Objective To investigate the efficacy of sorafenib alone or combination with transarterial chemoembolization(TACE)and percutaneous local cryotherapy(PLCT)for advanced hepatocellular carcinoma patients without operation opportunity. Methods Sixty-four advanced hepatocellular carcinoma patients were selected as our subjects,who were underwent treatment of sorafenib alone or combination with TACE and PLCT. Thirty-two cases with sorafenib therapy were served as sorafenib group and another 32 cases with sorafenib in combination with transarterial chemoembolization and PLCT were served as combination group. All patients were followed up for 6 - 32 months. The treatment efficacy and tumor development were recorded. Results All surgeries of the patients were succeed and no death or serious operation complications occurred. Of 64 patients, 11 were achieved a complete remission( CR),31 cases with partial remission( PR),14 cases with stable development(SD),and 8 cases with progressive disease(PD). In the sorafenib group,3 cases were with CR,11 patients with PR,12 with SD,and 6 patients with PD. In the combination group,8 patients were with CR,20 patients with PR,2 patients with SD and 2 patients with PD,and the difference was significant between the two groups(χ2 = 14. 028,P = 0. 003). The median periods to tumor progression were 20 and 53 weeks in the sorafenib group and the combination group,and the difference was significant( χ2 = 14. 773,P = 0. 000). Conclusion For hepatocellular carcinoma patients without operation opportunity,sorafenib combined with TACE and PLCT can increase the tumor remission rate and prolong the periods to tumor progression in patients with hepatocellular carcinoma.

ObjectiveTo investigate the effect of immunoglobulin G (IgG) extravasated from blood circulation on the expression of toll-like receptor 4 (TLR4) induced by peripheral lipopolysaccharide (LPS) in rat brain. Methods The rats were divided into four groups in random, 5 rats in each. Group one received LPS 100μg/kg by intraperitoneal administration, normal saline was given by intravenous injection 6 hours later; group two was injected with adrenalin (AD) 15μg/kg intravenously; group three was treated with LPS intraperitoneally, AD was injected 6 hours later; group four was injected normal saline intravenously as control. For all groups, the animals were sacrificed 30 min after the last injection, and the brains were taken for investigation of the TLR4 expressions by immunofluorescence staining and RT-PCR. Result Immunofluorescence staining showed that IgG immunoreactive product was patch-like, distributed in the brain parenchyma in all the animals that received AD. In the LPS+normal saline group, IgG was found merely around the blood vessels. Meanwhile, in LPS+AD animals, TLR4 immunoreactive product coexisted with microglia marker Iba-1 within the IgG extravasated area. The double-labeled cells dispersed in the brain parenchyma and near to the cerebral vessels. In the LPS+saline group, TLR4 positive cells were endothelial-like. RT-PCR results indicated that the expression level of TLR4 in the LPS+AD group were significantly higher than that in the LPS+saline group or AD group or the saline control (P<0.01). Conclusion Extravasated circulating IgG may enhance the TLR4 expression in the rat brain induced by peripheral LPS.

Objective To compare the functional and radiographic outcomes of modified dual plating and less invasive stabilization system(LISS)in treatment of patients with complex tibial plateau fractures.Methods From November 2003 to November 2004,84 patients with complex tibial plateau fracture were treated with modified dual plating or LISS fixation.Their functional and radiographic outcomes were reviewed to compare the differences in bone union time,full weight-bearing time,knee joint's HSS(The Hospital for Special Surgery)scores,complications, tibia plateau angle(TPA)and posterior slope angle(PA).Results The 84 cases had complete follow-up records and a mean tollow-up of 16.2 months(range,12 to 23 months).Nine complications occurred in the modified dual plating group.The mean full weight-bearing time and radiographic bone union time were 16.8 and 15.1 weeks respectively for this group which had a mean HSS score of 87.4 points.Six complications were found in the LISS group.The mean full weight-bearing time and radiographic bone union time were 14.1 and 13.8 weeks respectively for this group which had a mean HSS score of 89.6 points.There were no significant differences between the two groups in changes of TPA and PA.Conclusion LISS can provide as good fixation as modified dual plating can for most complex tibial plateau fractures,but additional medial plate fixation may be necessary for some special cases.

Background Stickler syndrome is a genetic connective tissue disorder that affects the ocular,skeletal,orofacial and auditory systems.To determine the gene mutation loci can offer a basis for genetic diagnosis and management of Stickler syndrome.Objective The aim of this study was to research the clinical characteristics of a pedigree with Stickler syndrome and identify the disease-causing gene mutation.Methods This study was approved by Ethic Committee of Peking Union Medical College Hospital.The clinical study and pedigree analysis were performed in one family with Stickler syndrome type Ⅰ (STL Ⅰ).Nine family members were examined with informed consent.The entire coding regions of COL2A1 gene with flanking intronic regions were amplified by PCR and directly sequenced.The detected sequence change was confirmed to be mutationloci by examining whether they existed in normal control individuals.Mutant proteins were predicted with online software.Results There were 4 generations and 11 members in this family,and 2 members died,including 1 patient.Three patients were found in 9living families.Inheritance of this family complicd with an autosomal dominant inheritance mode.All affected individuals showed the consistent phenotypes with STL Ⅰ,including high myopia,membranous vitreous anomaly and surface central flat,short nose,palatoschisis,etc.Mutation screening of COL2A1 gene revealed that the first base of intron 12 was deleted(IVS12+1G del).Nucleotide sequence analysis showed that this mutation led to the functional abnormal of this gene by forming termination cordon in advance.This mutation occurred in all affected individuals,however,no mutation was observed in any unaffected member or 100 normal unrelated individuals.Conclusions This study identifies a novel splice-site mutation(IVS12+ 1G del)in COL2A1 gene in a Chinese STL Ⅰ pedigree.This is the first report on a mutation in a Chinese STL Ⅰ family.

Histiocytosis, Langerhans-Cell ; Humans

OBJECTIVETo explore the relationship of bone cement distribution and the puncture angle in the treatment of thoracolumbar compression fractures with unilateral percutaneous kyphoplasty (PKP).

METHODSThe clinical data of 37 patients with thoracolumbar osteoporotic compression fractures underwent PKP between January 2013 to March 2014 were retrospectively analyzed, all punctures were performed unilaterally. There were 6 males, aged from 65 to 78 years old with an average of (71.83 ± 6.15) years; and 31 females, aged from 57 to 89 years old with an average of (71.06 ± 7.89) years. Imaging data were analyzed and puncture angle and puncture point were measured before operation. According to the measured data, the puncture were performeds during the operation. Distribution area of bone cement were calculated by X-rays data after operation. The effect of bone cement distribution on suitable puncture angle was analyzed; VAS score was used to evaluate the clinical effects.

RESULTSThe puncture angle of thoracic vertebrae in T8-T12 was from 28° to 33° with an average 30.4°; and the puncture angle of lumbar vertebrae in L1-L5 was from 28° to 35° with an average of 31.3°. Postoperative X-rays showed the area ratios of bilateral bone cement was 0.97 ± 0.15. Bilateral diffuse area were basic equal. Postoperative VAS score decreased significantly (1.89 ± 1.29 vs 7.03 ± 1.42).

CONCLUSIONThrough measure imaging data before operation with PKP,the puncture point and entry point can be confirmed. According the measured data to puncture during operation, unilateral puncture can reach the distribution effect of the bilateral puncture in the treatment of thoracolumbar compression fractures.

Aged ; Aged, 80 and over ; Bone Cements ; Female ; Fractures, Compression ; surgery ; Humans ; Kyphoplasty ; methods ; Lumbar Vertebrae ; injuries ; surgery ; Male ; Middle Aged ; Spinal Fractures ; surgery ; Spinal Puncture ; methods ; Thoracic Vertebrae ; injuries ; surgery

Objective: To assess the effect of coronary collateral circulation (CCC) on myocardial viabilityin patients with chronic total occlusion of left anterior descending (LAD) artery. Methods: A total of 101 consecutive patients with confirmed diagnosis of total LAD occlusion in our hospital were enrolled. Rest 99mTc-MIBISPECT myocardial perfusion and 18F-FDG PET were performed, in addition all patients received coronary angiography (CAG) at 3 months front and back. Both images were reconstructed in the same machine and QPS software was used to obtain the summed rest score (SRS), abnormal resting total perfusion defect (TPD), viable and non-viable myocardium, LVEDV, LVESV and LVEF in relevant patients. Based on CAG result, the patients were divided into 2 groups: CCC group, n=39 and No CCC group, n=62; according to existing old myocardial infarctionand location of LAD occlusion, the patients were further divided into 4 subgroups. The above parameters were compared among different groups. Results: There were 86 male and 15 female patients with the mean age at (59.92±11.43) years. Relevant parameters in CCC group and No CCC groupwere as in SRS: (21.23±9.68) vs (28.56±8.76), TPD: (30.03±13.69) %vs (40.37±12.50) %, viable myocardium: (21.77±13.12) % vs (13.66±9.23) %, non-viable myocardium (8.28±8.58) %vs (27.40±12.97) %, all P<0.05; in LVEDV: (109.82±30.01) ml vs (173.71±57.69) ml, LVESV: (62.82±22.39) ml vs (122.53±51.66) ml, LVEF: (43.85±8.46) % vs (31.03±8.30) %, all P<0.05. Conclusion: Our preliminary study found that CCC could maintain left ventricular rest perfusion, myocardial viability and protect cardiac function in patients with chronic total LAD occlusion.

OBJECTIVETo explore methods and clinical outcomes of microsurgical technique in treating patients with severed palm.

METHODSFrom January 2009 to December 2012,45 patients with severed palm were treated by replantation through microsurgical technique, included 37 males and 8 females, aged from 13 to 45 years old with an average of 25. Postoperative survival rate and evaluation standard of upper limb replantation function posposed by Chinese Medical Association were applied for evaluate clinical outcomes after operation.

RESULTSForty-five patients with severed plam were treated by replantation. Thirty-nine patients (121 fingers) were survived,and survival rate was 87%. All patients were followed up 3 to 15.5 months with an average of 11.5 months. According to evaluation standard of upper limb replantation function posposed by Chinese Medical Association, the total score was 80.27 +/- 1.93, and 27 cases got excellent results, 8 good and 4 poor.

CONCLUSIONThe success of replantation of severed palm depends on grasping operation indication strictly, knowing complexity and local anatomic relationship, debridement completely during operation, making full use of remaining organization, arteriovenostomy through microsurgical technique as early as possible, constructing circulation and repairing injuried nerve rationally.

Adolescent ; Adult ; Female ; Hand Injuries ; physiopathology ; surgery ; Humans ; Male ; Middle Aged ; Reconstructive Surgical Procedures ; Replantation ; Young Adult

ObjectiveTo identify the framework for assessing health related quality of life(HRQOL) of Kaschin-Beck disease(KBD),in order to reflect the impact of KBD on quality of life in patients with the disease.MethodsQualitative descriptive research was adopted.Semi-open ended questions were developed by using the World Health Organization(WHO) definitions of health and quality of life.Group interview and face to face interviews were conducted on 48 patients with KBD and 29 health care experts on KBD in Linyou and Yongshou counties,Shaanxi province,which were higher prevalence areas of KBD.Content template analysis was conducted and the template was based on the WHOQOL-100's framework.ResultsThe framework of HRQOL for KBD included four domains:physical activity,familial/social support,economic and psychological state.There were also eleven facets which were:pain and discomfort,physical function and activity limitation,diet and sleeping,social relationship,concerns of family responsibilities,social support,economic,housing and the surrounding environment,appearance concerns,mental health,and general state of health.The total entries were 69.ConclusionsThe framework for assessing HRQOL of KBD is established.The framework highlights the impact of KBD on the patients' quality of life with higher specificity.

?AIM:To evaluate the objective visual quality of patients who underwent corneal cross-linking for the keratoconus using double-pass analysis system.? METHODS: Advanced keratoconus patients who underwent UV - riboflavin corneal cross - linking from January to July 2015 were included. The outcomes of their objective scattering index ( OSI ) , predicted visual acuity ( VA ) , the cut - off frequency of modulation transfer function ( MTF cut- off ) , the Strehl ratio ( SR ) were compared before and 6mo after corneal cross-linking.?RESULTS: A total of 13 patients ( 16 eyes ) were included. There was no statistically significant difference between pre- and 6mo postoperative data in uncorrected visual acuity, best corrected visual acuity, refractions and mean value of Sim-k (P>0. 05). Non-invasive average tear film break up time ( NIAvg-BUT ) detected by the Sirius system decreased after corneal cross-linking ( P<0. 05 ) . Using double - pass analysis system, no statistically significant change was found in MTF cut off, Strehl Ratio, OSI before and after treatment(P>0. 05). Tear Film Analysis Mean OSI increased at 6mo postoperatively (P<0. 05).? CONCLUSION: The subjective visual quality isn’t effected by corneal cross-linking. The tear stabilities of patients are influenced by these operations at 6mo postoperatively. More observations on long-term effect are needed to be taken in the future.