Neurofibromatosis type 1 is a term of Von Recklinghausenan.It is an autosomal dominant inherited disease which derived by neural crest cell.Prevalence of this disease is 1/3000 1/3500 and is a disease with the highest mutation rate.The pathogenesis of neurofibromatosis type 1 is associated with the deficiency of NF1 gene.Recently,the genetics and genomics research of neurofibromatosis make a great progress.With the development of gene linkage and position cloning technology,the gene sequence of neurofibromatosis type 1 has been found.Recent research of genetics and genomics of NF1 and the structure and function,abnormal expression,the relation of genotype and phenotype,the mutation sensitivity domain of NF1 gene were reviewed.

Objective To explore the short-term and long-term efficacy of laparoscopic versus open liver resection(LLR)in the treatment of hepatocellular carcinoma(HCC). Methods The perioperative and follow-up data ofLLR(n = 43)and OLR(n = 73)for the treatment of HCC at the same period were analyzed respectively. Results There were no significant differences in the age,gender,Child-Pugh classification,liver cirrhosis, AFP,and hepatic virus affection between the 2 groups. 1 case in the LLR group was converted for the open surgery and another one case in the OLR group died in the perioperative time. However ,compared with OLR group ,the intraoperative blood loss of LLR group(P < 0.05)was larger and the mean operative time(P < 0.05)was longer. The liver function indicators of the regular hepatectomy subgroup were recovered more quickly(P<0.05),but the indicators of remaining subgroups were not significantly different. The postoperative ambulation time (P < 0.05), the postoperative eating time(P < 0.05),the postoperative hospital stay(P < 0.05)were all shorter. The VAS score was lower(P<0.05). There was no difference in the incidence and distribution of complications and the inci-dence of severe complications between the two groups(all P>0.05). The first and third year disease-free survival rates were 82.2%,53.8%for LLR and 91.5%,75.9%for OLR,respectively(χ2=0.55,P=0.46). The first and third 5 year overall survival rates were 94.2%,79.3%for LLR and 94.9%,47.3%for OLR,respectively(χ2=1.06, P=0.30). Conclusion LLR for HCC treatment is a safe and effective way with the advantages of minimal opera-tive trauma,quick recovery and significant short-term efficacy.

Objective To evaluate the clinical efficacy of different treatments on non-small cell lung cancer (NSCLC) patients with brain metastases and to explore the influential factors of the prognosis.Methods The NSCLC patients with brain metastases treated from Jan.2010 to Dec.2011 were follow-up.The survival time and influences resulted from the treatments were analyzed.Results The average survive time of these patients was (11.93±5.53) months,and the median survive time was 11 months.The 6-month,1-year and 2-year overall survival rates were 90.7 ％,41.1％ and 6.4 ％,respectively.Multivariate analysis showed that control of extracranial lesions,Kamofsky score,target therapy and age were independent predictive factors of survival,and the OR value were 0.358 (95 ％ CI0.217-0.593),0.302 (95 ％ CI 0.182-0.502),0.170 (95 ％ CI 0.098-0.296) and 1.635 (95 ％ CI 1.010-2.647),respectively (all P ＜ 0.01).Conclusions Radiation therapy is an effective treatment on non-small cell lung cancer with brain metastases.Biological target therapy can effectively improve survival.The survival time also is correlated with age,Karnofsky score and control of extracranial lesions.

Objective To investigate the significance of an accessory hepatic vein (AHV) in the interventional treatment of Budd-Chiari syndrome (BCS).Methods From Mar.2011 to Jun.2013,35 patients with BCS who also had an AHV were included into this study.The patients were divided into two groups according to whether the AHV was obstructive:21 patients had obstruction to the inferior vena cava (IVC) and to the 3 hepatic veins (HV),but the AHV was open; 14 patients had obstruction to the AHV and the 3 HVs.In 13 of these patients the IVC was open,but 1 patient had obstruction to the IVC.During treatment,the patients in the AHV open group underwent balloon dilation or stent insertion of IVC ; patients in the AHV obstruction group underwent balloon dilation or stent insertion of AHV.The patient with obstruction to the IVC underwent balloon dilation of IVC first.Results All patients were successfully treated without any procedure-related complications.In the AHV open group,the average pressure of the AHV decreased from (28.81 ± 6.23) cmH2 O (1 cmH2O =0.098 kPa) before treatment to (18.62 ± 5.06) cmH2O after treatment (P ＜ 0.01) ; the average serum TBIL decreased from (23.24 ± 6.41) μmol/L before treatment to (19.52 ± 4.31) μmol/L after treatment (P ＜ 0.01) ; the average serum albumin changed from (33.76 ± 3.74) g/L before treatment to (34.05 ± 3.62) g/L after treatment (P =0.485).In the AHV obstruction group,the average pressure of the AHV decreased from (36.29 ± 11.65) cmH2O before treatment to (22.07 ± 7.67) cmH2O after treatment (P ＜ 0.01) ; the average serum TBIL decreased from (31.24 ± 9.54) μmol/L before treatment to (20.93 ±7.26) μmol/L after treatment (P ＜0.01) ; the average serum albumin changed from (32.14 ± 4.55) g/L before treatment to (32.11 ± 4.47) g/L after treatment (P =0.861).During follow-up,no patients experienced recurrence of symptoms in the AHV open group; one patient experienced recurrence of symptoms 5 months after treatment in the AHV obstruction group.This patient received a second balloon dilation of the AHV.Conclusions For patients with BCS with IVC and the 3 HVs obstruction,if the AHV was open,we could only treat the IVC.For patients with BCS with AHV and the 3 HVs obstruction,AHV dilation followed by recanalization of AHV was also effective.

Objective: To find out the influence of stabilization occlusal splint on intra-articular pressure of the temporomandibular joint. Methods: A muti-channel measuring and analysis system for intra-articular pressure of the temporomandibular joint was developed in this study. Twenty-two subjects were selected to detect the intra-articular pressure of the temporomandibular joint before and after wearing stabilization splint. Results: Before splint wearing, the intra-articular pressure of the temporomandibular joint at intercuspal occlusion status was 61.3?48.5 mm Hg (1 mm Hg=0.133 kPa), the negative pressure at open mouth status was 33.2?34.2 mm Hg. After splint wearing, the pressure at intercuspal occlusion status was 39.5?24.5 mm Hg, the negative pressure at intercuspal at open mouth status was 36.3?25.3 mm Hg. The intra-articular pressure of the temporomandibular joint after wearing splint was significantly lower than the pressure before splint was used, the pressure of mouth opening status was stable before and after splint was used. Conclusion: Stabilization occlusal splint can reduce the intra-articular pressure of the temporomandibular joint.

Objective To investigate the clinical effect of blocking nail in treatment of hypertrophic nonunion after interlocking intramedullary nailing of femoral shaft fractures.Methods A retrospective case series study was made on 11 patients with hypertrophic nonunion following interlocking intramedullary nailing of femoral shaft fractures from January 2006 to February 2016.There were 8 males and 3 females,aged (32.4 ± 6.8) years (range,19-48 years).Comminuted fractures were noted in three patients,long oblique or spiral fractures in two,multi-segment fractures in two,and transverse fractures in four.Time interval between this surgery and intramedullary nailing of femoral shaft fractures was (11.5 ±3.1) months (range,6-18 months).All blocking nails were inserted into pulp cavity of distal ends of the site of the bone nonunion.Fracture healing time,wound healing and postoperative complications were detected.Outcome was evaluated using the Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI).Results All patients were followed up for (27.2 ± 4.6) months (range,24-36 months).Bone union was observed within (9.8 ± 3.4) months except that bone nonunion occurred in two cases requiring locking plate fixation and autologous bone grafting to provide union,with the healing rate of 91％.No wound infection,nail breakage and nail loosening occurred after operation.At postoperative 3 months,4 months,5 months,6 months,12 months and 24 months,VAS and ODI differed significant from the preoperative detections (P ＜ 0.05).Conclusion Blocking nail technique is associated with increased stability of intramedullary nails and fracture ends,low incidence of complications and high bone healing rate in treatment of hypertrophic nonunion after femoral interlocking intramedullary nailing.

Objective: To investigate the effects of extract of Bulbus Allii Caespitosi on cardiocyte viability of swines with myocardial reperfusion injury by analyzing the 18F-fluorodeoxyglucose ((18)F-FDG) position emission tomography (PET) imaging. Methods: Twenty-four swines were randomly divided into sham-operated group, untreated group, trimethazine group and extract of Bulbus Allii Caespitosi group. Myocardial reperfusion injury was induced by plugging the anterior descending coronary artery of swine with sacculus. Bulbus Allii Caespitosi or trimetazidine was given twice daily for 28 days. Then myocardial perfusion was detected with (18)F-FDG PET/CT and the radioactivity distribution was evaluated. Results: Compared with the untreated group, Bulbus Allii Caespitosi and trimetazidine could improve the activity of myocardial cells after myocardial infarction (P<0.01), and there were no significant differences between Bulbus Allii Caespitosi and trimetazidine (P>0.05). Conclusion: Bulbus Allii Caespitosi can improve myocardial metabolism after ischemia and reperfusion in swines.

Objective:To observe the clinical characteristics with different peritoneal transport type in patients with continuous ambulatory peritoneal dialysis (CAPD),and to investigate the factors associated with peritoneal transport function.Methods:The clinical data of 158 CAPD patients were analyzed retrospectively.According to peritoneal equilibration test,a method for evaluation of the peritoneal transport function,the patients were divided into 2 groups:a high average and high peritoneal transport group (Group A,n=84) and a low average and low peritoneal transport group (Group B,n=74).T-he demographics,clinical biochemical indexes and the incidence of cardiovascular complications were compared between the 2 groups.Logistic regression analysis was used to find the factors relevant to peritoneal transport function.Results:The level of serum albumin (ALB) in the Group B was significantly higher than that in the Group A (P＜0.05).The 4 h dialysate/plasma creatinine (D/Pcr),high-sensitivity C-reactive protein (hs-CRP),body mass index (BMI),and the rates of cardiovascular complications in the Group A were significantly higher than those in the Group B (P＜0.05).Correlation analysis showed that the D/Pcr was positively correlated with the BMI,serum hs-CRP and cardiovascular complications (r=0.179,0.373 and 0.426,respectively,P＜0.05),while it was negatively correlated with ALB (r=-0.393,P＜0.01).Logistic regression analysis showed that the high BMI (OR=1.178,P＜0.05),cardiovascular complications (OR=5.035,P＜0.01),and the low serum ALB (OR=0.852,P＜0.01)were the risk factors for high peritoneal transport.Conclusion:The serum ALB level,BMI and the cardiovascular complications are associated with high peritoneal transport,which are useful markers for predicting the peritoneal transport function before peritoneal dialysis.

This article was aimed to study the main effective substances and characteristics of Ke-Luo-Xin (KLX) capsule. HPLC-DAD system was applied in the establishment of HPLC finger prints of serum of rats after taking KLX capsule. And then, serum samples taken KLX capsule, blank blood serum, and single crude drugs were com-pared. Constituents absorbed into the serum were determined by HPLC-DAD system. The results showed that meth-ods for serum HPLC fingerprinting had good precision, reproducibility and stability. A total of 13 constituents migrat-ing to the blood were detected, of which 9 were prototype constituents, 4 were metabolites. Prototype constituents in-cluded aloeemodin and chrysophanic acid. It was concluded that 13 constituents migrating to the blood may be the main effective substances of KLX capsule.

OBJECTIVETo investigate the prognostic factors for non-small cell lung cancer(NSCLC)in patients under 40 years of age.

METHODSThe clinicopathological data of 148 young patients with NSCLC were retrospectively analyzed. Kaplan-Meier and Cox regression analyses were used to analyze the relationship between prognostic factors and survival time.

RESULTSThe patients were followed-up for 6 - 148 months, and the follow-up rate was 100%. In the whole group, 122 patients died and 26 cases were surviving. The 1-, 3- and 5-year survival rates were 54.7%, 10.4% and 5.6%, respectively. The median survival time (MST) was 14.7 months. Kaplan-Meier analysis showed that Karnofsky performance status (KPS), clinical stage, treatment modality and serum CEA were related with prognosis (P < 0.05). Multivariate analysis indicated that KPS, clinical stage, treatment modality and serum CEA were independent prognostic factors (P < 0.05).

CONCLUSIONSKPS, CEA, clinical stage and treatment modalities are independent prognostic factors in young NSCLC patients.

Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoembryonic Antigen ; blood ; Carcinoma, Non-Small-Cell Lung ; blood ; drug therapy ; pathology ; radiotherapy ; surgery ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Karnofsky Performance Status ; Lung Neoplasms ; blood ; drug therapy ; pathology ; radiotherapy ; surgery ; Male ; Neoplasm Staging ; Pneumonectomy ; methods ; Proportional Hazards Models ; Retrospective Studies ; Survival Rate ; Young Adult