Background: Prospective registry studies of congenital heart disease (CHD)-associated pulmonary artery hypertension (PAH) are rare. We established a multicenter registry of CHD-PAH: the TACHYON (TAiwan Congenital Heart disease associated with pulmonarY arterial hypertension) registry.
Methods: The prospective TACHYON registry was initiated in January 2016.
Medicine (Baltimore)
August 2019
Medical decisions should be well-planned to improve prognosis and reduce complications of mediastinal tumors. In this study, we analyzed the clinical presentations of pediatric mediastinal tumors and their correlation with long-term clinical outcome.Forty patients under 18 years of age diagnosed with mediastinal tumors at China Medical University Children's Hospital between 2001 and 2016 were enrolled.
View Article and Find Full Text PDFKawasaki disease (KD) is the most common cause of acquired cardiac disease in children in developed countries. However, little is known regarding the role of transcriptomic targets of KD in the disease progression and development of complications, especially coronary artery aneurysms (CAA). The aim of our study was to identify transcripts affected by KD and their potential role in the disease.
View Article and Find Full Text PDFIdentifying the causes of high fever syndromes such as Kawasaki disease (KD) remains challenging. To investigate pathogen exposure signatures in suspected pathogen-mediated diseases such as KD, we performed immunoglobulin (Ig) profiling using a next-generation sequencing method. After intravenous Ig (IVIG) treatment, we observed disappearance of clonally expanded IgM clonotypes, which were dominantly observed in acute-phase patients.
View Article and Find Full Text PDFCardiac rhabdomyoma (CR) is the most common cardiac tumor in newborns. Approximately 75% of cases are associated with tuberous sclerosis complex. Although these tumors usually spontaneously regress after 2 years of age, they can be life-threatening when they obstruct major cardiac inflow or outflow pathways.
View Article and Find Full Text PDFBackground: Chylothorax in children is a relatively rare cause of pleural effusion. However, it is usually a common complication of cardiothoracic operations like open-heart surgery. Other etiologies for chylothorax, such as trauma or malignancy, occur more common in adults and rare in children.
View Article and Find Full Text PDFKawasaki disease (KD) is an acute, inflammatory, and self-limited vasculitis affecting infants and young children. Coronary artery aneurysm (CAA) formation is the major complication of KD and the leading cause of acquired cardiovascular disease among children. To identify susceptible loci that might predispose patients with KD to CAA formation, a genome-wide association screen was performed in a Taiwanese KD cohort.
View Article and Find Full Text PDFRationale: Kawasaki disease (KD), an acute febrile vasculitis, is the most common cause of acquired heart disease in childhood; however, diagnosing KD can be difficult.
Objective: To identify unique proteomic biomarkers that can be used to facilitate earlier diagnosis of KD.
Methods And Results: We enrolled 214 children with fever and clinical features suggestive of KD.
Background: Kawasaki disease (KD) is an acute and systemic vasculitis. Its complications in coronary artery aneurysms (CAA) make KD one of the leading causes of acquired cardiovascular diseases in childhood. Low density lipoprotein receptor-related protein 1B (LRP1B) is abundantly expressed in the medial layer of coronary arteries and involved in endothelium inflammations.
View Article and Find Full Text PDFBackground: Patients with Kawasaki disease (KD), a pediatric systemic vasculitis, may develop coronary artery aneurysm (CAA) as a complication. To investigate the role of glutamate receptors in KD and its CAA development, we performed genetic association studies.
Methods And Results: We examined the whole family of glutamate receptors by genetic association studies in a Taiwanese cohort of 262 KD patients.
Kawasaki disease (KD) is pediatric systemic vasculitis with the classic complication of coronary artery aneurysm (CAA). It is the leading cause of acquired cardiovascular diseases in children. Some severe cases present with multi-organ involvement or neurological dysfunction.
View Article and Find Full Text PDFBackground: The sorting nexin (SNX) family is involved in endocytosis and protein trafficking and plays multiple roles in various diseases. The role of SNX proteins in Kawasaki disease (KD) is not known. We attempted to test whether genetic SNX variation associates with the risk of coronary artery aneurysm (CAA) formation in KD.
View Article and Find Full Text PDFThe BLK and CD40 loci have been associated with Kawasaki disease (KD) in two genome-wide association studies (GWAS) conducted in a Taiwanese population of Han Chinese ancestry (Taiwanese) and in Japanese cohorts. Here we build on these findings with replication studies of the BLK and CD40 loci in populations of Korean and European descent. The BLK region was significantly associated with KD susceptibility in both populations.
View Article and Find Full Text PDFTo find new candidate loci predisposing individuals to Kawasaki disease, an acute vasculitis that affects children, we conducted a genome-wide association study in 622 individuals with Kawasaki disease (cases) and 1,107 controls in a Han Chinese population residing in Taiwan, with replication in an independent Han Chinese sample of 261 cases and 550 controls. We report two new loci, one at BLK (encoding B-lymphoid tyrosine kinase) and one at CD40, that are associated with Kawasaki disease at genome-wide significance (P < 5 × 10(-8)). Our findings may lead to a better understanding of the role of immune activation and inflammation in Kawasaki disease pathogenesis.
View Article and Find Full Text PDFBackground: The use of thoracostomy tube for drainage of parapneumonic effusion is an important therapeutic measure. In this study, we compared the effectiveness and complications between chest tube and pigtail catheter thoracostomy for drainage of parapneumonic pleural effusion in children.
Methods: We retrospectively reviewed the medical records of children with parapneumonic effusion during the period of July 2001 through December 2003.
Kawasaki disease (KD) is an acute systemic vasculitis syndrome that primarily affects infants and young children. Its etiology is unknown; however, epidemiological findings suggest that genetic predisposition underlies disease susceptibility. Taiwan has the third-highest incidence of KD in the world, after Japan and Korea.
View Article and Find Full Text PDFBackground: In spite of advances in many noninvasive instruments for studying cardiac anomalies in children, cardiac catheterization (CC) is still an essential method for the precise calculation of cardiovascular hemodynamic status and for performing therapeutic interventions. Accordingly, all adverse events (AE) related to CC are a major concern to pediatric cardiologists.
Patients And Methods: A total of 220 children with congenital heart disease (CHD) who received cardiac catheterization in our tertiary care hospital between the period of January 2000 and December 2002 were studied.
Objective: To evaluate growth and neurodevelopmental outcomes in preterm very low birth weight (PVLBW) infants treated with oral probiotics for the prevention of necrotizing enterocolitis (NEC).
Study Design: A prospective follow-up study was performed in a cohort of PVLBW infants enrolled in a single center with a masked randomized control trial to evaluate the efficacy of oral probiotics in preventing NEC. Growth measures included weight, length, and head circumference.
The butyrophilin-like 2 (BTNL2) gene is a member of the B7 receptor family that probably functions as a T cell costimulatory molecule. Because altered T cell functions are implicated in dysregulation of the immune response seen in Kawasaki disease (KD), it is reasonable to speculate that BTNL2 gene is involved in the pathophysiology of KD. The purpose of this study was to investigate whether polymorphisms of the BTNL2 gene are associated with KD and the development of coronary artery lesions (CALs) in Taiwanese children.
View Article and Find Full Text PDFOver the past few decades, Taiwan has seen striking improvements in the life expectancy of its 400 registered beta-thalassemia major (beta-TM) patients due mainly to adequate transfusion regimens and effective iron chelation therapy. Since 1995, Taiwanese citizens have enjoyed universal health care through National Health Insurance (NIH), receiving comprehensive treatment at minimal cost. In 1984, a national program for thalassemia prevention, control, and hematopoietic stem cell transplantation (HSCT) was initiated.
View Article and Find Full Text PDFElevated serum levels of interleukin-10 (IL-10) have been reported in patients with Kawasaki disease (KD). IL-10 reduces the inflammatory actions of macrophages and T cells and it may play a significant role in the regulation of inflammatory vascular damage associated with systemic vasculitis. The aim of this study was to examine whether -592 IL-10 promoter polymorphism is a susceptibility or severity marker of KD in Chinese patients in Taiwan.
View Article and Find Full Text PDFInterleukin-18 (IL-18)-656T/G, -607A/C, and -137C/G promoter polymorphisms had been reported associated with Kawasaki disease (KD). An IL-18 genetic A/C polymorphism at coding position 105 (rs549908) has been linked with asthma, rheumatoid, and systemic lupus erythematosus. We tested a hypothesis that the IL-18 105A/C genetic polymorphism confers KD susceptibility.
View Article and Find Full Text PDFBackground: Chest pain is a common complaint in children visiting the emergency department (ED). True organic problems like cardiac disease are rare. We assess and analyze the etiology of chest pain among children visiting a pediatric ED in one medical center.
View Article and Find Full Text PDFBackground: Ongoing low-grade inflammation and endothelial dysfunction persist in patients late after Kawasaki disease (KD). Statins not only reduce cholesterol, but also improve surrogate markers of atherosclerosis and endothelial dysfunction, but their effects for children late after KD complicated with coronary arterial abnormality (CAA) has not been evaluated.
Methods And Results: The 11 KD children complicated with CAA (mean age 12.