Ying-Jui Lin

Correlation of pandemic (H1N1) 2009 viral load with disease severity and prolonged viral shedding in children.

Younger children may require a longer isolation period and more aggressive treatment.

Augmented TLR2 Expression on Monocytes in both Human Kawasaki Disease and a Mouse Model of Coronary Arteritis

Background Kawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis. Methods Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG) treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old) were intraperitoneally injected with LCWE (1 mg/mL) to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14. Results Both human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL)-2, IL-6, IL-10, monocyte chemoattractant protein (MCP)-1, and tumor necrosis factor (TNF)-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR)-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment. Conclusion This result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by modulating TLR2-mediated immune activation on CD14+ monocytes.

Myocarditis Complicated by Complete Atrioventricular Block: Nine Years' Experience in a Medical Center

BACKGROUND 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. CONCLUSIONS 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.

Aminoguanidine attenuates hypertension, whereas 7-nitroindazole exacerbates kidney damage in spontaneously hypertensive rats: the role of nitric oxide.

Nitric oxide (NO) deficiency contributes to hypertension and end-organ damage. Three nitric oxide synthase (NOS) isoforms have been identified: neuronal NOS (nNOS), inducible NOS (iNOS), and endothelial NOS (eNOS). Whether selective nNOS or iNOS inhibition exacerbates kidney damage in spontaneously hypertensive rats (SHRs) remains unclear. Seven-week-old SHRs were randomly assigned to 4 groups (n=8 for each group): group 1, SHRs receiving no treatment; group 2 (SHR+7-NI), SHRs given 7-nitroindazole (7-NI, nNOS inhibitor) in their drinking water (10mg/kg/day); group 3 (SHR+salt), SHRs given 1% NaCl; and group 4 (SHR+AG), SHRs given 0.1% aminoguanidine (AG; iNOS inhibitor) in drinking water. The mean arterial pressure of SHRs treated with salt was significantly elevated compared with untreated controls. While AG caused a decrease of mean arterial pressure at 8 and 12 weeks of age in SHRs, both 7-NI and salt exacerbated kidney injury. In addition, AG significantly increased l-arginine levels and the l-arginine-to-asymmetric dimethylarginine (ADMA) ratio in the kidney. Salt treatment decreased renal nNOS-α protein levels and reduced dimethylarginine dimethylaminohydrolase (DDAH) activity. Salt and AG treatment increased nNOS-β and l-citrulline levels in SHR kidneys. AG attenuates hypertension development by upregulation of l-citrulline-to- l-arginine conversion and an increase in the l-arginine-to-ADMA ratio in SHR kidneys. 7-NI impairs renal function but has no effect on blood pressure, suggesting reno-protective role for the nNOS. Salt exacerbates kidney damage mainly through decreasing renal nNOS-α protein levels and DDAH activity. Our findings highlight the protective role of the nNOS/NO pathway in the development of kidney damage in SHRs.

Early Differentiation of Kawasaki Disease Shock Syndrome and Toxic Shock Syndrome in a Pediatric Intensive Care Unit.

Background: Kawasaki disease shock syndrome (KDSS) and toxic shock syndrome (TSS) can present as shock and fever with skin rash, but the management of these 2 groups of patients is different. This report proposes to help clinicians earlier distinguish these 2 diseases and expedite institution of appropriate therapy. Methods: We retrospectively reviewed the medical records of patients admitted to the pediatric intensive care unit with the diagnosis of KDSS or TSS from January 2000 through December 2010. Clinical, laboratory and echocardiographic data were collected for analysis of differences between them. Results: Seventeen patients met the inclusion criteria of KDSS and 16 had a confirmed diagnosis of TSS. The mean age of the KDSS group was significantly younger than that of the TSS group (36.8 ± 41.1 vs. 113.3 ± 55.6 months, P < 0.001). Significantly lower hemoglobulin and age-adjusted hemoglobulin concentrations were noted in the KDSS group [Hb, age-adjusted Z score, −1.88 (range, −3.9 to 3.9) vs. 0.89 (range, −6.4 to 10.8), P = 0.006]. The median platelet count of the KDSS group was nearly twice that of the TSS group [312 × 103 per &mgr;L (range, 116–518) vs. 184.5 × 103 per &mgr;L (range: 31–629), P = 0.021]. Echocardiographic abnormalities, such as valvulitis (mitral or tricuspid regurgitation) and coronary artery lesions, were significantly more common in the KDSS group (P = 0.022). Conclusions: Echocardiography, anemia and thrombocytosis are useful early differentiating features between KDSS and TSS patients.

Transcatheter Closure of Atrial Septal Defects with Superior-anterior Rim Deficiency Using Amplatzer Septal Occluder

BACKGROUND/PURPOSE 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 (> or = 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 procedures 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.

Transcatheter Closure of a Left Circumflex Coronary Artery Fistula in Two Children Using the Amplatzer Vascular Plug

A left circumflex coronary artery fistula (CAF) is a rare anomaly. This report describes two young children with progressive left coronary artery dilation due to left CAF demonstrated by serial echocardiography. Cardiac catheterization performed for both children confirmed the presence of a markedly tortuous and dilated left circumflex artery, with the CAF draining directly into the right ventricle. Transcatheter closure of the CAF using the Amplatzer vascular plug was successfully accomplished without any complications. A good outcome was achieved. The echocardiography at the 12- and 18-month follow-up visits showed reversion of the coronary artery to normal size.

Balloon Angioplasty for Native Coarctation of the Aorta in Neonates and Infants With Congestive Heart Failure

BACKGROUND 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, 2.1-8.0kg). 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.

Impact of extracorporeal membrane oxygenation support on clinical outcome of pediatric patients with acute cardiopulmonary failure: a single-center experience.

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.

Predictors of Atrial Septal Defect Occluder Dislodgement.

The aim of this study was to identify the factors that influence atrial septal occluder dislodgement in adults and children.From June 2003 to June 2013, a total of 213 patients (115 adults and 98 children) diagnosed with secundum atrial septal defects (ASD) underwent transcatheter closure of their defects with an atrial septal occluder (ASO) in our hospital. The ASO was implanted under transesophageal echocardiography (TEE) guidance. Ten patients suffered from ASO dislodgement, and the other 203 patients comprised the successful group. We compared the preprocedural data related to general demographics, defects, margins, and minor post-implantation complications between the two groups with the goal of identifying the factors that affected ASO dislodgement.Univariate logistic regression analyses identified a high Qp/Qs value, the Qp/Qs ratio > 3.13, ASO size, ASO size greater than 32 mm, ASO size/BSA ratio > 15.13 and IAS erosion, floppiness or aneurysm formation as factors with significant predictive value. Multivariate analysis revealed that a Qp/Qs ratio > 3.13, and interatrial septum (IAS) erosion, floppiness and aneurysm formation post-implantation were independent predictors of ASO dislodgement (P = 0.001 and P = 0.006, respectively) in both adults and children.Percutaneous device closure of ASDs is safe and effective in the current era. The Qp/Qs ratio > 3.13 and IAS erosion, floppiness or aneurysm formation post-implantation might be predictors of ASO dislodgement in adults and children.