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Background Persistent patent foramen ovale (PFO) and patent ductus arteriosus (PDA) increase the adult risk of cryptogenic embolic stroke and chronic pulmonary hypertension. To understand the characteristics of PFO and PDA in newborns, we investigated the spontaneous closure rate and derived the determinants for residual defects. Methods We utilized the database of congenital heart disease (CHD) in Xiamen ChangGung Memorial Hospital from 2015 to 2017 and allocated 2523 eligible newborns into four groups according to PDA, PFO, both or neither at birth. A total of 574, 1229, 202 and 518 newborns were assigned into the group of PFO and PDA, PFO alone, PDA alone and non-PFO/non-PDA, respectively. Regular echocardiographic follow-ups at baseline, 6, 12 and 24 months after birth were performed for evaluating the spontaneous closure rate in the subjects. Regression analysis was carried out to study the risk factors of residual congenital defects. Results Newborns with PFO alone had the youngest birth age and lowest birth weight among the four groups. About one in four PDA-alone newborns had concomitant small ASD, i.e.

Background: Turner syndrome (TS) is characterized by growth failure, primary ovarian failure, cardiac anomalies, and other anomalies. Cardiovascular abnormalities such as bicuspid aortic valve (BAV), coarctation of the aorta (CoA), aortic stenosis (AS), and aortic dilatation (AD) account for some cases of TS-related early mortality. In this study, we investigated the correlations between cardiovascular phenotypes and karyotypes in TS. Methods: We conducted a retrospective cohort analysis of 105 local patients with TS aged 6–43 years between January 1994 and December 2018. They were categorized into two groups of complete monosomy X (45,X) and other X chromosome abnormalities. Most of the patients underwent echocardiography (n = 88, 83.8%), cardiac computed tomography (CT) angiography, and/or cardiovascular magnetic resonance imaging (MRI) (n = 58, 55.2%). We used independent the Student's t test, chi-square test or Fisher's exact test, and log-rank test to compare differences in continuous data, proportions, and Kaplan–Meier survival analysis results between the two TS groups. Results: 45,X was the most common karyotype (n = 47, 44.8%). Phenotypically, cardiovascular malformations were found in 29 patients with TS (27.6%). BAV (n = 6), CoA (n = 3), AS (n = 2), ASD (n = 1, 2.5%), and PAPVR (n = 1, 2.5%) were found in only the 45,X group. The mean age at AD onset was 25.55 ± 5.78 years (mean ± SD). Survival analysis of age at onset of AD demonstrated no significant difference between the two groups (p = 0.051). Conclusion: Cardiovascular abnormalities, such as BAV, CoA, AS, and AD, are common and potentially progressive in patients with TS, especially those with the 45,X karyotype. They should receive immediate cardiological assessments upon receiving diagnosis, regular assessments, and treatment to carefully control blood pressure, even with no apparent congenital heart disease. Key Words: aortic dilatation, aortic stenosis, bicuspid aortic valve, coarctation of the aorta, turner syndrome

Background Renal artery stenosis is one of the secondary causes of pediatric hypertension. Cases with critical unilateral renal artery stenosis manifesting with the hyponatremic hypertensive syndrome are rare and a comprehensive description of this disorder in the pediatric population is lacking in the literature. Case presentation We describe a 4-year-old boy who presented with severe hypertension, profound hyponatremia, hypokalemia, nephrotic range proteinuria, and polyuria. Distinctly, the diagnosis of hyponatremic hypertensive syndrome secondary to unilateral renal artery stenosis was confirmed in light of laboratory and radiographic findings of severe natriuresis, elevated renin, and unilateral small kidney. Two weeks following nephrectomy, there was resolution of hyponatremia, hypokalemia, nephrotic range proteinuria and hypertension. Conclusions Findings of hyponatremia, hypokalemia, hypertension, polyuria, and unilateral renal hypoplasia can be attributed to a unifying pathology of unilateral renal artery stenosis. Electronic supplementary material The online version of this article (10.1186/s12882-019-1246-9) contains supplementary material, which is available to authorized users.

Aim To validate the initial psychometric properties of a Healthcare Needs Scale for Youth with Congenital Heart Disease. Background As the number of patients with congenital heart disease surviving to adulthood increases, the transitional healthcare needs for adolescents and young adults with congenital heart disease require investigation. However, few tools comprehensively identify the healthcare needs of youth with congenital heart disease. Design A cross-sectional study was employed to examine the psychometric properties of the Healthcare Needs Scale for Youth with Congenital Heart Disease. Methods The sample consisted of 500 patients with congenital heart disease, aged 15–24 years, from pediatric cardiology departments and covered the period from March - August 2015. The patients completed the 25-item Healthcare Needs Scale for Youth with Congenital Heart Disease, the questionnaire on health needs for adolescents and the WHO Quality of Life-BREF. Reliability and construct, concurrent, predictive and known-group validity were examined. Results The Healthcare Needs Scale for Youth with Congenital Heart Disease includes three dimensions, namely health management, health policy and individual and interpersonal relationships, which consist of 25 items. It demonstrated excellent internal consistency and sound construct, concurrent, predictive and known-group validity. Conclusion The Healthcare Needs Scale for Youth with Congenital Heart Disease is a psychometrically robust measure of the healthcare needs of youth with congenital heart disease. It has the potential to provide nurses with a means to assess and identify the concerns of youth with congenital heart disease and to help them achieve a successful transition to adult care. This article is protected by copyright. All rights reserved.

Objective To investigate the influence of surgical correction on biomarkers of endothelial dysfunction in children with congenital heart disease and to evaluate anthropometric data. Methods Children with pulmonary hypertension (PH) or Tetralogy of Fallot (TOF) who were scheduled for corrective surgery were enrolled in this prospective study. Age-matched healthy children were included as controls. Demographic, haemodynamic and cardiac ultrasonography data were collected. Blood samples were taken pre-surgery, 24–48 hours post-surgery and again 3–6 months later. Several biomarkers (protein C, soluble platelet selectin [CD62P], soluble endothelium selectin [CD62E], soluble leukocyte selectin [CD62L], plasma von Willebrand Factor [vWF] atrial natriuretic peptide [ANP], brain natriuretic peptide[(BNP] and insulin-like growth factor-1 [IGF-1]) were measured. Results Sixty-three children (32 with PH, 15 with TOF, and 16 controls) were enrolled. No significant differences between the PH and TOF groups were observed in the expression of biomarkers pre- and post-surgery. IGF-1 levels were closely related to anthropometric data, particularly those children with PH. Expression of IGF-1 and weight/height normalized after corrective surgery. Conclusions No significant endothelial dysfunction was observed in children with PH or TOF before or after corrective surgery. Significant retardation of growth, particularly weight, was found before surgery and may be related to IGF-1 suppression.

Pediatric myocarditis symptoms can be mild or as extreme as sudden cardiac arrest. Early identification of the severity of illness and timely provision of critical care is helpful; however, the risk factors associated with mortality remain unclear and controversial. We undertook a retrospective review of the medical records of pediatric patients with myocarditis in a tertiary care referral hospital for over 12 years to identify the predictive factors of mortality. Demographics, presentation, laboratory test results, echocardiography findings, and treatment outcomes were obtained. Regression analyses revealed the clinical parameters for predicting mortality. During the 12-year period, 94 patients with myocarditis were included. Of these, 16 (17%) patients died, with 12 succumbing in the first 72 hours after admission. Fatal cases more commonly presented with arrhythmia, hypotension, acidosis, gastrointestinal symptoms, decreased left ventricular ejection fraction, and elevated isoenzyme of creatine kinase and troponin I levels than nonfatal cases. In multivariate analysis, troponin I > 45 ng/mL and left ventricular ejection fraction < 42% were significantly associated with mortality. Pediatric myocarditis had a high mortality rate, much of which was concentrated in the first 72 hours after hospitalization. Children with very high troponin levels or reduced ejection fraction in the first 24 hours were at higher risk of mortality, and targeting these individuals for more intensive therapies may be warranted.

Table S1. Clinical and laboratory characteristics before and after treatment. (DOC 32 kb)

The objective of this study was to determine the incidence, risk factors, outcome, and clinical significance of pericardial effusion (PE). We retrospectively analyzed outcomes of 272 pediatric patients undergoing their first hematopoietic stem cell transplantation (HSCT) from 1998 to 2016. In total, 15% (3/20) and 5.9% (15/252) of autologous and allogeneic HSCT recipients, respectively, were identified with PE. However, there was no statistically significant difference in the incidence of PE between the 2 groups. The mean age at transplantation was 11.12 ± 5.41 y. Eighteen patients developed PE at 4.13 ± 4.44 mo after HSCT. PE was confirmed by echocardiogram in all patients. Three patients presented with severe PE with cardiac tamponade and required urgent pericardiocentesis. Overall survival (OS) rates for patients who developed PE were 83.3% and 38.9% at 100 d and 3 y, respectively, after HSCT. Death was not directly attributable to PE in patients who died in the first year after HSCT. Multivariable analysis identified the following variables to be associated with OS: PE (relative risk[RR]: 3.70; 95% confidence interval [95% CI]: 1.89-7.23; P < 0.001), active disease at HSCT (RR: 1.59; 95% CI: 1.02-2.49; P < 0.001), and thalassemia (RR: 0.62; 95% CI: 0.45-0.84; P < 0.001). PE is, thus, a debilitating and significant complication of pediatric HSCT. Therefore, prospective studies are required for better determination of the etiology and optimal method of PE treatment after HSCT.

a Division of Nephrology, Department of Pediatrics, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan b Graduate Institute of Medical Sciences, National Defense Medical Center, Taipei, Taiwan c Division of Nephrology, Department of Medicine, Tri-Service General Hospital, Taipei, Taiwan d Division of Cardiology, Department of Pediatrics, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan e Department of Pediatrics, En Chu Kong Hospital, Taipei, Taiwan f Division of Neonatology, Department of Pediatrics, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan g Division of Allergy, Asthma and Rheumatology, Department of Pediatrics, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan

Objective To analyze the efficacy of a novel palivizumab protocol for hemodynamically significant congenital heart disease (hsCHD) in subtropical areas without clear respiratory syncytial virus seasonality. Study design Since July 2013, the National Health Insurance program has provided reimbursement for palivizumab prophylaxis with a novel monthly protocol in selected patients with hsCHD under 1 year of age. We performed a multicenter study to assess the trend of respiratory syncytial virus hospitalizations in patients with hsCHD from 2010 to 2016 during the prepalivizumab, transition, and postpalivizumab periods, and compared treatment and propensity-matched control groups. Results A total of 747 patients were enrolled in the study group and 809 in the control group. The male:female was 836:720. Cyanotic CHD was observed in 42.9% of patients. The mean age at diagnosis of CHD was 32.9 days. After 516 685 patient-days of follow-up and a mean of 3.9 doses of palivizumab in the treatment group, respiratory syncytial virus hospitalization rates decreased by 53% and 49% before and after match compared with the control group (P = .009 and .029, respectively). Hospitalization days and intensive care unit admission rate also decreased similarly in the treatment group. The efficacy of this protocol was more prominent in patients with cyanotic hsCHD. The annual respiratory syncytial virus-associated hospitalization rates also decreased significantly from the prepalivizumab to the palivizumab period (from 4.8% to 2.0%; P = .038). Conclusion Palivizumab prophylaxis through the novel monthly protocol for patients with hsCHD is effective in reducing respiratory syncytial virus-related hospitalizations.

Objectives: Patients with systemic lupus erythematosus (SLE) have a higher risk of myocardial involvement, which can result in ventricular dysfunction. Little is known about the chronic influence of SLE on heart function in children and adolescents. This is the first study to demonstrate long-term changes in left ventricular function in patients with juvenile-onset SLE. Methods: This was a longitudinal study of 92 patients with juvenile-onset SLE. Two-dimensional echocardiography was performed by a single pediatric cardiologist at baseline, with follow-up at six-month intervals. Clinical and laboratory parameters, disease activity, treatment, nailfold capillaroscopy, and the traditional risk factors for atherosclerosis were evaluated. The baseline comparison of ventricular function was performed against 50 age-matched controls, and the follow-up results were analyzed using generalized estimating equations. Results: The patients’ mean age at baseline was 15.9 ± 4.3 years, the mean disease duration was 3.6 ± 3.2 years, and the mean follow-up duration was 4.5 ± 1.6 years. At baseline, the mean left ventricular ejection fraction (LVEF) was 74.7 ± 5.6% and the mean E/A ratio of left ventricular diastolic filling was 1.7 ± 0.3 (E: the peak velocity at rapid left ventricular filling; A: the peak velocity during left atrial contraction). The LVEF of the SLE patients was similar to the healthy controls and it did not change during the follow-up period. In contrast, the E/A ratio was lower in the SLE patients than in the healthy controls (1.7 ± 0.3 versus 1.88 ± 0.37; p = 0.002), and it decreased significantly with time (B ± SE, −0.013 ± 0.006, p = 0.023). In multiple analyses, abnormal microvasculature in nailfold capillaroscopy had a negative effect on LVEF progression ( p = 0.039). Disease duration of SLE and proteinuria were risk factors associated with the descent of E/A ratio ( p = 0.014 and p = 0.015, respectively). Conclusion: In patients with juvenile-onset SLE who were free of cardiac symptoms, there was evidence of declining ventricular diastolic function with time. Abnormal nailfold microvasculature, proteinuria and longer disease duration were the main risk factors for worsening of ventricular function.

To differentiate severe congenital neutropenia (SCN) from autoimmune neutropenia (AIN) in patients with persistent neutropenia ≤1000/mm(3) over three months, we evaluated anti-neutrophil auto-antibodies, candidate genes of ELANA, HAX1 and GCSFR, and neutrophil elastase (NE) activity in 38 patients (21 females; average onset age 14.12 ± 2.49 months) in a primary immunodeficiency disease center between 2004 and 2011. In 30 patients, detectable anti-neutrophil auto-antibodies were HNA1a in 16 patients, HNA1c in 15, MHC Class I in 14, HNA1b in eight, MHC Class II in five, and HNA2a in three. Their average neutropenia duration was 27.04 ± 2.08 months. Of eight patients without detectable auto-antibodies, three had ELANE mutations [Ser126Pro, Arg170Phe and Cys223stop] and recurrent muco-cutaneous infections and sepsis. The patient with nonsense ELANE mutation [Cys223stop] had the lowest NE activity (16.8). Thus, patients with ELANE mutations have undetectable antibodies and more severe and younger-onset muco-cutaneous infections, prolonged healing and decreased serum NE activity that require prompt intervention.

This study aimed to evaluate increased coronary artery dimensions in patients with paediatric-onset systemic lupus erythematosus (SLE) in comparison with healthy controls, and to identify risk factors associated with increased coronary artery dimensions in the SLE patients.As part of a longitudinal cohort study of coronary artery disease (CAD) in paediatric-onset SLE, 83 children with SLE and 36 healthy controls were enrolled for a cross-sectional analysis. Their coronary artery diameters were measured by echocardiography while their body mass index (BMI), blood pressure, and other cardiovascular factors were recorded. The age at diagnosis, serum uric acid (UA) and creatinine levels, and other lupus-related factors were further evaluated in SLE patients. Data were analysed using linear regression.Mean body surface area (BSA)-adjusted dimensions of the left coronary artery (LCA) and right coronary artery (RCA) were significantly larger in SLE patients than in controls (both p0.001). The age at diagnosis, BMI, and serum UA and creatinine levels were associated with LCA and RCA diameters. There were no correlations between the coronary artery diameters and blood pressure, SLE duration, SLE Disease Activity Index (SLEDAI), C-reactive protein (CRP), C3, C4, anti-double-stranded-DNA (anti-dsDNA), or lipid profile. In multivariate analysis, serum UA level, age at diagnosis, and BMI were consistently associated with coronary artery dimensions (p0.001, p = 0.008, and p = 0.006 for LCA; p = 0.020, 0.013, and 0.008 for RCA).Increased coronary artery diameters were found in children with SLE and were associated with higher serum UA levels. The pathogenic mechanisms warrant further investigation.

Background The purposes of this study were to characterize the symptoms and signs of children with myocarditis at the time of presentation to the hospital and to identify the predictors of death. Methods This was a 5-year retrospective study in a tertiary hospital. We collected demographic data and clinical symptoms and signs when children with myocarditis presented at the hospital. The outcome for patient was classified as either survival or death, and the predictors of death were identified. Results Over the 5-year period, 27 children (14 boys and 13 girls) met the definition of clinical myocarditis. The mean age of the myocarditis patients was 9.1 ± 5.1 years (range, 0.08–17.9 years), and the maximum age was 10–12 years. The most common presentation was gastrointestinal symptoms. We used extracorporeal membrane oxygenation on nine (33%) children, and pacemaker was implanted in eight (30%). Six (22%) children died in this study, and only one of them was younger than 6 years. The poor prognosis predictors were gastrointestinal symptoms, hepatomegaly, and hypotension. Conclusions Pediatric myocarditis presents primarily with gastrointestinal symptoms in Taiwan. Careful check of heart rhythm may provide a useful objective marker of myocarditis. The predictors of a poor prognosis were gastrointestinal symptoms, hepatomegaly, and hypotension.

Background Respiratory distress and patent ductus arteriosus (PDA) in neonates are mutually perpetuating. Contrary to the situation in premature infants, the recognition, clinical relevance and optimal management of PDA in full-term neonates are unclear. The present study aimed to identify PDA as a possible cause of respiratory distress in term and near-term neonates, and to examine the clinical responsiveness of PDA to different treatment modalities in mature-gestational-age neonates. Methods Patients with gestational ages of over 34 weeks were included in this retrospective chart review; they had PDA as the sole recognizable cause of respiratory distress and were free of all other diseases. Clinical responsiveness to different regimens, including conservative treatment, drug therapy with preload reduction and inotropic agent with or without the addition of indomethacin, and surgical intervention were analyzed. Results Forty-four neonates qualified for this study. Six received no treatment and their cardiorespiratory symptoms resolved within 1 week (regimen A). Symptoms in 11 neonates were relieved after use of diuretic and inotropic agents (regimen B). Twelve neonates became asymptomatic without further intervention after indomethacin treatment in addition to preload reduction and inotropes (regimen C). A total of 15 of the 44 infants underwent PDA ligation (regimen D) due to persistent heart failure following regimens B or C, but had speedy resolution of respiratory symptoms following surgery. There were significant differences in birth body weight and hemodynamic variation based on left atrium to aortic root dimensional ratio between the treatment (regimens B, C and D) and non-treatment (regimen A) groups (p Conclusion PDA plays an important role in prolonging respiratory distress in term or near-term neonates. Although most infants respond to noninvasive medical treatment, surgical ligation during the neonatal period is warranted in certain mature infants. Surgical treatment should be considered in patients with smaller birth body weights and those with increased left atrium to aortic root dimensional ratios.

Objective To characterize the atherosclerotic risk factors in the progression of subclinical atherosclerosis in patients with juvenile-onset systemic lupus erythematosus (SLE). Methods This was a longitudinal study of 76 patients with juvenile-onset SLE. Carotid arteries were evaluated using ultrasonography at baseline and at followup visits at 6-month intervals over the 6-year study period. Clinical and laboratory parameters, disease activity, treatment, and traditional risk factors for atherosclerosis were evaluated. Data were analyzed using generalized estimating equations. Results The mean ± SD age of the patients at baseline was 15.01 ± 3.48 years and the mean ± SD disease duration was 2.65 ± 2.5 years. The mean ± SD duration of followup was 3.74 ± 1.24 years. The mean ± SD intima-media thickness (IMT) of the common carotid arteries differed significantly between the patient and control (n = 38) groups (0.63 ± 0.08 mm versus 0.54 ± 0.06 mm; P < 0.001). The presence of lymphopenia at diagnosis and at baseline and higher levels of serum creatinine and C-reactive protein at baseline were positively associated with progression of carotid IMT (P = 0.006, P = 0.043, P = 0.037, and P = 0.049, respectively). In multivariate analysis, only lymphopenia at baseline and at diagnosis were consistently associated with progression of IMT (P = 0.012 and P = 0.045, respectively). Conclusion In patients with juvenile-onset SLE, some nontraditional risk factors for the progression of subclinical atherosclerosis were identified. Lymphopenia was the only independent risk factor for the progression of IMT. The pathogenic mechanisms warrant further investigation.

BackgroundBalloon angioplasty (BA) is an alternative to surgical repair for coarctation of the aorta (CoA) in children. However, its role in the treatment of native CoA in neonates and infants remains controversial. The purpose of this study was to report the midterm outcomes of BA for native CoA in neonates and infants with congestive heart failure (CHF).MethodsBetween July 2000 and March 2007, 18 neonates and infants with native CoA and CHF who underwent BA were enrolled. Patients without recoarctation were designated as group A, while those with recoarctation or CHF were designated as group B. The clinical presentations, laboratory data, and outcomes were compared between groups.ResultsThere were 10 patients in group A and eight in group B. The mean age was 2.8 ± 3.1 months (range, 0.7–11 months). Mean body weight was 4.0 ± 1.9 kg (range, 2.1–8.0 kg). CHF improved markedly in all patients immediately after BA, with a reduction in systolic pressure gradient from 36.4 ± 12.0 to 5.6 ± 6.0 mmHg (p < 0.001). The recoarctation rate was 44% (8/18). The risk factors for restenosis were post-BA systolic pressure gradient > 10 mmHg (p = 0.007) and CoA diameter < 3 mm (p = 0.013).ConclusionsThe outcomes of BA for native CoA in neonates and infants with CHF remain poor. The incidence of recoarctation is high in neonates and patients whose post-BA systolic pressure gradient is > 10 mmHg or whose CoA diameter is < 3 mm.

Background: A polymorphism in the monocyte chemoattractant protein 1 (MCP-1) gene regulatory region has been associated with asthma in Caucasians. This polymorphism is possibly endemic to the Asian region, but its impact on Asian populations is unclear. In addition, the relationship of this marker with life-threatening asthma has not been clarified. The aim of this study was to test the genetic association between the MCP-1 –2518A/G polymorphism and asthma/atopy in a cohort of Chinese children, with particular emphasis on those patients who had experienced life-threatening asthma attacks. Methods: Forty-eight children with near-fatal asthma, 134 mild-to-moderate asthmatics, 69 allergic-disorder cases without asthma, and 107 nonasthmatic, nonatopic control children were genotyped by a polymerase chain reaction-based assay. Results: Comparison of the four groups of children (n = 358) revealed no detectable differences in genotype or allele frequencies of the MCP-1 –2518A/G polymorphism. There was no evidence of association between the polymorphism and any of the outcomes of interest including clinical severity, blood eosinophil count, atopy, total serum IgE levels, and degree of bronchial hyper-responsiveness. Conclusion: These results suggest that the MCP-1 –2518A/G polymorphism is not a risk factor for near-fatal asthma. Furthermore, this polymorphism seems to play no role in the development of asthma or atopy in Chinese subjects, possibly as a result of the genetic heterogeneity between Asian and Caucasian populations with respect to regulation of MCP-1 expression. Our results underscore the necessity of accounting for ethnic background in the investigation of asthma-predisposition genes.

A 1-year-4-month-old girl who presented with pericardial effusion and superior vena cava (SVC) syndrome caused by a mediastinal mass was later proved to be a case of acute myeloid leukemia (AML) with mixed-lineage leukemia-gene translocation. The unusual presentation and the giant blasts with basophilic vacuolated cytoplasm had led to initial misdiagnosis of mediastinal lymphoma. She developed progressive SVC syndrome, unresolved pericardial effusion, and extensive leukemia cutis after initial induction therapy. She died soon after second-course chemotherapy. To our knowledge, this is the first reported case of childhood acute myeloid leukemia presenting with mediastinal granulocytic sarcoma causing pericardium invasion and SVC syndrome.

Mitochondrial diseases are a group of disorders caused by pathologic dysfunction of the mitochondrial respiratory chain that present with a wide range of clinical expression. Cardiorespiratory complications have previously been described in association with mitochondrial disease; however, pulmonary hypertension has rarely been reported. Pulmonary hypertension is characterized by elevated pulmonary arterial pressure and secondary right ventricular failure. It is a life-threatening condition with a poor prognosis if untreated. We report a case of 3-year-4-month-old boy who had mitochondrial A3243G point mutation with pulmonary hypertension. The unusual features of our case strengthen the concepts of pulmonary hypertension should be considered as another potential manifestation of mitochondrial disease.

The purpose of this report was to develop novel biodegradable occlusion devices for closure of atrial septal defects (ASD). To manufacture the biodegradable occluders, polycaprolactone (PCL) components were first fabricated by a lab-scale micro-injection molding machine. They were then assembled and hot-spot welded into double umbrella-like devices of 50 mm in diameter. A special mechanism at the axis of the occluder was designed to self-lock the occluder after the two umbrellas were expanded. Furthermore, a nanofibrous matrix of poly-d-l-lactide-glycolide (PLGA)/type I collagen blend was produced via electrospinning to develop biodegradable and biomimetic anti-shunt membranes for the occluders. Characterization of the biodegradable PCL occluders was carried out. PCL occluders exhibited mechanical properties comparable to that of commercially available Amplatzer occluders. The sealing capability of biodegradable occluders was found superior to that of Amplatzer occluders. In addition, the cell attachment and spreading of endothelial cells seeded on the PLGA/collagen nanofibrous matrix and the interaction between cells and PLGA/collagen nanofibers were studied. The nanofibrous membranes made of PLGA/collagen were very effective in promoting cell proliferation during culture.

Our study was designed to prospectively determine whether or not unrelated cord blood transplantation (CBT) can produce outcomes comparable to related donor transplantation for children with β-thalassaemia. In 35 patients, 40 transplants were performed between October 2003 and September 2009. HLA matching at enrolment was 6/6 (n=8), 5/6 (n=16), 4/6 (n=27), or 3/6 (n=1) by low-resolution HLA-A, -B, and high-resolution DRB1. These patients received non-manipulated grafts without ex vivo expansion or T-cell depletion. The median number of nucleated and CD34+ cells infused was 7.8 × 10(7)/kg (range, 2.8-14.7 × 10(7)/kg) and 4.0 × 10(5)/kg (range, 1.7-19.9 × 10(5)/kg), respectively. The 5-year OS and thalassaemia-free survival after the first transplant were 88.3 and 73.9%, respectively. The cumulative incidence of TRM at 2 years was 11.7%. Fourteen patients developed chronic skin GVHD. Thirty patients were alive and transfusion-independent with a Lansky performance score ≥80% achieved between 6 and 76 months post transplant (median, 36 months). These data compare acceptably with the survival rates of related-donor BMT for thalassaemia and suggest that patients without an available HLA-compatible sibling but who have well-matched unrelated donors should also be considered for CBT.

Of all patients with systemic lupus erythematosus (SLE), 15—20% are diagnosed during childhood, with disease onset prior to the age of 16 years. Because disease expression in SLE is influenced by environment factors and differs between racial and ethnic groups. The aims of this review were to describe prevalence, clinical manifestations, common infectious complications, and outcome of pediatric-onset SLE in Asia. The prevalence of pediatric-onset SLE was 6.3—19.3 per 100,000 in Asia. The ratio of female to male was 4.7—6.2. The mean age at diagnosis of pediatric-onset SLE was 8.6—13.5 years. The most common clinical features of pediatric-onset SLE in Asia were cutaneous rashes, arthritis, hematological involvement and nephritis. The occurrence of nephritis varies from 29% to 81%. The most common histopathology of lupus nephritis was diffuse proliferative glomerulonephritis (WHO Class-IV) which occurred in 39.4—54% of case of lupus nephritis. Pediatric-onset SLE patients with infections have poor outcomes than uninfected patients. Gram-negative bacilli are the most common microorganisms responsible for bacteremia in Asian patients with SLE. Recurrent major infections predict poorer disease outcome and associated organ damage in pediatric-onset SLE. Improving the survival of SLE patients was reported in Asia in recent decades. The survival was 92% at the age of 5 years, 86% at 10 years and 79% at 15 years in children with SLE in Taiwan in 2008.

Objectives To report a young patient with systemic lupus erythematosus (SLE) complicated by aortic dissection. The relevant literature on the association of SLE and aortic dissection is reviewed. Methods We describe an adolescent patient with childhood-onset SLE diagnosed aortic dissection with presentation of hemothorax. The literature review was performed by a PubMed search using the keywords systemic lupus erythematosus (SLE), aortic dissection, hemothorax, and carotid intima-media thickness (CIMT). Results A 17-year-old male was admitted to the hospital for severe abdominal pain. His past medical history included childhood-onset SLE complicated with lupus nephritis. Acute aortic dissection complicated with hemothorax was diagnosed and he died despite medical therapy. The accelerated CIMT progression of our patient, 0.14 mm in 20 months, might suggest ongoing premature atherosclerotic changes in the aortic wall. On reviewing the English literature, 21 cases of aortic dissection in SLE have been analyzed and discussed. Patients younger than 40 years of age, hypertension, and long-term steroid use are common features of aortic dissection in SLE patients. The possible pathogenesis of aortic dissection in SLE includes atherosclerosis, degeneration, and vasculitis. The widely accepted CIMT measured by ultrasound could be a potential diagnostic tool to access the risk of cardiovascular events. Conclusions Aortic dissection is a rare complication of SLE, but could take place in an adolescent patient with childhood-onset disease. It is important to include aortic dissection as a differential diagnosis in SLE patients with unexplained sharp abdominal, chest, or back pain.

Duchenne muscular dystrophy (DMD) is the most common hereditary neuromuscular disease in children. It is an X-linked hereditary dystrophinopathy due to the absence of dystrophin. Its onset is often in early childhood and presents with proximal distribution of weakness and a progressive course. Cardiac involvement in DMD is common, and its onset is usually after the age of 10 years. The most common cardiac manifestations are a dilated cardiomyopathy and cardiac arrhythmia. However, pericardial effusion with cardiac tamponade is a very rare cardiac complication of DMD. We report a boy with DMD who initially presented with progressive dyspnea and an enlarged cardiac silhouette on chest radiography who subsequently developed a large pericardial effusion with cardiac tamponade. Early recognition of pericardial effusion with cardiac tamponade is important for institution appropriate therapy.

The spontaneous rupture of a Silastic catheter is a rare occurrence. We describe our experience of managing a tiny premature infant with embolization of a Silastic percutaneous central venous catheter (PCVC) and discuss the possible mechanisms of the embolization. A 28-week, 980 g, preterm male infant received a Silastic PCVC (Epicutaneo Cava Catheter, Vygon, Germany) for parenteral nutritional support at 4 days of age. The catheter was introduced percutaneously and advanced without difficulty through the right antecubital vein, and was subsequently withdrawn 2 cm following confirmation of tip position using radiography. A following chest radiograph, taken 15 hours later, showed rupture of the catheter, and an echocardiogram revealed a piece of the catheter had lodged between the right atrium and the right ventricle. The dislodged fragment of the catheter was retrieved successfully using a snare catheter (Microvena, White Bear Lake, Minn) by a pediatric cardiologist without complications. We want to stress that clinicians should be aware that rupture of the catheter is rare and can also occur asymptomatically and that an embolized fragment can be safely removed without extensive surgical manipulation, even in a tiny premature infant.

Protein-losing enteropathy (PLE) is a serious complication of a Fontan operation and has a very high mortality rate. The purpose of this study was to investigate the incidence, clinical manifestations, diagnostic approaches, laboratory findings, therapeutic modalities and outcome of patients with PLE at our institution.The diagnosis of PLE was based on clinical manifestations and laboratory studies. We reviewed medical records of patients who received a Fontan operation at our hospital form July 1985 to October 2005.A total 101 patients underwent various modifications of the Fontan procedure during this period. Nine of the 75 patients (12%) who survived 30 days after surgery developed PLE, including 4 boys and 5 girls. The median time interval between the Fontan operation and onset of PLE was 3.7 years (range 1.2 to 9.7 years). Laboratory examination showed low serum albumin levels and increased fecal alpha-1-antitrypsin excretion. Lymphangiectasia was found on intestinal biopsy. Six patients had cardiac catheterization after development of PLE which demonstrated an elevated mean right atrium pressure (22.5 +/- 6.4 mmHg, range 16 to 33 mmHg) and mean pulmonary artery pressure (22.3 +/- 6.4 mmHg, range 16 to 33 mmHg). Treatment included diet modification, albumin infusion, diuretics, inotropes, corticosteroids, heparin, and surgery. Four patients received medical treatment only. Two of these patients died due to sepsis and heart failure and 2 survived with partial relief of PLE. The remaining five received surgery for PLE after medical treatment failure. Three of them died after the operation and the two survivors were free of PLE, but one died of ventricular tachycardia 8 years later. The overall mortality rate was 67% (6/9).The current treatment for PLE is associated with a very high mortality rate. Further investigation is needed to determine the exact mechanism of the disease and to develop new therapeutic approaches.

Transcatheter closure of ostium secundum atrial septum defect (ASDII) using the new self-centering occluder, Amplatzer Septal Occluder (ASO), has been well developed in recent years. We describe the importance and role of transesophageal echocardiography (TEE) in the selection and closure of such defects.Thirty patients referred for transcatheter closure of ASDII by ASO were enrolled in this study. During catheterization, two-dimensional TEE was performed on all patients during and after transcatheter closure. ASD size and morphology were assessed by TEE before catheterization. The ASD stretched diameter was also measured by TEE and fluoroscopy.With the aid of TEE, transcatheter closure of an ASD was successfully, safely and effectively performed on 29 patients. The mean ASD diameter determined by TEE was 17.4 +/- 4.8 mm. The mean stretched diameters measured by TEE and fluoroscopy were 18.7 +/- 5.6 mm and 17.9 +/- 5.5 mm, respectively. The mean device diameter was 19 +/- 5.6 mm. Immediate complete closure was documented by color Doppler TEE in 29 patients. Complications were encountered in one patient, with the device becoming dislodged into the main pulmonary artery. The device was retrieved by surgery and the defect was repaired in the operating room.Transcatheter closure of ASDs using an Amplatzer device is feasible, safe and effective. Two-dimensional TEE can provide useful information before, during and after transcatheter closure of ASDs.

Enterovirus 71 (EV71) can sometimes cause fatal or disabling diseases in children; therefore EV71-infected children with cardiopulmonary failure were investigated at Chang Gung Children's Hospital to discover the prognostic predictors.We investigated 27 EV71-infected children with cardiopulmonary failure from May 2000 to September 2001 and analyzed their clinical data to find predictors associated with unfavorable outcomes of deaths or ventilator dependence.Of the 27 patients, 8 (30%) died and 10 (37%) were ventilator-dependent. Troponin I levels correlated most strongly with fatality, with 5 of the 6 children with troponin I levels40 ng/ml dying (P = 0.001). Other factors correlated with fatality were cerebrospinal fluid white blood cell countor =100/microL (P = 0.002) and initial systolic pressureor =100 mm Hg (P = 0.05). Of the 19 survivors, 10 (53%) were left with central hypoventilation, dysphagia and/or limb weakness plus atrophy. The factors associated with ventilator dependence included higher inotrope equivalent (P0.001), duration of hypotensionor =40 hours, initial blood systolic pressureor =100 mm Hg, positive EV71 isolation and ageor =12 months.Poor prognostic factors were related to cardiovascular and neurologic damage; therefore physicians may consider advanced cardiovascular support for EV71-infected children with cardiopulmonary failure.

We describe a patient with systemic lupus erythematosus who developed Staphylococcus aureus endocarditis following dental work. Multiple brain and cutaneous septic emboli developed despite aggressive antibiotic therapy.

Chemokines play an important role in the physiology and pathophysiology of acute and chronic inflammatory processes. We investigated whether chemokines such as RANTES (regulated upon activation, normally T cell expressed and secreted) promoter and monocyte chemoattractant protein-1 (MCP-1) regulatory polymorphisms were associated with systemic lupus erythematosus (SLE) in Chinese children.Forty-six patients with SLE and 107 healthy children of comparable ages were studied for genotypes with polymerase chain reaction-based assays.The frequency and distribution of genotypes of the -28(C/G) RANTES gene polymorphism were significantly different between the 2 groups (p0.001), and the RANTES -28G allele was significantly more frequent in patients with SLE than in healthy controls (23.9% vs 11%; p = 0.006, OR 2.37, 95% CI 1.25-4.28). There was no significant difference in the frequency or in the distribution of genotypes of the -2518(A/G) MCP-1 and the -403(G/A) RANTES gene polymorphisms between patients and controls (p = 0.32 and p = 0.19, respectively). The RANTES -28G allele was also significantly associated with higher initial levels of antinuclear antibody, lower levels of C3, and higher incidences of central nervous system lupus.In the Chinese population, children with RANTES -28C/G polymorphisms have increased risk of developing SLE. Healthy controls with the C/G or G/G genotype were 2.37 times more likely to have SLE compared to those with the C/C genotype.