Objective To studiy the surgical outcomes of children with congenital aortic stenosis (AS),and summarized the experience of surgical procedures.Methods From February 2006 to November 2011,a total of 49 consecutive children with AS underwent surgical treatments.Twenty-nine patients were male and 20 patients were female.The median age was 17 months (1 month to 14 years),and median weight was 15.6 kg (3.2-47.0 kg).Peak AS gradients was (74.9 ±20.4)mmHg (45-123 mm Hg) before surgery.Fourteen patients had isolated congenital AS,4 had combined moderate-to-severe aortic insufficiency (AI).Thirty-Three patients had associated cardiac anomalies,including ventricular septal defect,atrial septal defect,patent ductus ateriosus,coarctation of aorta,subaortic stenosis,and supravalvular mitral stenosis.Thirty-one patients underwent aortic valvotomy,9 valvuloplasty,2 Aortic valve replacement (AVR),1 Ross procedure,2 Ross-Konno procedure,and 4 Konno/AVR.Median follow-up was 20 months (2-55 months).Clinical and echocardiographic follow-up data were analyzed.Results There was 1 postoperative death who died of heart failure due to severe mitral valve insuffciency.Latest follow-up data showed that the survivals performed in satisfactory cardiac function.Left ventricular ejection fraction (LVEF)was 0.69 ±0.10,and fraction shortening (FS) was 0.38 ±0.09.Peak AS gradients decreased significantly after surgery to (38.6 ± 15.8) mm Hg (20-73 mm Hg),P ＜ 0.001.One patient needed reintervention because of severe recurrence of adhesion in the commissure 3 months after the previous surgery.Totally 31 patients underwent simple valvotomy procedure.Postoperative AI was none or trace in 5,mild in 20,and moderate in 6.Two surgical techniques were used to repair the aortic valve according to the anatomy of the valve and the cause of insufficiency,including commissuroplasty in 6 and pericarial casp extension in 3.Nine patients underwent valvuloplasty procedure,post-operative AI was none or trace in 3,mild in 5,and moderate in 1.We put more attention to the 7 patients who had moderate AI valvotomy or valvuloplasty,and the latest follow-up showed that these patients were in good condition and there was no deterioration the degree of AI.Conclusion Long-term outcomes show that aortic valvotomy and valvuloplasty are reliable and effective approaches for congenital AS in children.When moderate-to-severe AI exists,other procedures such as Ross,Ross-Konno,AVR,or Konno/AVR are preferred.

Objective A retrospective review of the use of delayed sterna closure (DSC) after pediatric cardiac operations.The purpose of this study is to access the morbidity of DSC and to analyze the risk factors that may predict the need for DSC.Methods Between January 2008 and December 2011,5 546 infants (2 843 males,2 703 females) underwent cardiac operations through midterm sternotomy in Shanghai Children' s Medical Center.Median age was 5 months(1 day to 11 months) and mean weight was 6.1 kg(2.0-12.5 kg).The pathologies included transposition of great artery(TGA),corctation of aorta (CoA),interruption of aortic arch (IAA),pulmonary atresia (PA),truncus arteriosus (Truncus),complete atrioventricular septal defect(CAVSD),total anormalous pulmonary vein connection (TAPVC),double outlet of right ventricle(DORV),tetralogy of Fallot(TOF),and ventricular septal defect(VSD),et al.All hospital records were reviewed and clinical data were studied and analyzed.Results A total of 313 patients had DSC (5.6％).There were 191 males and 122 females.The reasons for DSC are hemodynamic instability in 296,bleeding in 11,and ECMO(or VAD) in 6 patients.Two hundred and fiftyone (80.2％) patients were less than 5 kg,84 (26.8 ％) patients were neonates,and 125 (39.9％) patients aged between one and three months old.The incidences of DSC for these patients were 13.9％,34.4％,and 18.4％ respectively.The diagnosis of complex congenital heart defects had a high risk of DSC.The incidences of DSC for TGA,CoA,IAA,PA,Truncus,CAVSD,TAPVC,and DORV were 28.8％,17.8％,43.1％,9.0％,30.4％,6.5％,and 10.8％ respectively.The DSC patients had longer duration of CPB time(147 min versus 72 min) and clamping time(81 min versus 40 min).In 282 patients the sternums were closed 1-11 days after the initial operation.In 3 cases the trial of closure failed for the first time and succeeded a second time until several days later.Fouty-six patients died ultimately including 15 patients died after the closure of sternum with a total mortality of 14.7 ％.Surgical site infection occurred in 17 patients (5.4％),and surgical intervention were needed in 4 severe cases(1.3％).Conclusion Neonates,an age less than 3 months,weight less than 5 kg,long CPB time and clamping time,and the diagnosis of complex congenital heart defects were risk factors of the need for delayed sterna closure after pediatric cardiac operations.

Objective The purpose of this study was to assess the value of prenatal ultrasound diagnosis for fetal ear auricle malformations.Methods The coronal and sagittal planes of fetuses ears were obtained prospectively in 6239 singleton fetuses in the First Affiliated Hospital of Shantou University Medical College for the period from 2012 February to 2015 December,the ultrasound images and pregnancy outcomes were analyzed in 11 cases of fetuses ear auricle malformations diagnosed prenatally.Results Eleven Cases of fetuses ear auricle malformations include with 7 cases of microtia,3 cases of low-set ears and 1 case of anotia.Eleven cases were combined with other structural malformations were as followings,3cases with craniocerebral congenital malformation,5 cases with dentofacial deformity,5 cases with malformation of heart,3 cases with limb deformity.Cordocentesis was performed in 7 cases among which 6 with abnormal karyotype,including 2 cases of trisomy 21,2 cases of trisomy 13,2 cases of trisomy 18,1 case of 22ql 1 abnormalities.Compared with the postpartum facial examination,prenatal ultrasound correctly diagnosed 10 cases of fetal ear auricle malformations,missed diagnosis 1 case of microtia.Conlusions Fetus with ear auricle abnormalities have characteristic prenatal ultrasound imaging;prenatal ultrasonography can provide reliable information in the diagnosis of this disease.This study suggests that antenatal ear auricle length measurements might be a promising sonographic screening method for the detection of abnormal karyotype in pregnancy.

OBJECTIVE To investigate the deafness-related gene mutation frequency and hotspots in patients of Fuzhou city with non-syndromic hearing loss (NSHL). METHODS Peripheral blood samples were obtained from 88 cases of patients with hearing loss after clinical history inquiry and clinical examination. Their genomic DNA was extracted from peripheral blood by extraction kits to undergo polymerase chain reaction, traditional capillary electrophoresis sequencing and High-throughput sequencing so as to detect the mutations of deafness-related gene. RESULTS Among the 88 patients with NSHL, the gene mutation frequency was 34.09%.In the patients, 14 cases had mitochondrial 12 S rRNA mutations, six cases had GJB2 gene mutations and three cases had SLC26A4 mutations, two cases had MYO15A mutations, the other five cases had MYO7A, OTOF, TECTA, TMC1 and ILDR1 gene mutation respectively. CONCLUSION Among the 88 patients with NSHL, the most frequent mutation causing hereditary deadness was mutation in mitochondrial 12 S rRNA, followed by GJB2 and SLC26A4, The other genes such as MYO7A, OTOF, TECTA, TMC1 and ILDR1 gene were infrequent. The study could provide theoretical reference in genetic diagnosis, prevention and cure of hearing loss.

Objective To determine the serum concentration of MCP-1 and the expression of MCP-1 protein in the pancreas in the piglet with chronic obstructive pancreatitis and to explore the role of MCP-1 protein in pancreatic fibrosisits.Methods The piglet model of chronic obstructive pancreatitis was established by incomplete ligation of the pancreatic duct.The piglets were sacrificed at 4, 6, 8 weeks after induction.Pathological changes of pancreas were examined.Pancreatic fibrosis was assessed by VG staining.Serum MCP-1 concentrations were detected by ELISA method.MCP-1 and α-SMA, PDGF, TGF-β1 and NF-κB protein expression were detected by immunohistochemistry.Results The induction was successful in 14 piglets ( 58.3% ).Mild atrophic changes, interstitial fibrosis, chronic inflammatory cell infiltration could be observed in the body and tail of pancreas from the 4th week in the experimental group.The most obvious changes occurred in the 8th week.Stage Ⅰ pancreatic fibrosis occurred in 5 piglets (35.7%), stage Ⅱ in 4 piglets (28.6%), stage Ⅲ in 5 rats ( 35.7% ).Seurm MCP-1 at 4, 6, 8 weeks was ( 102.44 ± 36.25 ) pg/ml,(97.84 ± 28.67) pg/ml, ( 94.32 ± 28.42 ) pg/ml, respectively, and was significantly higher than that in control group [ ( 10.42 ±5.86) pg/ml, (8.58 ±4.86) pg/ml, (8.22 ±4.58) pg/ml, P ＜0.01 ].There was no MCP-1 protein expression in the control group;MCP-1 protein was detected in the successful induction group, and MCP-1 expression was positively correlated with expressions of the PDGF, TGF-β1, α-SMA and NF-κB.Conclusions MCP-1 may play an important role in the course of pancreatic fibrosis in chronic obstructive pancreatitis.

Objective To correlate dynamic parameters at contrast enhanced CT and interstitial fibrosis grade of non-small cell lung cancer (NSCLC). Methods Twenty-nine patients with NSCLC were evaluated by multi-slice CT. Images were obtained before and at 20,30,45,60,75,90,120,180,300,540,720,900 and 1200 s after the injection of contrast media, which was administered at a rate of 4 ml/s for a total of 420 mg I/kg body weight. Washout parameters were calculated. Lung cancer specimens were stained with hematoxylin-eosin stain and collagen and elastica double stain. Spearman test was made to analyze correlation between dynamic parameters and interstitial fibrosis grade of tumor. Results Twentynine NSCLC demonstrated washout at 20 min 12. 1 (0. 32-58.0 ) HU, washout ratio at 20 minutes 15.3% (0. 3%-39.2% ), slope of washout at 20 minutes 0. 0152 %/s ( 0. 0007%/s-0. 0561%/s ).Interstitial fibrosis of 29 lesions was graded as grade Ⅰ (10), grade Ⅱ (14) and grade Ⅲ (5). There were significant correlation between washout at 20 min ( r = - 0. 402, P ＜ 0. 05 ), washout ratio at 20 min ( r =-0.372,P＜0.05), slope of washout ratio (r = -0.459,P ＜0.05) and interstitial fibrosis grade in tumors. Conclusion NSCLC washout features at dynamic multi-detector CT correlates with interstitial fibrosis in the tumor.

Objective To explore risk factors of cardiac surgery in neonates with low body weight.Methods Retrospective analysis was made in neonates weighing less than 3 kg at surgery(n =192;group 1) and 3 to 6 kg(n =517;group 2),who had undergone open cardiac repairs from January 2006 to December 2015 at our institution.Patients were grouped according to the primary procedure performed and analyzed according to their weight at the time of surgical intervention.Patients were also analyzed according to preoperative risk scores.Univariate versus multivariate risk analysis was performed.Results Hospital early mortality in group 1 was 25.0％ (n =48) versus 14.5 ％ (n =75) in group 2 (P =0.001).Compared with group 2,neonates in group 1 had a significantly higher mortality for simple arterial switch procedure.Lower body weight remained strongly associated with mortality risk after stratifying the population by preoperative risk scores category levels 2.Within group 1,age,weight at surgery,preoperative risk score and type of procedure were not associated with significant differences in early mortality.Cardio-pulmonary bypass time,aortic cross-clamp time and the presence of a surgical complication were independent risk factors for early mortality in group 1.Conclusion Among neonates weighing less than 3.0kg who underwent open cardiac surgery,perioperative hemodynamic status,reflected by bypass time,cross-clamp time,and surgical complications,strongly influenced early mortality.In contrast,low body weight itself was not associated with early mortality.

Objective To analyze the clinical effect of pedicle screw fixation in the treatment of multi-segmental lumbar fracture and dislocation under the guidance of visualization technique. Methods A total of 21 patients with multi-segmental lumbar fracture and dislocation were selected from November 2012 to November 2013. Before the screw implantation, the structure of bilateral pedicle was observed through Mimics software and the implantation parameters were measured. The position of pedicle screws by postoperative CT scan, operation time, and the satisfaction of the patients were assessed. The percentages of anterior vertebral height and Cobb′s angle were measured before operation, 2 weeks and 8 months after operation. Results All patients were satisfied with informed consent score and the way of pedicle screw and the selection of plant were more reasonable. With better screw position, shorter operative time and less blood loss and adverse reactions, pedicle screw fixation achieved good effect. Conclusion With high security and considerable clinical value, pedicle screw fixation in the treatment of multi-segmental lumbar fracture and dislocation under the guidance of visualization technique has exact and good effecct.

Objective A retrospective study of patients in the past ten years who underwent surgeries because of secondary subaortic stenosis(SSS) after previous repair of congenital heart disease(CHD) was performed.The anatomy,indication,and surgical outcomes were studied.Methods Between January 2004 and October 2013,forty-eight patients underwent surgeries because of SSS.Thirty-five patients were males,and 13 were females.The mean age was(54.0 ± 34.9) months (12-156 months).The mean weight was(16.1 ±6.2)kg(6.0-37.5 kg).And the mean interval between the first and second surgery was(39.6 ±25.0) months(11-115 months).The primary diagnosis of CHD included ventricular septal defect,double outlet of right ventricle,corctation,interruption of aortic arch,patent trucus arteriosus,atrio-ventricular defect,tetralogy of fallot,transposition of great arteries,and univentricle.Results Twenty-five patients were discrete subaortic stenosis,and had the fibromuscular ridge resected.Twenty-three patients were diffuse type of subaortic stenosis.Myectomy,reconstruction of the intraventricular baffle,and modified Konno procedure were done in 8,5,and 10 patients separately.One patient died of cardiac failure postoperatively.One developed complete left bundle branch block,and another one developed complete atrioventricular block.The pressure gradients dropped from(76.8 ±20.4)mmHg to(12.4 ±8.4)mmHg,P ＜0.001.One patient was lost in follow-up and the left 46 patients had been followed up for 27 ～ 144 months [mean (59.6 ± 28.3) months] with no late death.The recent echocardiography results showed that the pressure gradients were(16.3 ± 15.2) mmHg.Five patients required reoperations due to recurrence of stenosis and the freedom from reoperation was 82.8％ at 10 years.Conclusion Earlier diagnosis and intervention is recommended for SSS,and excellent relief can be achieved.

Small proteins specifically refer to proteins consisting of less than 100 amino acids translated from small open reading frames (sORFs), which were usually missed in previous genome annotation. The significance of small proteins has been revealed in current years, along with the discovery of their diverse functions. However, systematic annotation of small proteins is still insufficient. SmProt was specially developed to provide valuable information on small proteins for scientific community. Here we present the update of SmProt, which emphasizes reliability of translated sORFs, genetic variants in translated sORFs, disease-specific sORF translation events or sequences, and remarkably increased data volume. More components such as non-ATG translation initiation, function, and new sources are also included. SmProt incorporated 638,958 unique small proteins curated from 3,165,229 primary records, which were computationally predicted from 419 ribosome profiling (Ribo-seq) datasets or collected from literature and other sources from 370 cell lines or tissues in 8 species (Homo sapiens, Mus musculus, Rattus norvegicus, Drosophila melanogaster, Danio rerio, Saccharomyces cere-visiae, Caenorhabditis elegans, and Escherichia coli). In addition, small protein families identified from human micro-biomes were also collected. All datasets in SmProt are free to access, and available for browse, search, and bulk downloads at http://bigdata.ibp.ac.cn/SmProt/.