Maple syrup urine disease (MSUD) is a rare inherited disorder of branched-chain amino acid metabolism presenting with life threatening encephalopathy and maple syrup odor in urine in affected individuals.Blood levels of branched-chain amino acids(BCAA) significantly increase.It is classified into 5 forms according to the clinical course,and classified 4 molecular phenotypes based on the affected locus of the branched chain αr-ketoacid dehydrogenase complex.Treatment of MSUD is divided into acute decompensation stage treatment and life-long dietary restriction treatment.

Objective To investigate the feasibility and efficacy of using small intestinal submucosa (SIS) as scaffold in cystoplasty augmentation for treating the neurogenic bladder.Methods We retrospectively reviewed our database of 14 patients with neurogenic bladder,including ten male and four female,between January 2011 and March 2014.The mean age in those patients was 29 years old (range 14-56 years old).The etiology of neurogenic bladder included myelomeningoceles in 8 cases and spinal cord injuries in 6 cases.The urodynamic examination showed that the mean volume of maximal bladder pressure was (150.1± 64.2) ml,mean bladder compliance was (5.2± 3.9) ml/cmH2O,maximal detrusor pressure was (44.1 ± 29.2) cmH2O.The SIS cystoplasty was performed in all patients,which the Surgisis(R) ES was used during operation.In order to enlarge the bladder,the 4-layer tissue graft was fashioned into a strap shape,rehydrated and continuously sutured to the mucous edge of opening bladder walls.Seven patients accepted the ureter reimplantation with anti-reflux technique simultaneously.Serial video-urodynamics,magnetic resonance urography (MRU) and serum analyses were used to assess the outcomes of surgery.Results All patients were undergone the operation successfully.The mean operative time was 120 min.Bowel function returned promptly after surgery.No metabolic consequences were noted.Renal function was preserved.The duration of follow-up ranged from 6 to 48 months (mean 24 months).Compared to the preoperative findings,there were significant increasing in maximum bladder capacity (P＜0.05) at the 6,12 and 24-month follow-up (150.1± 64.2) ml preoperatively,(274.9±88.7) ml at 6 months,(322.5± 144.4) ml at 12 months and (279.9± 157.9) ml at 24 months,The significant differences also exhibited in the increasing of bladder compliance (P＜0.01) at the 24-month follow-up [(5.2±3.9) ml/cmH2O preoperatively,(26.1 ±29.4) ml/cmH2O at 24 months] and decreasing of maximum detrusor pressure (P＜0.05) at the 12 and 24-month follow-up [(44.1±29.2) cmH20 preoperatively,(20.1±9.8) cmH2O at 12 months,(20.2±19.1) cmH2O at 24 months].Two patients were observed scaffold rupture one week postoperatively and healed with patent drainage.One patient developed calculi in neo-bladder at 3-months follow-up and treated with transurethral lithotripsy.Four patients were noted vesico-ureteral reflux at 12-month follow-up.The botulin toxin detrusor injection was used in 2 cases.After indwelling the catheter for 3 months,the symptoms of reflux disappeared.The oral administration of solifenacin (5mg twice per day) and tolterodine (4mg once per day) was used in other 2 patients,who also accepted the catheter indwelling at the same time.The symptom of reflux relieved in only patient.Conclusions SIS can be used effectively as a scaffold for bladder augmentation.Tissue engineering technology provides a potentially viable option for genitourinary reconstruction in patients with neurogenic bladder.

ObjectiveTo evaluate regional and global systolic function of left ventricle (LV) after mitral valve replacement(MVR) of different methods by 2-dimensional strain (2DS).MethodsAccording to the operational method whether preserve the posterior leaflet and its subvalvular apparatus,48 patients who underwent MVR were divided into two groups,the preservation group (group A) and the resection group (group B).Echocardiography was examinated before and after MVR and the apical four-chamber view,two-chamber view and long-axis view of LV were acquired.Regional peak strain (Sp) and global strain (GS) of LV longitudinal movement were analysed by 2DS software.Results①Compared to preoperation,the Sp in basal segment of posterior septum and inferior wall and middle segment of lateral wall in group A increased significantly ( P ＜0.01 or P ＜0.05).The Sp of group B were improved in both basal and middle segments of posterior septum ( P ＜0.05),while declined in middle segment of lateral wall and anterior wall,basal segment of lateral wall and apical segment of anterior wall significantly (P ＜0.01 or P ＜0.05).②Compared with group A,subtractions between preoperative and postoperative Sp of group B decreased in middle segment and apical segment of anterior wall,middle segment of lateral wall and middle segment of inferior wall significantly ( P ＜0.01 or P ＜0.05).③The GS of group A increased significantly ( P ＜0.05),while that in group B tended to reduce with no statistical significance ( P ＞0.05).Compared with group A,subtractions between preoperative and postoperative GS of group B droped significantly (P ＜ 0.05).ConclusionsAppropriate preservation of the posterior leaflet and its subvalvular apparatus has morebeneficial effect in improving the early regional and global function of LV after surgery,which would be recommended in MVR.Early regional and global systolic function of LV after MVR could be accurately evaluated by 2DS relatively,which has the application value of guiding clinical treatment and estimating prognosis.

ObjectiveTo compare orthotopic liver transplantation (OLT)and combined liverkidney transplantation (CLKT) in the treatment of severe hepatitis B.MethodsIn this study 52 patients of severe hepatitis B were allocated to OLT (40 cases) or CLKT( 12 cases) at our department from Jan.2001 to Sep.2005.The perioperative complications and the result of follow-up were analyzed.ResultsThe preoperative renal functions in CLKT cases were severer than that in OLT cases.Postoperative severe infection was more common in CLKT cases than that in OLT cases.In OLT group 28 patients (70％)suffered from early posttransplant renal dysfunction,among them 11 patients needed dialysis,whilst there were 2 (16.7％ ) patients who needed dialysis in CLKT group (P ＜0.01 ).The posttransplant mortality in OLT group was 40％ ( n =16),significantly higher than that in CLKT ( 16.7％,n =2) ( P ＜ 0.01 ).In OLT group,9 cases developed severe renal failure and died.No one died of renal failure in CLKT group.ConclusionsThe prognosis is more favorable to perform CLKT in patients who suffered from severe hepatitis B with chronic renal dysfunction before transplantation.

Objective 　Determination of Wilson disease gene mRNA expression in human fibroblast cell strain (Me32aT22/2L) by reverse transcription-polymerase chain reaction (RT-PCR). Methods　Using lipofection reagent, the plasmid vector carrying the Wilson disease gene (pRc/CMV-WD) was transferred into Me32aT22/2L cultured in serum free complement medium. RT-PCR was used to determine WD mRNA expression in Me32aT22/2L. Results　 Wilson disease gene expression was detected in Me32aT22/2L, while no specific signals were detected in untransfected fibroblast. Conclusions　It demonstrated that Me32aT22/2L strain could express the Wilson disease gene, suggesting that Wilson disease gene transfer might develop a new approach to study Wilson disease.

Objective To evaluate the effect of oxycodone preconditioning on liver injury induced by intestinal ischemia-reperfusion (I/R) in rats and the role of different opioid receptors.Methods Fiftyfour adult male Sprague-Dawley rats, weighing 200-300 g, were randomly divided into 9 groups (n =6 each) using a random number table: sham operation group (group S), group I/R, oxycodone preconditioning group (group OP) , μ receptor antagonist CTOP group (group CTOP) , δ receptor antagonist naltrindole group (group NTD), κ receptor antagonist nor-binaltorphimne group (group BNI), CTOP + oxycodo ne preconditioning group (group CTOP+OP) , naltrindole + oxycodone preconditioning group (group NTD+ OP) , and nor-binaltorphimne + oxycodone preconditioning group (BNI+OP).The model of intestinal I/R was established by occlusion of the superior mesenteric artery for 45 min followed by 2 h reperfusion in anesthetized rats.The superior mesenteric artery was only exposed, but not occluded in group S.In OP,COTP+OP, NTD+OP and BNI+OP groups, oxycodone 0.5 mg/kg was injected intravenously at 10 min prior to ischemia.COTP 1 mg/kg and naltrindole 5 mg/kg were injected intravenously at 20 min prior to ischemia in COTP+OP and NTD+OP groups, respectively.Nor-binaltorphimne 5 mg/kg was injected intravenously at 25 min prior to ischemia in group BNI+OP.In CTOP and NTD groups, the corresponding doses of CTOP and naltrindole were injected intravenously at 10 min prior to ischemia.In group BNI, the corresponding dose of nor-binaltorphimne was injected intravenously at 15 min prior to ischemia.The rats were sacrificed at 2 h of reperfusion, and left hepatic lobes were removed for microscopic examination and for detection of apoptosis in liver cells (using TUNEL).The apoptosis index (AI) was calculated.Results Compared with group S, the AI was significantly increased in the other groups (P＜0.05).Compared with group I/R, the AI was significantly decreased (P＜0.05) , and the pathological changes of livers were reduced in OP, COTP+OP, NTD+OP and BNI+OP groups, and no significant change was found in AI and pathological changes of livers in CTOP, NTD and BNI groups (P＞0.05).Compared with group OP, the AI was significantly increased (P＜0.05), and the pathological changes of livers were aggravated in COTP+ OP, NTD+OP and BNI+OP groups.There was no significant difference in AI and pathological changes of livers among groups COTP+OP, NTD+OP and BNI+OP (P＞0.05).Conclusion Oxycodone preconditioning can mitigate liver injury induced by intestinal I/R in rats, and μ, δ and κ receptors mediate the role with comparable effects.

Objective To investigate the changes and clinical significance of plasma N‐terminal pro‐brain natriuretic peptide (Nt‐proBNP) in patients with asphyxia of newborns combined with myocardial injury .Methods Fifty‐eight term neonates with neonatal asphyxia born within 24 h were included in our study ,18 of the 25 severe asphyxia cases were combined with myocardial injury ,15 of 33 mild asphyxia cases were with myocardial injury ,there were 25 subjects were not cardiac injured after asphyxia to‐tally .Patients of the same age without asphyxia or any cardiovascular disease were recruited into the control group .Levels of plasma Nt‐proBNP and creatine kinase isoenzyme (CK‐MB) were detected on the first ,third and the fifth days after birth for statistical a‐nalysis .Results On the first and third days after birth ,levels of plasma Nt‐proBNP in severe and mild asphyxia combined with my‐ocardial injury group were significantly higher than the groups without myocardial injury and the control (P<0 .05) .Levels of plas‐ma Nt‐proBNP in severe asphyxia myocardial injured group were significantly higher than those of the mild asphyxia myocardial in‐jured ones (P<0 .05) .On the fifth day ,the levels of plasma Nt‐proBNP in severe asphyxia myocardial injured group were signifi‐cantly higher than others (P<0 .05) .There was no difference in Nt‐proBNP levels between mild asphyxia myocardial injured group and the ones without myocardial injury (P>0 .05) .On the first day after birth ,levels of Nt‐proBNP ,CK‐MB were positively corre‐lated in the mild asphyxia group (r=0 .58 ,P<0 .05) and severe asphyxia group (r=0 .47 ,P<0 .05) .On the first day after birth , the cutoff value for myocardial injury was 695 .87 fmol/mL ,the area under the ROC curve was 0 .763 ,with the sensitivity of 71 .9%and specificity of 65 .4% ,the positive predictive value was 73 .3% and negative predictive value was 63 .8% .Conclusion The Nt‐proBNP level has important clinical value for early evaluation of the severity of myocardial injury after asphyxia .

OBJECTIVETo investigate the chemical constituents from roots of Boehmeria nivea.

METHODThe constituents were isolated by repeated column chromatography and preparative liquid chromatography; and their structures were elucidated by chemical properties and spectroscopic analyses.

RESULTSeven compounds were isolated and their structures were identified as tormentic acid (1), hederagenin (2), maslinic acid (3), 2alpha-hydroxyursolic acid (4), trans-p-hydroxycinamic acid (5), 2,4,4'-trihydroxychalcone (6), rutin (7).

CONCLUSIONCompounds 1-6 were obtained from this genus for the first time.

Boehmeria ; chemistry ; Drugs, Chinese Herbal ; analysis ; Oleanolic Acid ; analogs & derivatives ; analysis ; Plant Roots ; chemistry ; Triterpenes ; analysis

To describe the effects of endogenous testosterone levels on the visuospatial ability.Thevisuospatial ability,testosteronewere included as the key words,retrieving 46 papers in the database.35 relevant papers have been reviewed in the paper,as the results,we have found that the visuospatial ability of male was better than female.The performances of the visuospatial tasks in males were associated with the lev-els of endogenous testosterone,and the effects comprised two aspects:the organizational function and activa-tional function of the testosterone.The latter have received more attention from researchers.Until now,most studies of activational effects of testosterone on visuospatial ability remained on the level of behavioral re-search.The mental rotation task,navigation task and virtual reality scenes tasks were implied in these stud-ies.A nonlinear relationship has aroused a great controversy.Latest research suggests that further investiga-tion of the neural mechanisms related to visual-spatial ability is needed,using ERP and MRI techniques,to assess the relationship of endogenous testosterone levels with the relevant neural basis of visuospatial ability.

ObjectiveTo evaluate the clinical efficiency and safety of cefmetazon in the prevention Department of General Surgery,First People's Hospital,Shanghai Jiaotong University,Shanghai 200080,Chinaand or treatment of infections in general surgery. MethodsA multicenter,prospective and open-labeled trial was conducted. In the prevention group,1700 patients were enrolled in clean-infection surgery,cefmetazon was given 1 g iv half an hour before the surgery started,and 1 g iv twice daily after the surgery for 3 days.Clinical response was evaluated in terms of both cure ( disappearance of pre treatment symptoms)and pathogen. In the treatment group,897 patients were diagnosed as peritonitis, cholecystitis and cholangitis,the patients were given cefmetazon 2 g iv twice a day for 7 - 14 days,clinical response and microbiological efficacy were assessed.ResultsIn prophylactic group,1449 patients were finally included.The clinical efficacy was 100％ (1449/1449).In the treatment group,a total of 897 patients were enrolled,and 110 patients failed for assessment of clinical efficacy,787 patients were included in the PPS population,the clinical efficacy was 90.7％ (714/787); Bacterial eradication rate was 92％ (46/50).Adverse reaction rates in prevention group and treatment group were 1.3％ (22/1700) and 1.2％ (11/897),including mild nausea and vomitting.ConclusionsCefmetazon is effective and safe in prevention and treatment of Postoperative infections in general surgery.