Objective To analyze the clinical and pathological features of limb-girdle muscular dystrophy2B(LGMD2B).Methods The clinical and pathological features of 7 patients with LGMD2B were analyzed retrospectively.Results Seven patients had a slow onset,and progressive proximal muscle weakness,muscle atrophy,progressive,and incresed serum creatine phosphokinase; muscle biopsy showed different degree of muscle fiber degeneration,necrosis; stromal and inflammatory cell infiltration in muscle fiber; monoclonal antibody immunohistoehemical staining:showed expression of Dysferlin protein was not found in muscle cell membrane,Dystrophin,Sarcoglycans protein expression was normal.Monoclonal antibody immunohistochemical staining the proteins were expressed in normal cell membrane.Conclusions LGMD2B is a slow onset,progressive proximal muscle weakness,muscle atrophy.Histochemical staining on the basis of further immunohistochemical staining,to detect the membrane protein and Dysferlin protein expression,that is a necessary means to diagnose LGMD2B and inflammatory myopathies.

OBJECTIVE:To effectuate the pharmacy information communication with in the hospital.METHODS:The local area network concerning medicine information in the hospital was built by adopting FrontPage and Dreamweaver with the assists of Flash,Photoshop and Fireworks so as to provide services for the communication of pharmaceutical sciences informa-tion and the information collecting.RESULTS:The website on medicine information has been operating normally for11months and the visitors to which were more than5000person-time.CONCLUSION:Medicine information website has provided an active,healthy and an upward platform of communication and learning for all medical workers within the local area network of the hospital,which has make the gathering of clinical pharmacy information more promptly and effectively and which has achieve the expected effect.

Objective To realize the construction and expression of human dsFv genes to rabies virus. Methods The genes of dsFv VH and VL were amplified by PCR-based point mutagenesis strategy. Then the genes were cloned into plasmid pET-22b (+). E. coli BL21(DE3) was transformed with the recombinant expression plasmid and the protein was induced in Luris-Bertani medium by addition of IPTG. The inclusion body proteins of VH and VL were denaturalized by GuHCl, and then re-naturalized in refolding solution to form dsFv fragments. Afterwards, we evaluated the antigen-binding activity of the dsFv and its stability as compared with its originative scFv. Results The fragment genes of dsFv to rabies virus were constructed successfully by PCR-based point mutagenesis strategy. Sequence analysis proved that cysteines were introduced into the position 44 aa of VH and position 100 aa of VL. The dsFv VH and VL genes were expressed in PET22b(+)/BL21(DE3). The VH and VL protein folded into the active dsFv antibody fragments which showed specific binding capability to rabies virus. Conclusion We succeeded in achieving the stabilization of the human scFv to rabies virus and obtaining the active human dsFv antibody fragments. This established a solid basis for further study of the biological activaty and clinical application of dsFv to rabies virus.

Objective To identify the problems of medical record management standardization in clinical case management for the study of medical record quality and give some corresponding measures.Methods 3,000 medical records of patients in our hospital from January 2015 to December 2016 were collected and divided into control group (with 1,500 records from January 2015 to December 2015) and observation group (with 1,500 clinical medical records from January 2016 to December 2016).The control group adopted regular data management while the observation group employed standardized lean management.Medical record defects, rectification rate and the average scores of inpatient medical records between the two groups were compared and analyzed.Results The average score of inpatient medical records in observation group was significantly higher than that of the control group (t=13.672, P<0.05), while the defect degree and the modification rate of the medical records were significantly lower than those of the control group (P<0.05).Conclusion Standardized medical record management contributes to the improvement of the quality of medical records.

Objective To discuss the clinical value and nursing of the three-endoscope in the treatment of eholedoeholithiasis. Methods 45 eases of choledocholithiasis patients who were treated with LCDE (three-endoscope) were named as the research group.56 patients who received traditional open ab-dominal surgery were set as the control group. The average hospitalization time and satisfaction degree with nursing were compared, t test and χ2 test were adopted. Results The average hospitalization time was shorter and satisfaction degree with nursing was higher in the research group than those in the control group. Conclusions The treatment of choledochohthiasis with three-endoscope is safe and feasible, es-pecially when combined with antibiotics lavage and stone dissolution through naso-biliary duct.The opera-tion can widen the surgical indication,reduce the risk of surgery with little damage,clear stones completely, reduce postoperative complicatioas,make patients recover faster, shorten the hospital stay and achieve the same or better treatment results when compared to the traditional open abdominal surgery.

To investigate the clinical improvement of limping gait in patients with ankylotic hips and limb length discrepancy.Methods:From 1996 to 2005,12 patients with ankylotic hips and limb length discrepancy were treated by distraction osteogenesis with a mono-lateral external fixator and an intramedullary nail.The limb length discrepancy was 6.20-12.50 (median 8.45) cm.Limping gait was classified according to the recommendations of the American Academy of Orthopedic Surgeons/Hip Society and scored according to Harris:no limping scored 11 points,mild limping scored 8 points,moderate limping scored 5 points,while severe limping scored 0 points.Limping gait was severe in all patients pre-operatively and the total score was 0.Results:All patients were followed up for 30.00-46.00 (median 38.55) months,and all reported improvement in limping gait.The gain in length was 6.00-12.50 (median 8.20) cm,and the mean residual limb length discrepancy was 0-0.50 (median 0.20) cm.The total treatment time was 41.00-82.00 (median 61.50) weeks,the lengthening time was 14.00-38.00 (median 29.55) weeks.At the last follow-up,10patients had mild limping gait and 2 had moderate limping gait; the total score was 90.00.The median score was 7.50 (P25 was 8.00,P75 was 8.00).According to Wilcoxon signed rank test,the post-operative limping gait scores were significantly higher than pre-operative (P=0.001 ).Conclusion:Femoral lengthening can improve the limping gait significantly in ankylotic hips and limb length discrepancy.

Objective: To set a quantified diagnostic standard for large intestinal cancer of spleen qi deficiency syndrome. Methods: The spleen qi deficiency syndrome was identified by experts on the basis of clinical epidemiological investigation of 311 patients suffering from large intestinal cancer. Corresponding points were assigned to the correlative factors (traditional Chinese medicine symptoms) on the basis of symptom differences between spleen qi deficiency syndrome and non-spleen-qi-deficiency syndrome. The best threshold was determined by receiver operating characteristic curve (ROC) according to syndrome differentiation from expert team, and the quantified diagnostic standard was established. The syndrome identification from the expert team which was regarded as golden standard was tested retrospectively. Results: All the traditional Chinese medicine symptoms possibly related to spleen qi deficiency syndrome were analyzed based on the opinions of experts, and 28 symptoms were confirmed as candidate correlative factors. The occurrence of 11 symptoms between spleen qi deficiency syndrome and non-spleen-qi-deficiency syndrome showed statistical differences by means of crosstabs analysis (P<0.05). The 11 symptoms were filtered by logistic regression analysis, and tiredness, fatigue, loose stool, and poor appetite were finally determined as the symptoms relative to large intestinal cancer. These four symptoms were analyzed with conditional probability conversion and endowed with 16, 11, 4 and 8 points respectively. The diagnostic standard of spleen qi deficiency syndrome of large intestinal cancer was over 13 points. The sensitivity, specificity and accuracy of retrospective examination were all above 80%, and its positive likelihood ratio was 9.89. Conclusion: The quantified diagnostic standard for spleen qi deficiency syndrome of large intestinal cancer is in accordance with clinical characteristics of large intestine cancer and the characteristics of TCM syndrome diagnosis.

Objective To investigate the possible effects on radiosensitivity in human umbilical vein endothelial cells after transfection of pcDNA3.1+Ape1 plasmid. Methods The expressing vector pcDNA3.1+ Ape1, the control vector pcDNA3.1+or non-transfection cells was irradiated by 2, 4, 6, and 8 Gy photon beam at 48 h post-transfection. The value of initial and residual Oliver tail moment (OTM) under the alkaline single cell gelelectrophoresis assay and the colony forming test were utilized as the markers for the evaluaton of cells intrinsic radiosensitivity. The effect on radiosensitivity in human umbilical vein endothelial cells after transfection of the expressing vector pcDNA3.1+Ape1 was analyzed according to the radio-dose, compared to the empty vecor control and non-transfection cells. Results The initial and residual OTM value of endothelial cells transfected by 3 μg pcDNA3.1+Ape1 plasmid was lower significantly than ones of endothelial cells untransfected at 2 Gy irradiation (P<0.01), but was no significant difference at 8 Gy (P>0.05), and SF2 was higher remarkably in transfected cells than one in untransfected cells (P<0.05), but SF4, SF6 and SF8 were no significant differences (all of P>0.05). Conclusions The transfection of pcDNA3.1+Ape1 plasmid could enhance radioresistance of endothelial cells to the low-dose irradiation.

Objective To explore the possible role of vitamin D in the pathogenesis of IgA nephropathy (IgAN). Me-thods After the IgAN model was successfully induced at 12 weeks, the BALB/C mice were randomly divided into IgAN group (n=15) and IgAN+VitD group (n=15). The nephrosis mice were administrated with 100 μl/d propylene glycol or propyl-ene glycol+1,25(OH)2D, 3 ng/(100g?d), for 6 weeks. The control group was setted (n=15). The level of 24 hour urine protein was determined at week 0, 12 and 18. At week 18, the levels of serum 25(OH)D, ifbroblast growth factor 23 (FGF23) and galactose-deifcient IgA1 (Gd-IgA1) were detected. The mRNA and protein expressions of interleukin-21 (IL-21) in Peyer’s patches (PPs) were detected by lfuorescent quantitative reverse transcription-polymerase chain reaction and western blot respectively. The protein expression of Bcl-6 was detected by western blot. The percentages of Tfh cells/T lymphocytes, B220+IgM+/B lympho-cytes, B220+IgA+/B lymphocytes, B220-IgA+/B lymphocytes in PPs were determined by lfow cytometry. Results Compared with control group, the levels of 24 hour urine protein, FGF23 and Gd-IgA1 were increased, serum 25(OH)D was decreased, the mRNA and protein expressions of IL-21 and the protein level of Bcl-6 were increased, the percentages of Tfh cells/T lym-phocytes, B220+IgM+/B lymphocytes, B220+IgA+/B lymphocytes, B220-IgA+/B lymphocytes were elevated in IgAN group (P<0.05). These indicators were improved in IgAN+VitD group. Compared with the IgAN group, the differences were statisti-cally signiifcant (P<0.05), however compared with control group, some indicators showed no signiifcant differences (P>0.05). Conclusions 1,25(OH)2D may protect the microenvironment of PPs in IgAN through inhibiting the differentiation of Tfh cells and B cells and the generation of Gd-IgA1.