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Background. Fibroblast growth factor 21 (FGF-21) is a hormone that regulates glucose and lipid metabolism. High serum FGF-21 levels are associated with carotid atherosclerosis and coronary artery disease. This cross-sectional study aimed to assess the relationship between serum FGF-21 levels and carotid-femoral pulse wave velocity (cfPWV) in patients on maintenance hemodialysis (HD). Methods. Blood samples and baseline characteristics were collected from 130 HD patients. Serum FGF-21 concentrations were measured with an enzyme-linked immunosorbent assay kit. Aortic stiffness was defined as a carotid-femoral pulse wave velocity (cfPWV) of more than 10 m/s. Results. Of the 130 HD patients, aortic stiffness was diagnosed in 54 (41.5%). Serum FGF-21 levels were significantly higher in those with aortic stiffness than those without P

A Ni-WC coating was fabricated successively on a copper substrate by laser. The microstructure, hardness and wear resistance were investigated. The thickness of Ni-WC coating was 0.65 mm and average hardness of strengthening layer was 667HV0.1, which was approximately 7.8 times higher than the hardness of copper substrate. The wear volume of Ni-WC coating was only 0.014 mm3, which was only 1.02% wear volume of copper substrate. The decrease in wear volume was mainly due to presence of reinforced phases, such as γ-Ni, Cr0.19Fe0.7Ni0.11, M7C3, M23C6 and WC. In addition, there was no coating peeled off after 100 times trials of heat shock resistance experiment at 800℃. The experimental results show that this Ni-WC coating has a good metallurgical bonding strength and hardness, and it also has good wear resistance and high temperature property, which is helpful to improve the service life of mould copper plate.

Primary myxoid stromal tumor in infancy is a rare subtype of soft tissue sarcoma, with the feature of infiltration of surrounding tissue, rare metastasis and poor response to chemotherapy. As a result, surgery operation is the most effective among therapies. According to current documents, sarcoma locate in trunk, head, neck and limbs. Here is a case report of PMMTI locating in pharynx and larynx.

The aim of this study was to investigate the association between frailty and polypharmacy using three different frailty screening tools. This was a cross-sectional study of people aged ≥65 years. Participants were included and interviewed using questionnaires. Polypharmacy was defined as the daily use of eight or more pills. Frailty was assessed using a screening tool, including (1) the Fatigue, Resistance, Ambulation, Illness and Loss of Weight Index (5-item FRAIL scale), (2) the Cardiovascular Health Phenotypic Classification of Frailty (CHS_PCF) index (Fried’s Frailty Phenotype), and (3) the Study of Osteoporotic Fracture (SOF) scale. A total of 205 participants (mean age: 71.1 years; 53.7% female) fulfilled our inclusion criteria. The proportion of patients with polypharmacy was 14.1%. After adjustments were made for comorbidity or potential confounders, polypharmacy was associated with frailty on the 5-item FRAIL scale (adjusted odds ratio [aOR]: 9.12; 95% confidence interval [CI]: 3.6–23.16), CHS_PCF index (aOR: 8.98; 95% CI: 2.51–32.11), and SOF scale (aOR: 6.10; 95% CI: 1.47–25.3). Polypharmacy was associated with frailty using three frailty screening tools. Future research is required to further enhance our understanding of the risk of frailty among older adults.

To investigate mid- to long-term outcomes in children with coronary artery fistula (CAF). Methods: We retrospectively reviewed the medical records of patients seen between September 1996 and August 2011. We enrolled those diagnosed with CAF via echocardiography (Philips SONOS 7500 system and Philips IE33) or angiography. The mean follow time was 42.58 ± 3.4 months (range, 1–166 months). For comparative purposes, participants were grouped as acquired versus congenital, and symptomatic versus asymptomatic. We also measured the size of the coronary artery (CA) in patients with CA dilatation (CAD). Results: Out of 122 CAF patients, spontaneous closure was detected in 37 patients at 21.59 ± 3.45 months after diagnosis. This timeframe did not differ between the acquired and congenital groups (21.64 ± 6.26 months vs. 21.57 ± 4.15 months; p = 0.991). Ninety patients were asymptomatic and remained so; their spontaneous closure rate was 28.89%. Moreover, 24 patients had CAD, including 17 with Kawasaki disease and seven with congenital CAF. The CAs of all congenital-CAF-plus-CAD patients were initially > 5 mm; these patients underwent percutaneous transcatheter intervention, and their CA sizes decreased significantly (6.11 ± 0.79 mm vs. 3.76 ± 0.36 mm; p = 0.002). Conclusion: With the advanced sensitivity of echocardiography, CAF can be detected more easily than ever before. Most patients with small CAFs are asymptomatic and may experience spontaneous closure. Therefore, management of CAF depends on symptoms; if patients are asymptomatic and have small CAFs, intervention may not be necessary, especially in acquired cases. However, if patients present with symptoms or persistent dilatation of the proximal CA, surgical or percutaneous closure should be performed.

Background: Conventional therapy against acute pediatric cardiopulmonary failure (APCPF) caused by a variety of disease entities remains unsatisfactory with extremely high morbidity and mortality. For refractory APCPF, extracorporeal membrane oxygenation (ECMO) is one of the last resorts. Methods: In this study, the in-hospital outcomes of pediatric patients with refractory APCPF receiving ECMO support were reviewed. Results: Between August 2006 and May 2011, a single-center cohort study was performed in pediatric patients who required ECMO support due to cardiogenic shock or severe hypoxemia. A total of 22 patients with mean age of 7.0 ± 6.3 years received ECMO (male = 11; female = 11). The indications included acute fulminant myocarditis (AFM) (n = 6), congenital diaphragmatic hernia (CDH) (n = 3), acute respiratory distress syndrome (ARDS) (n = 6), enterovirus 71 (n = 3), viral sepsis (n = 2), refractory ventricular fibrillation due to long QT syndrome (n = 1), and pulmonary edema with brain herniation (n = 1). Eighteen patients received veno-arterial (VA) mode ECMO, while another four patients undertook the veno-venous (VV) mode. The duration of ECMO use and hospitalization were 6.1 ± 3.1 and 24.4 ± 19.4 days, respectively. The survival rate in patients with AFM was 100% (n = 6). Successful ECMO weaning with uneventful discharge from hospital was noted in 14 (63.6%) patients, whereas in-hospital mortality despite successful ECMO weaning occurred in 5 patients (22.7%). Failure in ECMO weaning and in-hospital death was noted in 3 patients (13.6%). Conclusions: ECMO resuscitation is an effective strategy in the clinical setting of APCPF.

Intrapulmonary shunting (IPS) due to pulmonary vascular dilatation is frequently observed among patients with end-stage liver disease (ESLD). This study investigated the prevalence and impact of IPS after liver transplantation (LT) in children. Methods: A total of 77 pediatric patients who underwent primary LT were enrolled. All patients had trans-thoracic contrast echocardiography before LT and at least 1 year after transplantation. The patients with IPS and without IPS after LT were designated as group 1 and group 2, respectively. Results: The prevalence of IPS after LT was 6.1%. The patients in group 1 (n=5) were younger (6.4 +/- 2.8 vs. 9.9 +/- 3.6, p=0.036) than in group 2 (n=72). There were no significant differences in gender, weight, hemoglobin level, O2 saturation, or complications between the two groups. Fourteen patients had abnormal liver function tests, two patients in group 1 and 12 patients in group 2 (p=0.22).The overall follow-up period was 6.7 +/- 2.7 years (range, 1.6–13.0). At the latest follow-up, all 5 patients with mild IPS after LT remain asymptomatic with good liver graft function. Conclusion: Among pediatric ESLD patients with preoperative IPS, approximately 6% continue to have mild IPS after LT. Patients with mild IPS after LT remain asymptomatic and have good liver graft function.

To study the influence of clinical audit on diagnosis, complications, and factors contributing to hospitalization of patients with infantile hypertrophic pyloric stenosis. Study Design: Retrospective cohort study. Method: There were 214 patients from 1991 to 2004 from three medical centers in Kaohsiung. Data were analyzed with respect to diagnostic methods, complications, and factors requiring patient hospitalization. Results: The ratio of male to female was 4.8:1 (177 males and 37 females). The diagnoses before admission were as follows: 22% had milk intolerance and 14.5% had esophageal reflux. There was a significant increase in the use of sonogram diagnostic test (p=0.005) and a decrease in the incidence of diagnosis by olive mass palpation but not by barium meal test. Surgery time of 48 hours after admission was significant with barium meal examination and related to longer hospital stay (p

Pediatric patients with atrial septal defect (ASD) may have failure to thrive. This study aimed to investigate body weight changes in pediatric patients after transcatheter closure of ASD. Methods: From June 2003 to September 2008, we enrolled 60 pediatric patients who underwent transcatheter closure of ASD. Changes in body weight percentile, heart rate, and resolution of right ventricular hypertrophy were compared before and after ASD closure. Patients were divided into two groups according to initial weight percentile: group A,

To evaluate the feasibility of echocardiography after transcatheter closure of patent ductus arteriosus (PDA) with coils. Methods: Between April 1998 and December 2006, 131 patients had transcatheter coil occlusion of their PDA using Gianturco coils. We hypothesized that post-procedural hemolysis would not occur if a residual shunt < 1 mm or if no continuous waveform was detected by echocardiography. Immediately after coil implantation, patients without and with a residual shunt as detected by echocardiography were designated to groups A and B, respectively. The clinical presentations, laboratory data and outcomes were compared between the two groups. Results: There were 101 patients in group A and 30 patients in group B. Patients in group B had larger ductal diameter (2.8 ± 0.9 mm vs. 1.6 ± 0.8 mm; p < 0.001), larger Qp/Qs (1.9 ± 0.9 vs. 1.3 ± 0.4; p = 0.001), higher frequency of more than one coil used (14/30 vs. 11/101; p < 0.001), and female predominance (22/30 vs. 53/101; p = 0.043) compared with group A. Trivial residual shunt was noted in 6 patients in group A and 20 patients in group B on the day after embolization. All shunts spontaneously closed within 6 months in patients of group A, while five patients in group B had a persistent shunt at the 1-year follow-up and thereafter. Although the patients in group B had higher residual shunt rate than group A during follow-up (p < 0.001), none of these patients suffered from hemolysis. Conclusion: Echocardiography is a feasible tool to assess residual shunt after PDA closure. If a residual shunt < 1 mm or if no continuous waveform is detected by echocardiography, the risk of developing hemolysis is low.

Balloon 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). Methods: Between 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. Results: There 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). Conclusions: The 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.

Myocarditis complicated with complete atrioventricular block (CAVB) is rare in children. The purpose of this study was to report the outcome of myocarditis with CAVB in our institution. Methods: Between June 1998 and June 2007, nine pediatric patients (aged from 1.5 to 16 years) were admitted, presenting with acute myocarditis with CAVB. We analyzed their clinical presentations, biochemistry and serology studies, chest X-rays, electrocardiograms, echocardiography, complications and outcomes. Results: Hypotension and Stokes-Adams seizures occurred in five and four of our patients, respectively. Cardiomegaly of chest X-ray was common in eight (89%) of our patients. Echocardiography revealed impaired left ventricular performance in three patients. Six patients suffered ventricular tachycardia (VT). Three cases of VT occurred before pacemaker implantation and the others occurred afterwards. Eight patients survived. Six of them regained sinus rhythm within 12 days (range 1−12 days), and two had a right bundle branch block at follow-up. Two patients had persistent CAVB, and one received permanent pacemaker implantation; the other received supportive care. One patient died due to persistent low cardiac output and a new onset of VT on the 4th admission day. During a follow-up period of 56 ± 27 months, all eight surviving patients remained asymptomatic. Conclusion: The outcome of CAVB complicated with myocarditis is variable. Most of our patients resumed normal heart function. The incidence of persistent CAVB was 22%. VT is a common and serious complication, but it can be effectively treated medically. Persistent low cardiac output after pacemaker implantation and late onset VT should be considered as risk factors of mortality.

To evaluate the outcome of transcatheter closure of atrial septal defects (ASD) with superior-anterior (SA) rim deficiency using Amplatzer septal occluder (ASO). Methods: Between June 2003 and March 2007, 84 patients with secundum type ASD attempted transcatheter insertion of ASO in our institution. According to the transesophageal echocardiographic findings, patients were divided into two groups: group A, with deficient SA rim (< 3 mm); group B, with sufficient SA rim (≥ 3 mm). There were 43 children and 41 adults (age range, 2.0–79.4 years; mean age, 22.0 ± 20.2 years). The failure rate, complications and the presence of residual shunt were compared between the two groups. Results: There were 34 patients in group A and 50 patients in group B. Failure of ASO implantation occurred in six patients, three in each group. One patient had two ASOs implanted for two separate ASDs. Therefore, the study cohort consisted of 78 patients with 79 ASO placed. Among 78 patients with successful implantation, five (6.4%) had persistent small residual shunt during follow-up (range, 1–46 months; mean, 21.6 ± 12.0 months). There was no statistically significant difference between group A and group B in the procedure's failure rate (p = 0.682), complications (p = 1.0) and the presence of residual shunt (p = 0.381) during the follow-up period. Conclusion: ASD with deficient SA rim is a common variation. Similar to ASD with sufficient rims, transcatheter closure of secundum type ASD is also effective for ASD with SA rim deficiency.

Intestinal pseudo-obstruction in atypical Kawasaki disease (KD) is rare. A boy aged 2 years and 6 months presented with a 7-day history of fever, coffee-ground vomit, and abdominal pain. Abdominal radiography and ultrasound showed a dilated duodenum. Peeling of the skin on his fingers and toes developed on hospitalization day 9. Echocardiogram revealed right and left coronary artery dilatation compatible with KD. He was treated with 2 g/kg intravenous immunoglobulin (IVIG), with rapid resolution of fever and relief of abdominal pain. Follow-up abdominal radiography and ultrasound showed improvement of bowel dilatation. This case illustrates that atypical KD can present with intestinal pseudo-obstruction. A high index of suspicion is required for early diagnosis, and prompt treatment with IVIG is recommended.

Background. Kawasaki disease (KD) is characterized by systemic vasculitis of unknown etiology. Our previous studies showed expression of CD40 ligand on CD4+ T cells correlated to the coronary artery lesion (CAL) and disease progress in KD. Other studies from Japan suggested the role of CD40L in the pathogenesis of CAL, and this might help explain the excessive number of males affected with KD but cannot be reproduced by Taiwanese population. This study was conducted to investigate the CD40 polymorphism in KD and CAL formation. Methods. A total of 950 subjects (381 KD patients and 569 controls) were investigated to identify 2 tagging single-nucleotide polymorphisms (tSNPs) of CD40 (rs4810485 and rs1535045) by using the TaqMan allelic discrimination assay. Results. A significant association was noted with regards to CD40 tSNPs (rs1535045) between controls and KD patients (P=0.0405, dominant model). In KD patients, polymorphisms of CD40 (rs4810485) showed significant association with CAL formation (P=0.0436, recessive model). Haplotype analysis did not yield more significant results between polymorphisms of CD40 and susceptibility/disease activity of KD. Conclusions. This study showed for the first time that polymorphisms of CD40 are associated with susceptibility to KD and CAL formation, in the Taiwanese population.

Kawasaki disease (KD) is characterized by systemic vasculitis of unknown etiology. High-dose intravenous immunoglobulin (IVIG) is the most effective therapy for KD to reduce the prevalence of coronary artery lesion (CAL) formation. Recently, the α2, 6 sialylated IgG was reported to interact with a lectin receptor, specific intracellular adhesion molecule-3 grabbing nonintegrin homolog-related 1 (SIGN-R1) in mice and dendritic cell-specific intercellular adhesion molecule-3 grabbing nonintegrin (DC-SIGN) in human, and to trigger an anti-inflammatory cascade. This study was conducted to investigate whether the polymorphism of DC-SIGN (CD209) promoter −336 A/G (rs4804803) is responsible for susceptibility and CAL formation in KD patients using Custom TaqMan SNP Genotyping Assays. A total of 521 subjects (278 KD patients and 243 controls) were investigated to identify an SNP of rs4804803, and they were studied and showed a significant association between the genotypes and allele frequency of rs4804803 in control subjects and KD patients (P=0.004 under the dominant model). However, the promoter variant of DC-SIGN gene was not associated with the occurrence of IVIG resistance, CAL formation in KD. The G allele of DC-SIGN promoter −336 (rs4804803) is a risk allele in the development of KD.

Kawasaki disease (KD) is characterized by systemic vasculitis with unknown etiology. Previous studies from Japan indicated that a gene polymorphism of ITPKC (rs28493229) is responsible for susceptibility to KD. We collected DNA samples from 1,531 Taiwanese subjects (341 KD patients and 1,190 controls) for genotyping ITPKC. In this study, no significant association was noted for the ITPKC polymorphism (rs28493229) between the controls and KD patients, although the CC genotype was overrepresented. We further combined our data with previously published case/control KD studies in the Taiwanese population and performed a meta-analysis. A significant association between rs28493229 and KD was found (Odds Ratio:1.36, 95% Confidence Interval 1.12-1.66). Importantly, a significant association was obtained between rs28493229 and KD patients with aneurysm formation (P = 0.001, under the recessive model). Taken together, our results indicated that C-allele of ITPKC SNP rs28493229 is associated with the susceptibility and aneurysm formation in KD patients in a Taiwanese population.