Biomarkers of intravenous immunoglobulin resistance and coronary artery lesions in Kawasaki disease.

Background: Currently, there are no reliable indicators for predicting intravenous immunoglobulin resistance and coronary artery lesions in the early stage of Kawasaki disease.

Methods: A total of 300 patients with Kawasaki disease were studied retrospectively. Laboratory data were compared between the intravenous immunoglobulin resistant (29 patients) and responsive groups, and between the groups with coronary artery lesions (48 patients) and without coronary artery lesions.

Efficacy and safety of lenalidomide for the treatment of acute myeloid leukemia: a systematic review and meta-analysis.

Background: Lenalidomide is effective for the treatment of low-risk myelodysplastic syndromes with deletion 5q abnormalities. However, whether lenalidomide leads to a significant improvement in treatment response and overall survival (OS) in cases of acute myeloid leukemia (AML) remains controversial. A systematic review and a meta-analysis were performed to evaluate the efficacy and safety of lenalidomide in the treatment of AML.

Correlation between rs198388 and rs198389 polymorphismsin brainnatriuretic peptide (NPPB) gene and susceptibility to congenital heart diseases in a Chinese population.

Objective: We discussed the correlation between SNP loci (rs198389 and rs198388) in brain natriuretic peptide gene (NPPB) and susceptibility to congenital heart diseases (CHD).

Method: Multiplex SNaPshot technique was adopted for profiling of SNP genotypes at loci rs198389 and rs198388 in NPPB gene among 150 cases of CHDand 150 normal controls.

Results: The distribution frequency of 3 genotypes (AA, AG and GG) at locus rs198389 was 40.

Hypercoagulation and elevation of blood triglycerides are characteristics of Kawasaki disease.

Background: Cardiovascular damages poses risks to children with Kawasaki disease (KD). Although hypertriglyceridemia and hypercholesteremia are risk factors of cardiovascular damages, studies on the blood lipid metabolism in KD are still limited. This study aims to analyze the blood lipids and coagulation in KD.

[Relationship between 22q11 microdeletion syndrome and congenital heart disease].

Objective: To investigate the relationship between 22q11 microdeletion syndrome and congenital heart disease.

Methods: Clinical screening assessment and genetic testing using standard fluorescence in-situ hybridization (FISH) were applied in 207 subjects suspected for 22q11 microdeletion syndrome. Patients with 22q11 microdeletion syndrome were examined by echocardiography, patients with complicated congenital heart disease were examined further by cardiac catheterization.

Charting the developmental trajectories of attention and executive function in Chinese school-aged children.

Attention is a complex domain that has reawakened research interest in recent years. There are relatively few studies that have examined age-related changes across different attention subcomponents, such as selection, maintenance, and control, using large samples covering a wide age range. The present study assessed performance in 466 participants in order to identify the ages at which mature performance was reached across differing attention subcomponents.

Interventional occlusion of congenital vascular malformations.

Background: New materials and devices have been used in the management of cardiac malformations. In this paper, we present our experience with interventional occlusion of congenital vascular malformations.

Methods: Between January 1997 and December 2005, 139 patients with congenital vascular malformations who had undergone interventional occlusion in the Children's Hospital, Zhejiang University School of Medicine were studied.

[Visual search attention in children with Williams syndrome].

Objective: To explore the characteristics of visual search attention in children with Williams syndrome (WS).

Methods: Twenty-two children with WS, 13 male and 9 female, aged 12 (6-16), 25 chronological age (CA) and mental age (MA)-matched children with Down's syndrome (DS), 45 biological age-matched children, and 43 mental age-matched children, aged 4 +/- 2 (2-8) underwent Peabody picture vocabulary test (PPVT). Visual search attention including selective, switch, and sustained attention, was tested using a set of computerized visual search tasks via a touch-screen.

Patent ductus arteriosus and pulmonary valve stenosis in a patient with 18p deletion syndrome.

We report on a patient with a partial deletion on the short arm of chromosome 18 (del 18p), who presented with dysmorphic features and delayed developmental milestones as well as with a patent ductus arteriosus (PDA) and pulmonary valve stenosis (PS). Several forms of congenital heart disease (CHD) are found in about 10% of patients with del (18p), but coexisting PDA and PS have not been reported. Del (18p) must be considered in patients with characteristic phenotypic abnormalities and congenital heart disease, including a combination of PDA and PS.

[Cardiovascular manifestations in 40 patients with Williams syndrome].

Objective: To evaluate the cardiovascular manifestations of Williams syndrome (WS) confirmed by fluorescence in situ hybridization (FISH).

Methods: Between July 2004 and January 2007, FISH was used to confirm diagnosis in 71 suspected WS cases by detecting chromosome 7q microdeletion. Cardiovascular abnormalities were assessed by echocardiography and Doppler echocardiography.

[A study on the social adjustment and its affective factors in Down syndrome children].

Objective: To explore the social adjustment status and affected factors thereof in Down syndrome children.

Methods: The family environment, cognitive development and social adjustment were examined in 36 Down syndrome children aged 52 - 167 months, 30 mental age-matched children aged 20 - 65 months, and 40 chronological age-matched children aged 43 - 144 months with questionnaire of family influential factors, Peabody Picture Vocabulary Test (PPVT) and Infants-Junior Middle School Students' Social-Life Abilities Scale from September 2004 to July 2006. The gender and general family environment were matched among the three groups.

Study on the social adaptation of Chinese children with down syndrome.

Purpose: To evaluate social adjustment and related factors among Chinese children with Down syndrome (DS).

Patients And Methods: A structured interview and Peabody Picture Vocabulary Test (PPVT) were conducted with a group of 36 DS children with a mean age of 106.28 months, a group of 30 normally-developing children matched for mental age (MA) and a group of 40 normally-developing children matched for chronological age (CA).

[Percutaneous balloon aortic valvuloplasty for congenital valvular aortic stenosis in children].

Objective: To assess the effect of the balloon valvuloplasty for congenital valvular aortic stenosis (AS) in children.

Methods: A total of twenty one children with AS accepted the treatment of percutaneous balloon aortic valvuloplasty (PBAV). One of them complicated with PDA underwent PDA closure by using Amplatzer occluder in the mean time.

[Study on clinical features and fluorescence in situ hybridization detections of 22q11 microdeletion syndrome].

Objective: To investigate clinical features and the diagnosis by fluorescence in situ hybridization (FISH) of 22q11 microdeletion syndrome (22q11 DS).

Methods: The clinical data of suspects were analyzed, and their peripheral blood samples were tested by FISH for microdeletion of 22q11. The diagnosis and correlated clinical factors of 22q11 DS were investigated by using the multiple factor Logistic regression analysis and Chi-square test.

[Transcatheter closure of perimembranous ventricular septal defects in children following transthoracic echocardiography].

Objective: To evaluate the efficacy and safety of transcatheter closure of perimembranous ventricular septal defects (VSD) in children following transthoracic echocardiography (TTE).

Methods: From September 2002 to December 2005, eighty-nine children (47 males and 42 females) with perimembranous (VSD) underwent an attempt of transcatheter interventional occlusion. Among the 89 children, one of them was diagnosed with patent ductus arterious (PDA) and six with VSD leakage after the surgical repair (three with leakage after the surgical repair of tetralogy of Fallot and three with leakage after the surgical repair of VSD).

[Transcatheter therapy of combined congenital heart diseases in children].

Objective: To investigate the methods of interventional catheterization for combined congenital heart disease and to evaluate its efficacy in children.

Methods: From March 1994 to December 2003, 15 cases (6 boys, 9 girls) underwent transcatheter intervention for combined congenital heart diseases. The procedure of transcatheter intervention was as follows: for pulmonary stenosis (PS) and atrial septal defect (ASD) or patent ductus arteriosus (PDA), PBPV first, occlusion of ASD or PDA later; for coarctation of aorta (COA) and PDA, dilation of COA first, occlusion of PDA 4-15 months later; for aortic stenosis (AS) and PDA, PBAV first, occlusion of PDA later; for ventricular septal defect (VSD) and PDA, all occlusions with detachable coils.