Objective To draw up the Chinese Words Reading Test and try to use it as way of premorbid intelligence. Methods 373 health middle-elderly people as study subjects, from five communities in Shanghai city, are between age 55 and 85, and their edueational background is junior high school or above, and their MMSE scores are more than 24 points. 34 of the cases have finished CWRT and Weehsler Adult Intelligence Scale (WAIS) Chinese Revised edition. The main analysis indicators of CWRT: CWRT-Ⅰ for number of right reading 50 characters and CWRT-Ⅱ for 50 words. Results Both CWRT-Ⅰ and CWRT-Ⅱ are relative to education level( r = 0.462,0.489, and P < 0.01), while the correlation with age is not signifieant. Performance of the male participants are better than that of the female. The coefficient of correlation between scores of CWRT-Ⅰ and full IQ of WAIS is 0.79. The mean and standard deviation of scores of CWRT-Ⅰ and CWRT-Ⅱ for the normal elder people with different education levels and gender are provided. Conclusion CWRT is not very diffieuh or easy, and is highly relative to score of WAIS. So it can be used as an evaluating tool for premorbid intelligence.

ObjectiveTo explore the clinical presentation, diagnosis, treatment, and outcome of the Barth syndrome (BTHS).MethodsClinical data were collected and analyzed from 3 patients with conifrmed genetic diagnosis of BTHS from June 2013 to October 2014.ResultsAll of the 3 patients were males and two of them were twins. The main clinical manifes-tations of the 3 patients were cardiomyopathy and heart failure, accompanied by different degrees of trabeculations of the left ventricle. Two of them were diagnosed of left ventricular noncompaction (LVNC). All of the 3 patients presented with motor retardation, muscle weakness, growth delay and signiifcantly increased urinary excretion of 3-methylglutaconic acid (3-MGC). One patient was found to have neutropenia. All 3 patients hadTAZ gene mutations which included a novel missense mutation (c.527A>G, p.H176R) detected in the twins and a known nonsense mutation (c.367C>T, p.R123X) identiifed in the other patient. All of the mutations were inherited from their mothers. During the follow-up, the twins died at 7 months old and 7.5 months old respectively. The other patient was still alive.ConclusionBTHS is one of the causes of cardiomyopathy in children. In the male patients who presented with muscle weakness, neutropenia, and increased urinary excretion of 3-MGC, especially in those com-bined with LVNC, BTHS should be screened.

Objective To investigate the conversion rate of subtypes of amnestic mild cognitive impairment (aMCI) to Alzheimer' s disease (AD) and assess the contribution of neuropsychological disturbance in progression from MCI to AD over 2 years. Method Subjects from memory clinic of Huashan hospital including 130 of who met the operational criteria for Mayo Clinic defined aMCI by neuropsychological tests covering global cognitive function, executive function, memory function, language function and visual spatial skills. They underwent multidimensional assessment and a neuropsychological battery at baseline and at follow-up, after about 2 years. Diagnosis for dementia was based on National Institute of Neurological and Communicative Disorders and Stroke; Alzheimer' s Disease and Related Disorders Association (NINCDS-ADRDA) criteria. Results Forty-four cases of 130 (33. 8% ) elder people with aMCI converted to probable AD with 17 aMCI-s and 27 aMCI-m subjects within an average 23.8 (6. 8 ) months. There was significant difference between the reversion rate of aMCI-s and aMCI-m (26. 2% vs 42. 9%, x2 = 3.957,P = 0. 047). Moreover, as the disease progressing,the two groups declined similarly in memory and executive function while in the aMCI-s group, the function of spatial structure relatively reserved in aMCI-s but function of language and attention diminished faster, and in the aMCI-m group, the ability of spatial structure declined even more significantly. Conclusion The aMCI-m is more likely to progress to AD than aMCI-s and it' s necessary to divide aMCI into aMCI-s and aMCI-m to help determine prognosis.

OBJECTIVEThe Pul-stent is a new cobalt alloys stent, specially licensed for pulmonary artery stenosis. The aim of this study was to investigate the value of the stent implantation as the treatment of postoperative pulmonary artery stenosis.

METHODClinical practice was carried out to evaluate effectiveness, safety and maneuverability of Pul-stent in the defined clinical setting. Transthoracic echocardiography, chest roentgenography and electrocardiography were carried out as follow-up studies at 1 and 3 months after procedures.

RESULTTen patients (7 males and 3 females) received Pul-stent implantation in left or right pulmonary arteries (9 stents in left and 1 stent in right). For 9 cases transcatheter stent implantation was performed and in I case hybrid procedure. The median age was 7. 9 years (range 3. 4 - 13. 7 years). The median weight was 22. 3 kg (range 13 - 32 kg). Among them 6 cases were post surgical repair of tetralogy of Fallot, 2 cases were after Fontan procedures, 1 patient was post surgical repair of pulmonary atresia with ventricular septal defect, and 1 patient had stenosis at Glenn pathway. The pressure gradient at the stenosis decreased from (31. 6 ± 10. 2) mmHg(1 mmHg =0. 133 kPa) to (7. 3 ± 10. 3) mmHg, and the diameter of the narrowest segment increased from (4. 0 ± 2. 3) mm to (9. 6 ± 2. 7) mm, the right ventricle to aortic pressure ratio decreased from 0. 54 to 0. 36, all of these improvements were statistically significant (t = 3. 9, -9. 7, 4. 5; P =0. 008, 0. 000, 0. 004). The total procedure time ranged from 55 to 220 min (median 117 min) , and the fluoroscopy time ranged from 9 to 67 min (median 26 min). There were 2 cases of post-stenting pneumorrhagia. No stent fracture, stent malposition and other severe complications were observed. Initial follow-up of 1 and 3-months showed good results with maintenance of improved caliber of the stented vessel, and the gradient across the stenosis area measured by echocardiography was (32. 0 ± 14. 6) mmHg after 3 months. Compared with before stentifng ((40.6 ± 15. 2) mmHg) and 1 month later ((30. 6 ± 13. 6) mmHg), the difference was not statistically significant (t =2. 2,1. 76; P =0. 07, 0. 10).

CONCLUSIONPul-Stent tracking and delivery was excellent, the initial experience has shown that Pul-stent implantation was effective and safe in treating post-operative branch pulmonary artery stenosis. Further follow-up study should be conducted to make sure whether those good results would be kept constant.

Constriction, Pathologic ; Female ; Follow-Up Studies ; Fontan Procedure ; Humans ; Male ; Postoperative Complications ; Pulmonary Artery ; pathology ; Pulmonary Atresia ; Stents

Objective To evaluate the diagnostic value of invasive cardiac catheterization for restrictive cardiomyopathy (RCM) and constrictive pericarditis (CP).Methods Twenty-seven children with CP or RCM hospitalized in Department of Cardiology,Shanghai Children's Medical Center,School of Medicine,Shanghai Jiaotong University from February 2002 to December 2015,were selected,including 10 patients who had been surgically documented CP and 17 patients with RCM who underwent cardiac catheterization.Intracardiac pressure waveforms were recorded and all the measurement indexes of pressure of all the patients were analyzed.The changes of cardiac pressure under deep breathing in 9 patients with local anesthesia was recorded.Results There were significant differences in pulmonary artery pressure,difference between left ventricular end-diastolic pressure (LVEDP) and right ventricular end-diastolic pressure (RVEDP),and the ratio of RVEDP/right ventricular systolic pressure between group RCM and group CP [(50.2 ± 12.0) mmHg(1 mmHg =0.133 kPa) vs.(38.1 ±6.8) mmHg,(7.8±5.5) mmHgvs.(1.8 ±4.7) mmHg,0.27 ±0.10vs.0.45 ± 0.20,respectively;t =2.912,2.787,2.418,all P ＜ 0.05].However,there was overlapping for these criteria,and the predictive sensitivity of any of the criteria was less than 66.7％.In patients with CP,reciprocal changes in the filling between right ventricle and left ventricle occurred during respiration.In patients with RCM,the right ventricle and left ventricle pressures moved concordantly with respiration.The systolic area index was greater in group CP than that in group RCM (1.20 ± 0.03 vs.0.70 ± 0.14),and the difference was significant (t =6.152,P ＜ 0.01).The systolic area index had a sensitivity of 100.0％ and a predictive accuracy of 100.0％ for the identification of patients with surgically proven CP.Conclusions Measurements in catheterization,especially the ratio of right ventricular to left ventricular systolic area during inspiration and expiration is a reliable catheterization criterion for differentiating CP from RCM.

Objective Patients with single-ventricle physiology pose a wide variety of therapeutic challenges.Pulmonary artery or anastomosis stenosis in single-ventricle physiology will have only subtle hemodynamic derangements due to the lack of the pumping chamber to force blood across the stenosis.This study aimed to evaluate the feasibility and usefulness of the stent implantation for treatment of branch pulmonary artery and anastomosis stenosis in children with univentricular hearts.Methods Retrospective analysis of 8 implanted stents between March 2014 and January 2015 in Shanghai Children's Medical Center.Transthoracic echocardiography,chest roentgenography and electrocardiography were carried out as follow-up studies at the second day after operation and 1,3,6,12 months after the procedure.Results In all of eight patients (6 males and 2 females),5 cases were post-Glenn operation and 3 cases were post-Fontan operation.The median age was 6.6 years (range 4.0-8.5 years).The median weight was 19.5 kg(range 13.8-25.6 kg).9 stents were implanted successfully in all patient,6 in left pulmonary arteries,2 in right pulmonary arteries and 1 in anastomosis.All stents were placed in the target lesion without any complication.The diameter of the narrowed segment improved from (3.63 ± 2.06) mm to (7.89 ± 1.62) mm (P ＜ 0.01).During follow-up no other complication occurred except thrombus in one patient because of discontinuation anticoagulation.Conclusion Branch pulmonary arterial stenosis in single-ventricle patients may often be underestimated due to the low pressure venous system and/or the development of venovenous collaterals bypassing and decompressing the pulmonary circuit.In these patients,even mild stenosis should be treated aggressively,especially in the presence of pleural effusions,pericardial effusions,protein-losing enteropathy and low-output states.Stent implantation is an effective method of treating branch pulmonary artery stenosis.

Objective To study the feasibility and clinical effect of trans umbilical single incision laparoscopic right hemicolectomy for right hemicolon carcinoma.Methods The clinical data of 35 patients with right hemicolon carcinoma were retrospectively analyzed,the 15 cases were received the trans umbilical single incision laparoscopic right hemicolectomy (single incision group) and 20 cases were received laparotomy right hemicolectomy (laparotomy group).The clinical indexes were compared between the 2 groups.Results The length of incision,intraoperative bleeding volume,passage of gas by anus time,feeding time,hospitalized time in single incision group were significantly better than those in laparotomy group [(5.5 ± 0.6) cm vs.(17.6 ± 2.2) cm,(84.0 ± 31.1) ml vs.(155.5 ± 43.1) ml,(2.00 ± 0.76) d vs.(3.75 ± 0.63) d,(5.3 ± 0.6) d vs.(6.5 ± 0.6) d,(9.3 ± 1.4) d vs.(13.5 ± 1.5) d],the operation time in single incision group was significantly longer than that in laparotomy group [(238.4 ± 19.3) min vs.(165.3 ± 25.8) min],there were statistical differences (P ＜ 0.05).There was no statistical difference in number of incision lymph node between the 2 groups (P ＞ 0.05).Postoperative complication in single incision group occurred in 2 cases,postoperative complication in laparotomy group occurred in 3 cases,there was no statistical difference (P ＞0.05).The follow-up time was 1-55 months,the median follow-up time was 28 months,local recurrence and distant metastasis were found in 2 cases in single incision group,and 4 cases was found in laparotomy group,there was no statistical difference (P ＞ 0.05).Conclusions Single incision laparoscopic right hemicolectomy for right hemicolon carcinoma is safe and feasible,which has the advantages of minimal trauma,aesthetic outlook,less bleeding,quick recovery and short hospitalization time,etc.It can be developed in the hospital which has some basis of laparoscopic surgery.

Objectives To explore the clinical symptoms, therapy and prognosis of tachycardia-induced cardiomyopathy (TIC) in children. Methods Clinical data of 22 children with TIC from July 2007 to July 2012 were retrospectively analyzed. Results TIC was mostly seen in male infants and 81.82%of TIC was caused by atrial arrhythmias. The clinical symptom relieved after arrhythmia and ventricular rates were under control with average effective treatment time of (14.00 ± 8.20) days. Ten patients had tachycardia recurrence, 7 of them had atrial arrhythmia and their clinical symptoms were improved after treatment;while 3 of them showed longer time of therapy with average treatment time of (19.50±8.40) days (P<0.05). Five children underwent radiofrequency ablation before school age and got good therapeutic effect. The post-treatment echocardiographic parameters showed cardiac function of TIC children was significantly improved after treatment, including left ventricular end-diastolic diameter index, left ventricular end-systolic diameter index, left ventricular ejection fraction and shortening score (all P<0.05). Conclusions Childhood TIC is a reversible myocardial dysfunction and its prognosis is good. TIC can be induced by various types of tachyarrhythmias and normally by atrial arrhythmia. The preferred treatment of TIC is administration of antiarrhythmic drugs but radiofrequency ablation is needed to ventricular arrhythmias induced TIC.

Objective To explore the clinical value of color Doppler flow imaging(CDFI) combined with quantitative analysis technique to evaluate renal flow perflusion. Methods Twenty patients with different renal diseases matched with healthy control subjects were examined with Philips iU22 CDFI system. CDFI system and Qlab quantitative technique were used to measure renal restrictive index(RI), vascularization index(VI), flow index(FI), vascularization-flow index (VFI). The patients all received renal single-photon emission computed tomography (SPECT) examination. Results The RI of 32 kidney with lower flow perfusion in SPECT were higher than in the contol group (P <0.01). VI,FI, VFI were lower than those in control(VI, VFI, P <0.01 ;FI, P <0.05). Conelusious The renal blood flow perfusion of patients with renal diseases are lower than that of healthy people. It has high clinical value using CDFI combined with quantitative analysis technique to evaluate the renal perfusion of the patients with renal diseases.

Objective To explore the effectiveness and safety of Linezolid (LIZ) in treating children with infective endocarditis (IE).Methods The clinical data of 112 children with IE and treated in the Shanghai Children's Medical Center of Shanghai Jiao Tong University School of Medicine from August 2008 to September 2015 were retrospectively analyzed.There were 64 boys and 48 girls,and the age of IE onset ranged from 1 month to 17 years [(6.0 ± 4.8) years].Twenty-nine patients received LIZ treatment ＞ 7 days (LIZ treatment group),including 21 males and 8 females,and the age ranged from 5 months to 15 years [(6.9 ±5.2) years].The remaining 83 patients were identified without LIZ treatment(non-LIZ treatment group),including 43 boys and 40 girls,and the age ranged from 1 month to 17 years [(5.7 ±4.7) years].The etiological results,curative effect and adverse reactions of the LIZ treatment group were observed.Results Among the LIZ treatment group,22 cases had congenital heart disease and 1 case had intravenous catheter.There were 10 cases with infection of staphylococcus aureus,6 cases with coagulase negative staphylococcus,5 cases with oral streptococci and Streptococcus bovis group,3 cases with streptococcus pneumonia,2 cases with enterococcus faecium and 3 cases with negative blood culture results.All cases of the LIZ treatment group received Vancomycin therapy at first,LIZ was given when the Vancomycin therapy failed(16 cases with temperature reiteration,1 case inadequate microbiological response),Vancomycin intolerance (6 neutropenia,2 renal toxicity,2 allergy),and oral maintenance therapy (2 cases).The duration of LIZ treatment ranged from 9 to 135 days [(39.2 ±27.2) days].Three patients (10.3％) had adverse effects during LIZ treatment,1 case with severe digestive symptoms after treatment,1 case with teeth discoloration,1 case with the indicators decreasing by 2 routine blood test.Meanwhile,16 cases of 85 patients (18.8％) had side effects during Vancomycin treatment,in which 7 cases with neutropenia,6 cases with rash and 3 cases with renal insufficiency.But there were no significant differences in adverse effects between LIZ and Vancomycin treatment (x2 =1.l19,P ＞0.05).Twenty-five cases were cured (86.2％) and 2 cases dead (6.9％) in the LIZ treatment group.And no significant difference was found in cure rate,or mortality between LIZ treatment group and non-LIZ treatment group (86.2％ vs.77.1％,x2 =1.090;6.9％ vs.8.4％,x2 =0.069,all P ＞ 0.05) at 6 to 84 (23.9 ± 19.1) months follow-ups.Conclusions LIZ can be used to deal with Vancomycin failure and IE caused by gram positive coccus.LIZ was generally well tolerated in patients with IE.It may be useful in cases of IE complicated by brain abscesses for the good distribution.It is 100％ orally bioavailable,allowing oral administration for outpatients.