified.

Objective To investigate the clinical value of preventive analgesia in patients with radical gastrectomy for gastric cancer.Methods The clinical data of 161 patients undergoing radical gastrectomy for gastric cancer at the Xijing Hospital from July 2012 to June 2013 were prospectively analyzed.A single-blind,randomized,controlled study was performed in the eligibe patients who were randomly divided into the preventive analgesia group and the control group based on a random number table.All the patients received major gastrectomy + Billroth Ⅱ anastomy or total gastrectomy + esophagojejunastomy,and then they received vein combined anesthesia and tracheal intubation.In the preventive analgesia group,10 mg dexamethasone and 200 mg parecoxib were administered by intravenous infusion before operation,7-8 mL ropivacaine (0.5％) were injected at the incisional site and transversus abdominis infiltration at open and close surgery and celecoxib was taken orally for 3 days.In the control group,vein analgesia pump was used after operation.The visual analogue scale (VAS),proportion of patients with postoperative out-off-bed activity time ＞ 8 hours,time of flatus,time of defecation and duration of hospital stay were recorded between the 2 groups.The measurement data with normal distribution were presented as x ± s.The comparison between groups was evaluated with the t test and repeated measures ANOVA.The data with skew distrubution and ordinal data were analyzed by the analysis of variance,and the count data were analyzed using the chi-square test.Results All the 161 patients who were screened for eligibility were randomly divided into the preventive analgesia group (87 patients) and the control group (74 patients).The scores of the VAS at postoperative day 1,2 and 3 were 2.8 ± 0.6,2.6 ± 0.4 and 1.8 ± 0.4 in the preventive analgesia group,and 5.3 ± 0.5,4.2 ± 0.6 and 2.4 ± 0.3 in the control group,with a significant difference between the 2 groups (F =4.25,P ＜ 0.05).The proportion of patients with postoperative first abmulation time ＞ 8 hours at postoperative day 1,2 and 3 were 8,17 and 20 in the preventive analgesia group,and 0,3 and 11 in the control group,with a significant difference between the 2 groups (x2 =7.60,10.26,3.16,P ＜ 0.05).The time of flatus,time of defecation and duration of hospital stay were (51 ± 24) hours,(61 ± 24) hours and (5.5 ± 3.0) days in the preventive analgesia group,compared with (71 ± 23) hours,(83 ± 30) hours and (6.3 ± 2.1) days in the control group,showing a significant difference between the 2 groups (t =5.32,5.04,0.17,P ＜ 0.05).All the patients in the 2 groups normally survived after surgery without respiratory depression,incision infection and adverse drug reaction.Conclusions Preventive analgesia in the perioperative treatment of patients with radical gastrectomy for gastric cancer could relieve the postoperative pain of patients,improve the bowel function and enhance the recovery of patients.Registry This study was registered with the Chinese Clinical Trial Registry with the registry number of ChiCTRTRC-11001440.

Objective To study the MRI findings of sporadic Creutzfeldt-Jakob disease and its clinical relations. Methods MRI of 10 cases CJD patients were examined 2-12 months after the onset. 6 cases were diagnosed using cerebral biopsy, 8 cases received CSF analysis for 14-3-3 protein, 8 cases showed special changes of electroencephalogram, PrP gene of 9 cases were analyzed. Results Symmetric bilateral high signals were observed in caudate nucleus and Putamen in T 2-weighted imaging and Flair imaging in 5 cases, but the pallidum and thalamus were normal. No changes were found in T1-weighted imaging. 2 cases showed brain atrophy, 1 case showed mild lacunar infarction,and the other 2 were normal. Conclusions Abnormal signals in basal ganglia of 4 patients of 129Met/Met homozygote occurred after 2.5 months averagely, they survived for 10.5 months at average. 1 patient of 129 Met/Val heterozygote showed abnormal signals in basal ganglia after 12 months, and survived for 16 months. The mean duration of patient with abnormal signals in basal ganglia (12.2 months) is longer than those without changes in basal ganglia (5.5 months). Symmetric high signal in bilateral caudate nucleus and Putamen is an important imaging feature of sCJD. It might be served as a diagnostic index in some circumstances.

Objective To detect point mutations of the PRNP in 10 sporadic Creutzfeldt-Jakob disease (CJD) patients. Methods Priori protein gene open reading frame was amplified by PCR of genomic DNA extracted from peripheral blood leukocytes. Products were sequenced and digested with restriction endonuc lease Nsp I to check the phenotype at codon 129. Results Two CJD patients were confirmed at autopsy. One full sequencing of the PRNP open reading frame revealed normal, but the other revealed a single novel mutation consisting of a cytosine-to-guanine substitution at nucleotide 729, causing asparagine to replace glutamic acid at codon 211. Among 8 probable CJD patients, 2 full sequencing of the PRNP open reading frame revealed anadenine-to-guanine substitution at nucleotide 751, causing lysine to replace glutamic acid at codon 219. The patients were methionine homozygosity at codon 129. Conclusions The E211D mutation was identified in a confirmed CJD patient. The novel point mutation might be associated with familial CJD. However, E219K identified in 2 possible CJD patients was included in polymorphism of the PRNP as well as M129V. Analysis of PRNP plays an important role for diagnose of familial priori disease.

Objective To investigate the molecular mechanism of calreticulin ( CRT) transcription induced by HBV and its viral proteins. Methods The human hepatocellular cell line, HepG2, was trans-fected with pHBV1. 3 and eukaryotic expression plasmids of HBV viral proteins, respectively. The expres-sion of CRT was measured after transfection. A reporter plasmid of CRT promoter was constructed to analyze the induction of CRT promoter by pHBV1. 3 and HBV viral proteins. Furthermore, two truncated and one C/EBPα site deficient mutants were constructed to evaluate the regulatory effects of HBx on CRT promoter. Fi-nally, HepG2 cells were transfected with HBx expression plasmids and the cellular localization of C/EBPαwas analyzed. Results In this study, pHBV1. 3 could significantly up-regulate the expression of CRT at mRNA and protein levels as well as enhancing the activity of CRT promoter. Among the seven HBV viral proteins, HBx could enhance the activity of CRT promoter and the expression of CRT at mRNA and protein levels. HBx could not induce the transcription of CRT when the C/EBPα binding site was deleted from the CRT promoter. The expression of HBx could promote the nuclear translocation of C/EBPα. Conclusion HBV and its viral protein HBx could up-regulate the CRT expression at transcriptional level. The transcrip-tional factor C/EBPα played a critical role in HBx-induced transcriptional activation of CRT.

Objective To investigate the outcomes of patients undergoing off-pump coronary artery bypass grafting (OPCABG) under morphine-fentanyl combined anesthesia. MethodsSeventy-two patients aged 41-64 yr undergoing OPCABG were randomly divided into 2 groups (n = 36 each): Ⅰ group morphine + fentanyl (group MF) and Ⅱ group fentanyl (group F). Anesthesia was induced with midazolam, etomidate, and vecuronium.Fentanyl 10-20 μg/kg was given iv when needed. The patients were mechanically ventilated after tracheal intubation. Anesthesia was maintsined with inhalation of 0.5%-2.0% isoflurane and intermittent iv boluses of vecuronium in both groups. Morphine 0.15 mg·kg-1·h-1 + fentanyl 8 μg·kg-1·h-1 were infused during operation in group MF, while in group F fentanyl 10 μg·kg-1·h-1 was infused. Morphine 0.75 mg/h was infused in group MF or fentanyl 10 μg/h in group F for postoperative analgesia after extubation. Recovery from anesthesia was assessed using a nine point (QoR) questionnaire. Pain was measured with VAS score and sedation with Ramsay sedation score ( 1 = fully awake, 6 = asleep, no response to verbal stimulus). The postoperative complications were recorded. ResultsQoR scores were significantly higher in group MF and in group F. The incidence of postoperative febrile reaction was significantly reduced in group MF. There was no significant difference in Ramsay sedation score and other postoperative complications between the 2 groups. ConclusionMorphine-fentanyl combined anesthesia is more beneficial for the prognosis in patients undergoing OPCABG compared with fentanyl combined anesthesia.

ObjectiveTo investigate mutations in exons 19, 20 and 21 of epidermal growth factor receptor (EGFR) gene in elderly patients with non-small cell lung cancer (NSCLC). MethodsEGFR gene mutations in exons 19, 20 and 21 were detected by nested PCR amplification and DNA sequencing in 46 elderly patients with non-small cell lung cancer. The relationship between mutations and clinical characteristics of these patients was analyzed. ResultsEGFR gene mutations were found in 56.5% (26/46) patients and 41.3% (19/46)were non-silent mutations. Mutation of exon 19 was detected in 6 cases (13.0%), mutation of exon 20 in 13 cases(28.2%) and that of exon 21 in 14 cases (30.4%). Seven patients among them had double mutations and the rest only had a single mutation. The incidence of EGFR gene mutations was higher in non-smokers than in smokers(P＜ 0.01). Higher EGFR mutation rate in exon 19, 20 and 21 were found in patients with clinical benefit who were treated with tyrosine kinase inhibitor(TKl)(P＜0.05). There was no difference in EGFR mutation rate between 60～69 age group and 70～85 age group. ConclusionsThe data suggest that the characteristics of EGFR gene mutations in elderly patients with NSCLC is the same as in the general NSCLC patients. The forecast informations of TKI treatment can be obtained by gene detection in elderly NSCLC patients.

Objective 　To investigate the neuropathological changes of central nervous system in Guillain-Barré syndrom. Methods　Brain, spinal cord and sciatic nerve were obtained from 22 cases of Guillain-Barré syndrome. Eight cases were examined by general autopsy, 14 cases were examined by limited autopsy. HE, KB, Bielschowsky, Weil and Sudan Ⅲ　staining were carried out, the sections were observed by light microscopy. Results　1.Cerebral superficial veins congested, widening of the cortical sulci, narrowed gyri and mild cerebellar tonsillar hernia were present. 2. Majority of cerbral neurons presented an ischemic changes. Slightly loss of hippocampal pyramidal neurons were found. There was chromatolysis of motor neurons of brain stem. Lymphocytic infiltration around the small vessels occurred in the pons and medullary oblongata in 8 cases. Focal demyelination was noted in pons and frontal white matter in 2 cases. Loss of Purkinje cells and appearance of glial nodules were observed in molecular layer of cerebellum. 3. Swellin, central chromatolysis and eccentric nuclei of anterior horn cells appeared in 16 cases, which were pronounced in cervical and lumbal segment of spinal cord. Vaculated neuroplasma and lymphacytic infiltrition could be seen. 4. Segmental demyelination and lymphocytic infiltration were the main neuropathological changes observed in 20 cases. There were two other cases in which the axon were severely involved, which showed swelling and breakdown of axons and as well as axonal bulbs. Conclusions　1. Lymphocytic infiltration in brain stem and spinal cord were in continuousness of pathological changes of peripheral nerves. 2. Finding of glial nodules suggested that there was possibility of infection of neurotropic virus. 3. Occurence of focal demyelination in cerebrum and brain stem indicated that Guillain-Barré syndrome may have combined involvement of central and peripheral nervous system.