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 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.

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 observe the histological changes of lamina extema cranii at both the graft and donor areas after having been grafted in animal experiment. Methods; 4 mini pigs aged from 8 to 12 months were selected. Part of the outer table of skull (2.5 cm × 1.0 cm) was decorticated and was divided equally. Both were implanted on each side of the snouts of animals. 2 pigs were selected randomly and were killed 12 weeks postoperation. Each 1, 0 cm ×0. 5 cm specimen was harvested and histological study was performed. The other 2 animals were killed 24 weeks after operation. Gross inspection of the donor site was performed and histological study was done. Results; In the graft area; bone absorption was obvious 12 weeks postoperation, but bone matrix increased obviously at 24 weeks postoperation. Results of scanning electron microscope showed that interface between bone graft and recipient site was discernment and collagen type I was malaligned 12 weeks postoperation. Collagen fibers were in good order and good mineralization was found 24 weeks postoperation. In the donor area; small amounts of new bone formation could be seen and alignment of cemental line was chaotic 12 weeks postoperation. At 24 weeks, bone formation was obvious and alignment of haversian canal was regular. Scanning electron microscopy showed that structure of the tissue was uneven and disorder at 12 weeks postoperation. Collagen fibers were in good order and good mineralization was found 24 weeks postoperation. On gross view, raw surface of the donor site got complete healing, os-teanagenesis was incomplete and introcession could be observed in the local area. Conclusion; Histological remodeling of lamina exter-na cranii is complete at 24 weeks after having been grafted. The donor site can regenerate but morphologic introcession could be observed.

BACKGROUND: Pathological changes of the brain tissue in patients with dementia of frontal type(DFT) are still controversial. This paper brought forward the pathological alterative characteristics of brain tissue in DFT patients through one pathological case study of the brain tissue in one dead dementia patient.OBJECTIVE: To validate one uncommon neurodegenerative disease complicated with dementia, DFT.DESIGN: A case analysis.SETTING: Department of Neurology of the First Hospital of Jilin University METHODS: Brain anatomy, serials of histological staining and immunohistochemical staining for PrP, tau protein, etc. were performed after 3 hours since the death of one patient with progressive dementia.stainingfrontal lobes. EEG displayed a paroxysmal high-amplitude slow wave with and the brain atrophy was limited to frontal lobe and the temporal lobe loss of neurocyte companied with significant gliosis since the second layer; However, the pyramidal cell was relatively healthy. No abnormality was munohistochemical staining had negative reactions.CONCLUSION: This case was typical DFT. This type of dementia should be considered in future analysis of the neurodegenerative disease complicated with dementia.

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.

To investigate the application of seminar teaching on postgraduate education of ophthalmology,postgraduate from Grade 2008 to Grade 2010 were divided into experiment groups and control groups.Seminar teaching and conventional teaching were applied to two groups respectively.The performances of seminar teaching groups at each grade were better than the control groups ( P ＜ 0.05 ).Thus,seminar teaching can enhance theory and operation ability of postgraduate.

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 effect of continuous blood purification (CBP) on inflammatory factors and immune function in patients with sepsis,in order to provide reference for clinical treatment of sepsis.Methods:A total of 76 patients with severe sepsis,who were treated in ICU of Daqing Oilfield General Hospital from June 2014 to December 2016,were selected.The patients were divided into conventional group (n=36) and CBP group (n=40) according to the received treatment method.The patients in the conventional group were treated with targeted therapy and life support,while the patients in the CBP group were treated with CBP on the basis of targeted therapy.The levels of interleukin-6 (IL-6),interleukin-10 (IL-10),tumor necrosis factor-α (TNF-α) and C reactive protein (CRP) of the patients in the two groups were detected and compared before treatment and after 3d of treatment.The CD3+,CD4+,CD8+T cell and the CD4+/CD8+ ratio before treatment and after 3 d of treatment were calculated and compared between the two groups.Results:There was no significant difference in the level of serum inflammatory factors between the two groups before treatment (P＞0.05).The levels of serum inflammatory factors in the two groups were significantly decreased after 3 d of treatment,and the levels of IL-6,TNF-α and CRP in the CBP group were significantly lower than those in the conventional group (P＜0.05).There was no significant difference in immune function indexes between the conventional group and the CBP group before treatment (P＞0.05).After 3d of treatment,CD3+,CD4+ and CD4+/CD8+ ratio were significantly increased in the two groups,and the CD4+ and CD4+/CD8+ ratio of the CBP group were sig-nificantly higher than those of the conventional group (P＜0.05).Conclusion:The application of CBP in the treatment of patients with sep-sis can obviously improve the expression of inflammatory factors of patients,and can improve the immune function and promote the re-covery of immune function.