Objective:To evaluate the safety and feasibility of the in-situ needle fenestration combined with the in vitro physician modified fenestration technique to reconstruct supra-aortic branches during thoracic endovascular aortic repair (TEVAR) for aortic arch lesions requiring landing at Z0 and Z1.Methods:From Nov 2017 to Dec 2019, eighteen patients who underwent both the in-situ needle fenestration and the in vitro physician modified fenestration techniques to extend the proximal landing zone to Z0 and Z1 during TEVAR were included in our study.Results:Sixteen patients underwent in vitro physician modified fenestration ,two patients underwent in vitro physician modified fenestration to reconstruct both the left common carotid artery and the innominate artery. All eighteen patients received in-situ needle fenestration to preserve the left subclavian artery. Supra aortic branches were preserved in all patients (38/38, 100%). There was no Type Ⅰ endoleak. Type Ⅱ endoleak was found in four paitnets (4/18). Type Ⅲ endoleak occurred in one patient (1/18). Type Ⅳ endoleak in four patients (4/18). Type Ⅲ endoleak needed open aortic arch repair 6 months later. The median follow-up time was 12 months. One (1/18) died in 12 months and the other patients were doing well.Conclusions:The joint application of the in-situ needle fenestration and the in vitro physician modified fenestration to reconstruct supra-aortic branches during TEVAR for aortic arch pathologies requiring landing at Z0 and Z1 was satisfactory.

OBJECTIVEFabry disease is a rare lysosome storage disease featuring X-linked recessive inheritance. The study was to explore potential mutations of alpha-galactosidase A (GLA) gene and their correlation with clinic manifestations in three Chinese pedigrees with Fabry disease.

METHODSAll exons and flanking sequences of GLA gene were amplified with PCR. Potential mutations were detected with bidirectional DNA sequencing. Correlation between particular mutations and clinic features were analyzed.

RESULTSA unreported missense mutation, c.797A>C (D266A) in GLA exon 5 was identified in pedigree 1. Also in exon 5, a missense mutation c.644A>G (N215S) was found in pedigree 2. In pedigree 3, a nonsense mutation c.355C>T (Q119X) was found in exon 2. The c.797A>C mutation was not detected in 200 unrelated male controls. The probands of pedigrees 1 and 3 had presented mainly with skin damage and chronic renal insufficiency, whilst the proband of pedigree 2 had presented with hypertrophic cardiomyopathy.

CONCLUSIONThe unreported c.797A>C (D266A) mutation is the sixth missense type mutation of the 266th codon of GLA gene, and all other 5 missense mutations reported previously had been confirmed to be responsible for Fabry disease. The c.797A>C mutation, not found in 200 unrelated male controls, may be the causative mutation in pedigree 1. The c.644A>G and c.355C>T mutations were first detected in Chinese patients. Variable phenotypes of Fabry disease may be in part attributed to the natures of particular mutations of GLA gene.

Adult ; Fabry Disease ; genetics ; Humans ; Male ; Middle Aged ; Mutation ; Pedigree ; alpha-Galactosidase ; genetics

OBJECTIVETo investigate the effect of epidural ropivacaine in combination with fentanyl for labor analgesia on the clinical outcome of labor.

METHODSA retrospective study was conducted involving 281 healthy primiparas, including 106 undergoing spontaneous labor who received epidural 0.15% ropivacaine in combination with fentany (1microg/ml) and 175 without epidural analgesia. The active phase duration, durations of each labor stages, delivery modes, management of labor, postpartum hemorrhage, incidence of fetal distress and asphyxia neonatorum were recorded in the two groups. The visual analogue scale (VAS) was used to assess the pain of uterine contraction, and modified Bromage scoring system applied to evaluate the lower limb motor block.

RESULTSThere were no significant differences in the duration of the first, third or the total labor stages between the two groups, but the second labor stage was prolonged in the labor analgesia group. The ratio of spontaneous labor, assisted vaginal delivery, and incidence of asphyxia neonatorum were higher, whereas the duration of the active stage was shortened in the analgesia group.

CONCLUSIONEpidural ropivacaine in combination with fentanyl in labor can decrease the incidence of cesarean section, and the duration of the active stage can be shortened with application of ocytocin.

Adult ; Amides ; administration & dosage ; therapeutic use ; Analgesia, Epidural ; methods ; Analgesia, Obstetrical ; methods ; Anesthetics, Combined ; Anesthetics, Intravenous ; administration & dosage ; therapeutic use ; Anesthetics, Local ; administration & dosage ; therapeutic use ; Female ; Fentanyl ; administration & dosage ; therapeutic use ; Humans ; Labor Pain ; drug therapy ; Pregnancy ; Pregnancy Outcome ; Retrospective Studies

Objective To provide the skin vessels morphologie basis for perforating flap of the antero- lateral thigh(ALT).Methods Six sides lower limbs of adult fresh cadaver specimens perfused with lead oxide-gelatine mixture were used.Observe the peraforators of anterolateral thigh by dissection.The elevated tissue was radiographed by X-ray and MSCT-scanning.Computer techniques was used for the detection and 3D-reconstruction of the regions of each perforators of ALT flap.Results The three-dimensional recostruct- ed digitized visible models perfectly displayed the anatomic structures of arteries on the anterolateral thigh, and,the morphology and distribution of the vascular territory of each perforator from descending branch of LCFA was displayed in the images of 3D-visualization,their effective morphology and distribution were same, and they accord with vascular territory of radiograph.On the 2D-image of X-ray,The margins of the anatomical distribution of each perforator from descending branch of the lateral circumflex femoral artery were determined, their total areas was about 25cm?24cm.Conclusion Since the digitized image of perforator territory can accord with the morphology of anatomy,and can off vascular structure insights into cutaneous perforator anato- my,their 3D visualizative models can be applied in pre-operative designing and virtual operation procedures, and can be helped for study of perforator flap.

In order to discover the mechanism of Xuebijing oral effervescent tablet (XBJOET) to treat infectious diseases, the effect of XBJOET on endotoxin induced rabbit fever and disseminated intravascular coagulation (DIC) was investigated. Auricle microcirculation in rabbit was detected by laser speckle blood perfusion imager system; coagulation function was measured by coagulation analyzer, fibrinolytic system was quantified by Elisa assay and micro thrombosis in tissues was observed with HE staining under light microscope. The results demonstrated that the body temperature of rabbit decreased significantly at 1-3 h after administration with 4.8, 2.4 and 1.2 g x kg(-1) XBJOET to endotoxin induced DIC rabbit model, the auricle microcirculation blood flow in model group (54.45 +/- 14.53) PU was lower than that in control group (77.18 +/- 12.32) PU. The auricle microcirculation blood flow increased markedly and there was significant difference between model group and 1.2 g x kg(-1) XBJOET group. There was significant difference between model group and control group in the content of PAI1 and FIB. The PAI1 levels in model and control groups were (30.48 +/- 2.46) ng x mL(-1) and (20.93 +/- 3.25) ng x mL(-1), respectively. The FIB levels in model and control group were (3.34 +/- 1.09) g x L(-1) and (4.84 +/- 1.10) g x L(-1), respectively. The content of PAI1 in rabbit plasma decreased notably, there were significant differences between model group and 4.8, 2.4 g x kg(-1) XBJOET groups. On the contrary the content of FIB increased. XBJOET possessed pharmacological activities of curing infectious fever and DIC, the mechanism of which is related to amelioration of microcirculation disturbance, inhibition of fibrinolytic system activation and coagulation and micro thrombosis elimination.
Administration, Oral ; Animals ; Blood Coagulation ; drug effects ; Body Temperature ; drug effects ; Disseminated Intravascular Coagulation ; blood ; chemically induced ; Drugs, Chinese Herbal ; administration & dosage ; pharmacology ; Ear Auricle ; blood supply ; Endotoxins ; Female ; Fever ; chemically induced ; drug therapy ; physiopathology ; Fibrinogen ; metabolism ; Male ; Microcirculation ; Partial Thromboplastin Time ; Plasminogen Activator Inhibitor 1 ; blood ; Prothrombin Time ; Rabbits ; Tablets ; Thrombosis ; pathology

OBJECTIVETo determine the optimal position of hypoglossal nerve in hypoglossal-facial nerve anastomosis and the eligibility of hypoglossal-facial nerve anastomosis with the cervical loop.

METHODSThe cervical course and adjacent structures of the hypoglossal nerve were observed on 21 adult cadavers. The hypoglossal nerve and facial nerve were taken from 3 fresh specimens, and the number of the fasciculus and the cross-sectional area of the nerve were measured.

RESULTSThe facial nerve trunk were monofascicular with a cross-sectional area of 5.1-/+0.2 (range 4.6-5.7) mm(2). The number of the fasciculus and the cross-sectional areas of the nerve trunk and the fasciculus were 1.6-/+0.8 (range 1-4) mm(2) , 7.5-/+0.7 mm(2) (range 6.8-8.0) mm(2), and 4.7-/+0.6 (4.1-5.5) mm(2), respectively, at the proximal segment of the hypoglossal nerve, 3.6-/+0.5 (1-5) mm(2) , 5.6-/+0.5 (4.9-6.1) mm(2) , and 1.6-/+0.4 (0.9-2.2) mm(2) at the distal segment, and 2.4-/+0.8 (1-3) mm(2), 1.1-/+0.7 (0.6-2.2) mm(2), and 0.5-/+0.3 (0.3-1.2) mm(2) at the cervical loop.

CONCLUSIONThe cervical loop is inadequate for facial nerve anastomosis and the proximal segment is large enough to allow partial harvesting of the hypoglossal nerve for neurotisation of the facial nerve.

Anastomosis, Surgical ; methods ; Cadaver ; Facial Nerve ; anatomy & histology ; surgery ; Humans ; Hypoglossal Nerve ; anatomy & histology ; surgery ; Nerve Transfer ; methods

The goal of this study is to investigate the population pharmacokinetics of oral given clozapine in Chinese schizophrenic patients and to identify possible relationships between population parameters and covariates including demography factors and CYP1A2 genetic polymorphism, so as to create the population pharmacokinetics model to guide individual clinical delivery. Details of drug dosage history, sampling time and concentration of 626 data points from 183 patients were collected retrospectively. The 183 patients were randomly allocated either to the index group (n = 168) or to the validation group (n = 15). Population pharmacokinetic data analysis was performed using the nonlinear mixed-effects model (NONMEM) program on the index group. The values of apparent clearance (CL/F), apparent volume of distribution (V/F) and the constant of absorption rate were estimated. A number of covariates including demographic index, coadministration of other drugs and CYP1A2 genotypes were evaluated statistically for their influence on these parameters. The final population model related clearance with day-dose/BSA (DBSA) and smoke habit (SMOK). Predictive performance of the final model evaluated with the validation group showed insignificant bias between observed and model predicted concentrations. Typical value of CL/F (non-smoking group), V/F and the constant of absorption rate were 28.5 L x h(-1) (5.05%), 1 290 L (16.7%) and 2.26 h(-1) (fixed), inter-patient variability (CV) in CL/F and V/F was) 42.2% and 10.0%, respectively. It was observed that the values of CL/F in the two smoking groups were higher than that in the non-smoking group. The residual variability (SD) between observed and model-predicted concentrations was 45.8 microg x L(-1).
Adolescent ; Adult ; Aged ; Antipsychotic Agents ; pharmacokinetics ; Asian Continental Ancestry Group ; genetics ; Clozapine ; pharmacokinetics ; Cytochrome P-450 CYP1A2 ; genetics ; Female ; Genetics, Population ; Humans ; Male ; Middle Aged ; Models, Theoretical ; Schizophrenia ; drug therapy ; genetics ; metabolism ; Smoking ; Young Adult

OBJECTIVETo retrospectively analysis the treatment characteristics of the systemic situation in patients with crush syndrome after Wenchuan earthquake happened in May 12th, 2008.

METHODSForty-nine patients with crush syndrome and subsequent acute renal failure (ARF) due to the earthquake were treated in West China Hospital. All of patients had been rescued from buildings that collapsed in Wenchuan earthquake. The major associated injuries were in the low extremities and upper extremities. 49 patients developed ARF with increased concentrations of serum creatinine (mean 64 022 U/L) had underwent haemodialysis. Hyperkalaemia was seen in 9 patients and four of them underwent haemodialysis. 49 patients were administered hemodialysis.

RESULTSNo patient died. All patients who suffered from the ARF were weaned from hemodialysis after admitted 7 to 35 days. Forty-five extremities underwent amputations and 52 extremities had fasciotomy.

CONCLUSIONSCrush syndrome requires urgent recognition and prompt surgical treatment with simultaneous measures to control hyperkalemia and ARF. The authors believe that immediate intensive care therapy and multi-subjective coordination would have improved the survival rate.

Acute Kidney Injury ; etiology ; surgery ; therapy ; Adolescent ; Adult ; Aged ; Amputation ; Child ; Crush Syndrome ; etiology ; surgery ; therapy ; Decompression, Surgical ; Earthquakes ; Female ; Humans ; Male ; Middle Aged ; Renal Replacement Therapy ; Retrospective Studies ; Treatment Outcome ; Wounds and Injuries ; complications

Objective To study the quality comparability of human coagulation factor Ⅷ(FⅧ)produced before and after the change of factory site.Methods A comparative study was carried out on quality quantitative indexes,related im-purities and stability data of FⅧ produced before and after the change of factory site.Results The FⅧ quantitative quality before and after the change of factory site all met the quality standard,and the related impurities including aluminum resi-due,tributyl phosphate residue,polysorbate 80 residue and PEG residue all met the quality standard.Other impurities in-cluding human fibrinogen,fibronectin,plasminogen,IgA,IgM and IgG were extremely low in content and equivalent in quality.The content of VWF(von Willebrand factor)had no obvious change before and after the change of factory site,but was significantly higher than that of other domestic manufacturers'commercial products.The results of accelerated stability and long-term stability tests showed that the titer of FⅧ fluctuated within the methodological error range,and the results all met the quality standard.Conclusion The change of factory site of FⅧ has no effect on the quality.

Background:For Chinese patients with hepatocellular carcinoma (HCC),surgical resection is the most important treatment to achieve long-term survival for patients with an early-stage tumor,and yet the prognosis after surgery is diverse.We aimed to construct a scoring system (Shanghai Score) for individualized prognosis estimation and adjuvant treatment evaluation.Methods:A multivariate Cox proportional hazards model was constructed based on 4166 HCC patients undergoing resection during 2001-2008 at Zhongshan Hospital.Age,hepatitis B surface antigen,hepatitis B e antigen,partial thromboplastin time,total bilirubin,alkaline phosphatase,γ-glutamyltransferase,α-fetoprotein,tumor size,cirrhosis,vascular invasion,differentiation,encapsulation,and tumor number were finally retained by a backward step-down selection process with the Akaike information criterion.The Harrell's concordance index (C-index) was used to measure model performance.Shanghai Score is calculated by summing the products of the 14 variable values times each variable's corresponding regression coefficient.Totally 1978 patients from Zhongshan Hospital undergoing resection during 2009-2012,808 patients from Eastern Hepatobiliary Surgery Hospital during 2008-2010,and 244 patients from Tianjin Medical University Cancer Hospital during 2010-2011 were enrolled as external validation cohorts.Shanghai Score was also implied in evaluating adjuvant treatment choices based on propensity score matching analysis.Results:Shanghai Score showed good calibration and discrimination in postsurgical HCC patients.The bootstrap-corrected C-index (confidence interval [CI]) was 0.74 for overall survival (OS) and 0.68 for recurrence-free survival (RFS) in derivation cohort (4166 patients),and in the three independent validation cohorts,the CIs for OS ranged 0.70-0.72 and that for RFS ranged 0.63-0.68.Furthermore,Shanghai Score provided evaluation for adjuvant treatment choices (transcatheter arterial chemoembolization or interferon-α).The identified subset of patients at low risk could be ideal candidates for curative surgery,and subsets of patients at moderate or high risk could be recommended with possible adjuvant therapies after surgery.Finally,a web server with individualized outcome prediction and treatment recommendation was constructed.Conclusions:Based on the largest cohort up to date,we established Shanghai Score-an individualized outcome prediction system specifically designed for Chinese HCC patients after surgery.The Shanghai Score web server provides an easily accessible tool to stratify the prognosis of patients undergoing liver resection for HCC.