Objective To observe the availability and safety of ventricular septal defect (VSD) occluder in infants and young children with large patent ductus arteriosus (PDA) associated with severe pulmonary hypertension.Methods Five patients (1 male and 4 fomale) of large PDA aged 5 months to 3 years,weighted from 5.1 to 15 kg,body surface area (BSA) 0.37-0.58 m2 underwent transcathter intervention with concentric VSD occluders from June 2008 to May 2009.Arterial ducta were tube-like and their diameters were 5.7 to 8.5 mm,with ulmonary vascular resistance from 4.8 to 5.7 Wood Unit,Qp/Qs 3.4-4.6.Three patients were given Bosentan after intervention.Results The large PDAs were successfully closed with VSD occluders,including 1 concentric perimembranous VSD occluder and 4 muscular VSD occluders.They all discharged 4 to 5 days with hidrosis and weight improved.Echocardiogram indicated VSD occluder was stable,no residue shunt and no stricture of left pulmonary artery and descending aorta were found.According to tricuspid and pulmonary regurgitation,pulmonary arterial pressure decreased differently and returned to normal after 6 months follow-up.Conclusion VSD occluder is available and effective to close large PDA associated with severe pulmonary hypertension in inrants and young children,but more cases and long-term follow-up are necessary.

Objective To assess the damage of limbic system pathways in relapsing-remitting multiple sclerosis (RRMS) by diffusion tensor imaging (DTI) tractography.Methods DTI tractography was used to acquire fiber numbers,fractional anisotropy (FA),mean diffusivity (MD) of cingulum,fornix,and uncinate fasciculus (UF) in 20 RRMS patients (MS group) and 20 healthy volunteers (control group),and statistical analysis was performed.Results Compared with control group,lower FA value (P＜0.01) and higher MD value (P＜0.05) were found in cingulum,fornix and UF of MS group,and the fiber numbers of fornix decreased in MS group (P＜0.001).There were significant differences between the left and the right UF in fiber numbers and FA value of control group and the fiber numbers of MS group.Negative correlations were found between FA value of cingulum and UF and EDSS scores in MS group (r=-0.572,-0.665,both P＜0.05),and positive correlations were found between MD value and EDSS scores (r=0.627,0.603,both P＜0.05).Conclusion DTI tractography is valuable in assessing MS associated limbic system damage and in monitoring the clinical progression of the disease.

Objective Transesophageal echocardiography (TEE) guided, minimally invasive perventricular device occlusion of ventricular septal defects ( VSDs) without cardiopulmonary bypass ( CPB) has been applied in multiple centers. We reported experiences and the mid-term results. Methods Four hundred and thirty-two cases from 4 cardiac centers were involved in the study. There were 235 males and 197 females, aged from 3 months to 15 years, with a body weight varying from 4.0 to 26.0 kg. Three hundred and fifty-one patients had perimembranous VSDs, 57 had intracristal or supracristal VSDs and 24 had muscular VSDs (17 had multiple muscular VSDs). The diameter of the VSD ranged from 3 to 12 (5.3 ±1.6 ) mm.For those with perimembranous or muscular VSDs, a 3 to 5 cm inferior sternotomy was made, but for those with intracristal or supracristal VSDs, a 2 to 3 cm incision was made parastemally through the left third intercostal space. Being monitored and guided with TEE, the device was deployed to occlude the VSD through the puncture at the free wall of the right ventricle. TEE was used for assessing the residual shunting, the left and right ventricular outlet tracts, valvular function and for detecting any arrhythmia, The devices would be released if the heart rhythm was normal, as well as the residual shunting and valvular regurgilalion were not detected. Results The procedure was completed successfully in 417 cases(96.5% ) and converted to traditional surgical closure with CPB in the other 15 cases(3.5% ). Concentric devices were used in 238 cases(57.1% )and eccentric devices were used in 179 patients(42.9% ). Successful procedures finished in less than 90 minutes, and the deployment and evaluation of the devices were completed in 5 to 60 (18. 2 ± 8.6) minutes. No residual shunt and detectable aortic or tricuspid insufficiency and arrhythmia was observed. Patients were extubated within 2 hours and discharged 3 to 5 days after the operation. During fellow-up period from 3 months to 2 years, no clinically significant complications occurred. Conclusion The minimally invasive device closure of VSD under TEE guidance without CPB is proved to be a simple, safe and effective treatment for a considerable number of children with VSD. Its use in the clinical practice should be encouraged.

Objective To analyze the clinical characteristics and prognostic factors of elderly patients with cytogenetically normal acute myeloid leukemia (CN-AML). Methods A total of 104 initial CN-AML patients were enrolled in this retrospective study. The clinical characteristics were collected and analyzed retrospectively. Factors affecting complete remission (CR) were analyzed by using chi square test. Univariate and multivariate analyses of prognostic factors were performed by using Kaplan-Meier and Cox hazard regression model respectively. Results After the first chemotherapy, 72 of 104 patients were able to be evaluated the efficacy, the CR rate was 38.9%(28/72) and total response rate was 55.6%(40/72). The white cell count<100 × 109/L and NPM1 mutation were related to a higher CR rate [59.4%(38/64) vs. 12.5%(1/8), 83.3%(10/12) vs. 36.4%(8/22), P<0.05]. Among 104 patients, the median overall survival (OS) was 6.9 months. Univariate analysis results demonstrated that age≥70 years, secondary AML, white cell count≥100×109/L, FLT3-ITD mutation, CD7 expression, achieving CR beyond 2 cycles of induction therapy and CCI score≥2 were influence factors on OS. In multivariable analysis, FLT3-ITD mutation (HR=7.61, 95%CI 1.80-32.11, P= 0.006) and achieving CR beyond 2 cycles of induction therapy (HR= 10.11, 95 % CI 2.38-43.03, P=0.002) were independent prognostic factors for OS in elderly patients with CN-AML. Conclusion The prognosis of elderly patients with CN-AML is the result of the combined effect of many factors, FLT3-ITD mutation and achieving CR beyond 2 cycles of induction therapy are independent prognostic factors in elderly patients with CN-AML.

0bjective To synthesis evidence of self-efficacy-based interventions to improve self-management and health behaviour change in patients with diabetes. Methods PubMed, Cochrane, MEDLINE, CINAHL, EBSCO and Web of Science were searched from 1977 to August 2018. The primary outcomes were self-efficacy and self-care ability. Pooled effect sizes of standardised mean difference ( SMD ) were calculated if outcome measurements and the units were different, and if outcome measurements and the units were the same, such as measuring blood glucose by HbA1c, weighted mean difference ( WMD ) were used to calculate pooled effect sizes. Results Nine eligible randomised control trials (RCTs) including 1578 participants were identified. The results showed that interventions based on self-efficacy significantly improved patients′self-efficacy ( SMD=0.69, 95% CI :0.58 to 0.80, P＜0.05) and self-management ( SMD=0.93,95% CI : 0.74 to 1.12, P＜0.05), helped them control blood glucose ( WMD=-0.69, 95% CI :-0.85 to-0.52, P＜0.05), reduced hospitalisation and emergency times, and improved their quality of life ( SMD=0.52,95% CI : 0.32 to 0.72, P＜0.05). Conclusions Even though the results showed significant improvement in self-efficacy and self-management after interventions, the conclusion needs to be generalised with caution because of the quality of RCTs and high heterogeneity. Future trials could consider RCTs with high quality, appropriate sample size, specific component of diabetes management, and reliable and valid scales or objective measures as outcomes.

Objective: To construct a novel tissue engineering complex, BMSCs sheet-RADA16 scaffold, by combining cell sheet and self-assembled peptides.. Methods: The self-assembled peptide RADA16 scaffold was wrapped with the BMSCs cell sheet. The morphology of the cells and the complex were observed by SEM and confocal laser microscopy, and the proliferation of cells was assessed by CCK-8. The osteogenic differentiation of BMSCs was examined by detection of related gene expression with RT-PCR. Results: Compared with the BMSCs cell sheet, the numbers of cells on RADA16 scaffold growth rapidly at 3 rd-8 th day, and BMSCs were more on the scaffold than those on the cell sheet (P＜0. 05) . RT-PCR results showed that the expression level of osteogenesis-related genes was higher in the complex (P＜0. 05) . Conclusion: BMSCs Sheet-RADA16 Scaffold may promote proliferation and osteogenic differentiation of BMSCs.

Objective:To summarize the experience of arterial duct (AD) stenting in children with ductus-dependent hypoplastic right heart syndrome (HRHS).Methods:Seven children including 4 cases of pulmonary atresia with intact ventricular septum (PA-IVS) with HRHS and 3 cases of critical pulmonary stenosis (CPS)-IVS with HRHS underwent AD stenting in Qingdao Women and Children′s Hospital between January 2012 and January 2019. During the same period, 9 patients of PA-IVS with HRHS received Blalock Taussig (B-T) shunt. Two groups of children on the operation time, hospital stay time, intensive care time and mortality were compared. T test or Mann-Whitney U test was used for comparison between the two groups. Results:There was no significant difference in the age (18 (7-100) vs. 17 (1-142) d, U=31.000, P>0.05) and weight ((3.8±1.1) vs. (3.7±1.3) kg, t=0.272, P>0.05) between the AD stenting group and the B-T group.The operation time ((108±7) vs. (160±49) min, t=-4.304), intensive care time ((3.4±1.0) vs. (6.3±4.5) d, t=-8.692) and total hospitalization time ((10.3±1.0) vs. (26.3±1.0) d, t=-7.822) in the AD stenting group were differed significantly compared with the B-T group (all P<0.05). The transcutaneous oxygen saturation improved significantly (0.723±0.125 vs. 0.926±0.005, t=-6.044, P<0.05) after AD stenting. The diameter of AD stent ranged from 3.5 to 4.0 mm, and the length of AD stent was 16-21 mm. There were no complications such as vascular injury, acute thrombus, catheter spasm and death in the AD stenting group. The mortality of children in the B-T group was 3 in 9 cases. Three cases in the AD stenting group received pulmonary valvulotomy and bilateral Glenn operation at 6, 9 and 9 months after AD stenting, respectively. Conclusions:AD stenting is a feasible, effective, safe and minimally invasive procedure for children with ductus-dependent HRHS. It can even be used as an alternative to B-T shunt.

Objective:To explore the value of current indications for fetal pulmonary valvuloplasty (FPV) by summarizing the postnatal diagnosis, treatment, and prognosis of fetuses with pulmonary atresia with intact ventricular septum (PA/IVS) and right ventricular hypoplasia (RVH).Methods:This prospective study was conducted at the Heart Center of Women and Children's Hospital, Qingdao University from September 2018 to March 2021, which included pregnant women who were (1) with fetal PA/IVS and RVH; (2) unable to receive FPV due to fetal position or gestational age despite the indications; (3) given integrated pre- and postnatal management. Prenatal fetal echocardiography assessment, postnatal diagnosis, treatment, and follow-up were summarized using Wilcoxon matched-pair signed-rank test.Results:A total of 35 singleton pregnant women were diagnosed with fetal PA/IVS and RVH by ultrasonic cardiogram and admitted during the study period. Among the 28 fetuses meeting the FPV indications, 18 underwent FPV, while the other 10 did not due to inappropriate fetal position or gestational age. After excluding four terminated pregnancies, the rest six cases were enrolled. The median gestational age at the initial prenatal fetal echocardiography diagnosis was 28.9 weeks (28.3-30.4 weeks). Compared with the initial evaluation, the fetal right ventricular to left ventricular length/diameter ratio [0.8 (0.6-0.9) vs 0.6 (0.5-0.8)] and tricuspid regurgitation velocity [4.7 m/s (3.2-5.1 m/s) vs 4.1 m/s (3.3-4.8 m/s)] were increased, while tricuspid valve Z value [－0.8(－1.6-0.8) vs 0.4 (－0.3-1.9)] and single-ventricular predictive score [0.5 (0.0-2.0) vs 2.0 (1.0-3.0)] were decreased when re-evaluated six weeks later ( T were－2.21, 2.00,－2.20, and 2.00; all P<0.05). All of the six fetuses were born alive with a median gestational age of 38.9 weeks (37.3-40.1 weeks). The median weight was 3 425 g (3 100-4 160) g after being transferred to cardiac intensive care unit. The median age was 12.5 d (0.0-20.0 d) at the first surgical intervention. The median follow-up duration was 15 months (11.8-18.5 months). At initial diagnosis, the single-ventricular predictive score was 1-2 points in four fetuses, and =3 points in two fetuses. There was no death during follow-up. Four patients achieved anatomical biventricular circulation, one achieved clinical biventricular circulation, and one still needed further follow-up, with single-ventricular predictive score at initial diagnosis of 1-3, 3, and 2 points, respectively. Conclusions:The prognosis is good in fetuses with PA/IVS and RVH who have FPV indications but do not receive intrauterine intervention, which suggests that the current FPV indications may be too broad, and a more suitable FPV indication need to be further explored given the difficulty of implementing FPV.

Objective To assess the changes of white matter microstructure in neuromyelitis optica spectrum disorders (NMOSD) with DTI based on tract-based spatial statistics (TBSS) method.Methods Conventional MR and DTI were performed in 20 NMOSD patients (NMOSD group) and 20 healthy volunteers (control group).DTI data were analyzed with TBSS procedure,which was a part of FSL software packages,and non-parametric statistical analysis was performed on the whole brain.Correlation between FA value of tracts with significant difference in NMOSD group and expanded disability status scale (EDSS) scores was analyzed.Results TBSS analysis revealed significantly (P＜ 0.05,FWE corrected) extensive decrease of FA value in cerebrum and cerebellum white matter fiber bundles,i.e.corpus callosum,fornix,corticospinal tract,unciform fasciculus,cerebellar peduncles etc.in NMOSD group,and decrease of AD value,increase of RD value in cingulum bundle,corpus callosum and fornix,while MD value only increased in fornix and retrolenticular part of the left internal capsule (P＜0.05,FWE corrected).Negative correlations were found between FA value of left uncinate fasciculus,right external capsule,left inferior cerebellar peduncle,bilateral anterior limb of internal capsule,medial lemniscus and EDSS scores (all P＜0.05).Conclusion Widespread white matter damage is observed in cerebrum and cerebellum in NMOSD patients by using TBSS analysis,which may partly correspond to the disabilities of NMOSD patients.However,the other microstructural changes of white matter tracts may suggest complicated pathological mechanism of NMOSD.

Objective:To review the clinical outcomes following perinatal multidisciplinary diagnosis and treatment of fetal D-transposition of great arteries (D-TGA).Methods:This retrospective analysis involved 37 fetuses (two fetuses were one of the twins) with D-TGA that were diagnosed by prenatal ultrasound at the Women and Children's Hospital, Qingdao University from January 2016 to December 2020. All the subjects received perinatal multidisciplinary diagnosis and treatment, from the Departments of Fetal Medicine, Genetics, Obstetrics, Ultrasonography, Pediatric Cardiology, Neonatology, etc., and the outcomes were described and summarized.Results:The detection rate of D-TGA was 0.059% (37/62 413), among which intact ventricular septum with D-TGA accounted for 56.8% (21/37) and ventricular septal defect with D-TGA for 43.2% (16/37). All the 37 cases were observed with normal nuchal translucency and four of them were at high risk in fetal Down syndrome screening. All the 31 cases who received non-invasive cell-free fetal DNA screening had normal results and two of 26 cases who received amniocentesis for karyotype analysis and chromosome microarray analysis were abnormal. In terms of pregnancy outcome, 19 pregnancies (51.4%) were terminated, of which 10 cases were terminated for medical reasons and others for non-medical reasons, and 18 cases gave birth to alive body (48.6%, 18/37). Postnatal ultrasound re-examination of one neonate revealed D-TGA with ventricular septal defect, patent ductus arteriosus, and bicuspid pulmonary valve malformation and severe hypoxia and acidosis occured. The patient was discharged after withdrawing treatment and was lost to follow-up. The other 17 neonates all underwent successful surgical treatment with a mean age of (10.2±6.0) d and length of hospital stay of (26.3±9.3) d. Postoperative follow-up (3.3±1.2) years showed all with good cardiac function.Conclusion:Perinatal multidisciplinary diagnosis and treatment of D-TGA can improve the success rate of postnatal treatment and prognosis.