AIM: To investigate association between children interleukin 1?(-511) gene polymorphisms and susceptibility to febrile seizures. METHODS: Children were devided into two groups: those with febrile seizures (FS) and healthy control, FS group was also devided into family history group and non-family history group. Single nucleotide polymorphisms of IL-1?-511 gene in promoters were detected by PCR-RFLP among these groups. RESULTS: Genotypes for IL-1? promoter -511 (T-T, T-C, C-C) in total febrile seizures and healthy controls were not significantly differen. Genotype IL-1?-511(T-T) in family history patients is 46.2% ,which is much higher than those in non-family history group (10.3%) and healthy control group (5.7%). The genotype proportions for IL-1? promoter-511 in family history group were markedly different from those in non-family history patient group and control group (P

Objective To evaluate the difference of tracheobronchial stenosis and airway malformation between double aortic arches and pulmonary artery sling in children. Methods Clinical feature and imaging data of spiral CT were retrospectively analyzed in children with double aortic arches or pulmonary artery sling who was hospitalized from July 2010 to July 2015 . Results There were 16 children ( 11 males and 5 females) with double aortic arches whose median age at onset was 3 . 5 months old. There were 47 children ( 28 males and 19 females) with pulmonary artery sling whose median age at onset was 4-month-old. In these 16 cases of double aortic arches, 14 cases were coupled with tracheobronchial stenosis. Two cases had more than one segment involved and they were segments II or III. Twelve cases had only one segment involved. Different degrees of tracheobronchial stenosis occurred in 47 cases of pulmonary artery sling, among whom 27 cases had more than one segment involved and the common segment was II and III ( 19 cases). Two cases had four segments involved and 20 cases had only one segment involved. There was statistical signiifcance in the number of involved segments between children with double aortic arches and pulmonary artery sling (χ2=13 . 588 , P=0 . 001 ). In 16 cases of double aortic arches, one case was combined with tracheal bronchus, and 1 case was combined with pulmonary hypoplasia. In 47 cases of pulmonary artery sling, 8 cases were combined with bridging bronchus, 3 cases combined with tracheal bronchus, 3 cases combined with pulmonary hypoplasia, 5 cases combined with bronchial deifciency or tracheal diverticula, and 2 cases combined with bronchopulmonary foregut malformation. There was statistical signiifcance in the number of cases combined with airway malformation between children with double aortic arches or pulmonary artery sling (χ2=5 . 333 , P=0 . 021 ). Conclusions The tracheobronchial stenosis and pulmonary airway abnormalities are more prominent in children with pulmonary artery sling than those in children with double aortic arch.

Objective To discuss the value of color Doppler echocardiography in diagnosis of scimitar syndrome .Methods The echocardiographic results of 6 patients with a diagnosis of scimitar syndrome were reviewed retrospectively .Their sonographic and hemodynamic characteristics were also analyzed connected with the reports in the literature .Results Three cases had dextrocardia and the others had mesocardia .All cases got right ventricular dimension enlargement .Total or partial of right pulmonary venous connection to the inferior vena cava were 3 cases respectively .All cases had right pulmonary artery hypoplasia .All of 6 cases echocardiographic results were in accordance with the findings by CT angiography and 4 cases were confirmed by operation .Conclusions The sonographic features of scimitar syndrome were obvious ,and echocardiography was contribute to early diagnosis of scimitar syndrome .

Objective To explore the diagnostic value of transthoracic echocardiography (TTE) for anomalous origin of right pulmonary artery (AORPA).Methods Echocardiographic data of 26 patients with AORPA proved by surgical operation were analyzed retrospectively.Results TTE showed that the pulmonary trunk and the left pulmonary artery were displayed normally,and the right pulmonary artery originated from the ascending aorta.AORPA in all 26 patients were diagnosed,and the coincidence rate was 100％.Among 26 patients,9 associated with Berry's syndrome,1 combined with coarctation of aorta,22 combined with patent ductus arteriosus,23 combined with atrial septal defect or patent foramen ovale,25 were found with severe pulmonary artery hypertension,and the diagnostic accuracy rate of TTE was 100％ (26/26),100％ (26/26),96.15％ (25/26),92.31％ (24/26) and 100％ (26/26),respectively.Conclusion TTE can early and accurately diagnose AORPA,also has high accuracy in diagnosis of other concomitant malformations.Multi-section scan can reduce misdiagnosis.TTE can be taken as the preferred inspection method for diagnosing AORPA.

Purpose Unilateral absence of pulmonary artery is a rare congenital pulmonary arterydysplasia whose untypical symptom often leads to missed diagnosis or misdiagnosis.Diagnostic and follow-up values of ultrasound cardiogram to pediatric congenital unilateral absence of pulmonary artery (UAPA) are explored in this paper.Materials and Methods Ultrasound cardiogram images of 35 UAPA patients confirmed by surgery or CT angiography in Guangzhou Women and Children's Medical Center from May 2009 to July 2016 were analyzed retrospectively,and images characteristics of the correctly diagnosed and missed diagnosis or misdiagnosed ultrasound cardiogram were analyzed.Results Among these 35 UAPA patients,21 cases were on the right side,14 cases on the left side,16 cases single UAPA and 19 cases combined with other intracardiac malformations.27 cases were initially diagnosed by ultrasound cardiogram,7 cases were missed diagnosed and 1 case misdiagnosed,with 77.1％ diagnosis accuracy.UAPA ultrasound cardiogram showed that normal bifurcation structure of pulmonary artery disappears.Main pulmonary artery continued to be one-side pulmonary artery,running to left or right and forming left branch or right branch of pulmonary artery.Pulmonary artery of the one-side absence of pulmonary segment supplied blood through aorta or other branches or vessels on the other side.After receiving unilateral pulmonary artery reconstruction surgery,ultrasound cardiogram of 8 patients of one month indicated that average flow rate of close-end anastomosis was (1.22± 0.17) m/s and far-end (2.17± 0.56) m/s.Conclusion Ultrasound cardiogram is efficient for UAPA preoperative diagnosis.Meanwhile,it is convenient,noninvasive and efficient in assessing anastomosis and pressure of pulmonary artery after surgery.

Objective To summarize the revascularization surgery results of patients with unilateral absence of a pulmonary artery.Methods From November 2014 to July 2016,5 patients with unilateral absence of a pulmonary artery underwent surgical revascularization at Guangzhou Women and Children Medical Center.The age and body weight at surgery was 1-6 months and 3.9-5.8 kg.One patient was diagnosed with unilateral absence of left pulmonary artery.The other 4 patients were diagnosed with unilateral absence of right pulmonary artery.Three patients were diagnosed with isolated unilateral absence of a pulmonary artery.One was associated with Tetralogy of Fallot.One was associated with atrial septal defect.Revascularization was performed by connection of hilar artery and the main pulmonary artery,using direct anastomosis,Gore-Tex conduit or pericardial conduit.Results There was no mortality.The ICU stay after operation were 3-14 days.The follow-up duration was 14-34 months.All patients were alive and free from significant pulmonary stenosis.Conclusion Revascularization surgery for patients with unilateral absence of a pulmonary artery is effective and achieved satisfactory results.

Objective To assess the application value of juxtaposition of the great vessels in diagnosis of right atrial isom-erism( RAI) by echocardiography.Methods We retrospectively analyzed 73 patients with right atrial isomerism in Guangzhou Women and Children's Medical Center from 2010-2017.All these patients were performed by echocardiography and compu-ted tomography(CT).We used the results of CT as the golden standard.Results 73 cases of RAI were diagnosed by CT.60 cases of RAI were diagnosed by echocardiography.Juxtaposition of the descending aorta(DAO) and inferior vena(IVC) was demonstrated in all these 60 patients.29 of 60 were on the left side of the spine(39.73％).31 of 60 were on the right side of spine(42.47％).In the other 13 patients of echocardiography missed diagnosis, the arrangement of the great vessels was nor-mal in 10 cases, and inverse in 3 cases.Conclusion Juxtaposition of the great vessels is one of the most important findings in patients with RAI.It is quick and simple and has a higher application value in diagnosis of RAI.

Objective To summarize the outcomes and experience of arterial switch operation (ASO) in the past 10 years in our center.Methods From September 2008 to July 2017,238 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.There were 193 male and 45 female.Median age at operation was 2 months (2 days to 10 years) and mean body weight was(4.2 ± 1.7) kg (1.8-20.6 kg).There were TGA and VSD 91 cases,TGA/IVS 110 cases,and Taussig-Bing anomaly 37 cases.Among them 24 patients had an aortic arch anomaly.Intramural coronary artery was found in 8 patients.Results All patients successfully completed the operation,one-stage ASO was perfonned in 232 patients.Two-stage ASO was performed in 6 patients.The mortality was 14.2％.The follow-up duration was 1-10 years (median time,46 mouths).There were 3 died.Two suffered sudden death,and another one arrhythmia.10-year survival rate was 92.8％.Conclusion The outcomes of ASO were satisfactory.The long-term reoperation rate was rare.