Mid- to long-term follow-up of pediatric patients with coronary artery fistula.

BACKGROUND/PURPOSE: 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.

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.

Small-bore pigtail catheters for the treatment of primary spontaneous pneumothorax in young adolescents.

OBJECTIVE: Small-bore pigtail catheters have been found to be effective in the treatment of primary spontaneous pneumothorax (PSP) in adults. The aim of this study was to compare the effectiveness of small-bore pigtail and large-bore catheters in the treatment of PSP in young adolescents. MATERIALS AND METHODS: Young adolescents (<18 years) with initial PSP were treated with aspiration (control group), small-bore pigtail catheters or large-bore catheters. Treatment was determined on a case-by-case basis with parental consultation. Success rate, recurrence rate (within 12 months), duration of hospital stay, duration of catheter insertion, and complications were analysed. MAIN RESULTS: There were 41 patients treated: aspiration, n=8; small-bore pigtail catheters, n=10; large-bore catheters, n=23. Demographic and baseline clinical characteristics were similar between groups. The success rates were 50.0% and 65.2% in the small-bore pigtail and large-bore catheter groups, respectively. Corresponding recurrence rates were 20.0% and 56.5%. There was no difference between the small-bore pigtail and large-bore catheter groups in the duration of hospital stay in patients for whom treatment was successful; however, the duration of catheter insertion was significantly shorter in the small-bore pigtail catheter group compared with the large-bore catheter group in patients for whom treatment was successful (p<0.05). There were no major complications in either catheter treatment group and few minor complications (small-bore pigtail catheter, n=2; large-bore catheter, n=4). CONCLUSIONS: The findings suggest that small-bore pigtail catheters may be as effective as large-bore catheters for the initial treatment of PSP in young adolescents.

Tricuspid regurgitation in acute phase of Kawasaki disease associated with intensive care unit admission.

Kawasaki disease (KD) is a systemic vasculitis and primarily affects children <5 years of age. Intensive care unit (ICU) admission is unusual, but there can be associated severe complications in KD patients. This study was conducted to identify risk factors for ICU admission. Retrospectively, we reviewed charts of all children who had a discharge diagnosis of KD from 2001 through 2009. Clinical presentation, laboratory data, and outcome were collected for analysis of the association with ICU admission in KD patients. Multifactor dimensionality reduction (MDR) was used to identify factor interactions. There were 334 KD patients, including 24 patients in ICU admission, included in the analysis. Coronary artery lesions (CALs) and failure of intravenous immunoglobulin (IVIG) treatment were more frequently found in the ICU group (P < 0.0001). Total counts of white blood cells, hemoglobin levels, C-reactive protein, and albumin levels showed significant association with ICU admission (P < 0.05). Moderate tricuspid regurgitation (TR) was found only in the ICU admission group. MDR analyses of factor interactions identified that TR interacted with CAL with a prediction accuracy of 77.78 %. (P = 0.001). Patients with KD who are IVIG resistant and/or who are found to have CALs are at increased risk for ICU admission. Most importantly, moderate TR was significantly found in KD patients only in the ICU group. This may highlight the great value of moderate TR in predicting ICU admission for patients with KD.

Predictors for Regression of Large Secundum Atrial Septal Defects Diagnosed in Infancy.

PUPOSE: To determine predictive factors of spontaneous closure or size reduction in large secundum-type atrial septal defects (ASD) diagnosed in infancy prior to catheterization or surgical intervention. METHODS: From June 2003 to October 2009, 59 infants diagnosed with secundum-type ASDs measuring ≥ 8 mm in the first year of life were retrospectively enrolled. We reviewed medical records, as well as electrocardiography and echocardiography findings. Patients were divided into 2 groups according to the last ASD size: group A (n = 23), ASD reduction in size to < 5 mm or spontaneous closure; group B (n = 36), size of ASD remained ≥ 5 mm. RESULTS: The ASDs spontaneously closed in 10 (17%) patients at a median age of 26.0 ± 5.1 months (range, 15-58 months), or decreased to < 5 mm in 13 (22%) (range, 6-27 months) patients. There was a significant difference in age at diagnosis between the 2 groups (p = 0.014). Patients in group A were younger than those in group B at the time of diagnosis. Changes in ASD size (p < 0.001) and body weight percentile (p = 0.01) were also significantly different fromthe 6-month follow-up. ASD diameter of ≥ 10 mm was a negative predictive factor for size reduction (p = 0.017). CONCLUSIONS: Spontaneous closure or size reduction of large ASDs was not uncommon in patients diagnosed during infancy. Patients with initial ASD sizes between 8 and 10 mm who were younger at the time of diagnosis and showed better weight gain were more likely to have favorable outcomes. KEY WORDS: Infancy; Large secundum atrial septal defect; Natural course; Spontaneous closure.

The impact of intrapulmonary shunting after liver transplantation in pediatric patients.

BACKGROUND/PURPOSE: 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, O(2) 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.

CD40 Gene polymorphisms associated with susceptibility and coronary artery lesions of Kawasaki disease in the Taiwanese population.

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.

DC-SIGN (CD209) promoter -336 A/G (rs4804803) polymorphism associated with susceptibility of Kawasaki disease.

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.

Lack of association between CLEC5A gene single-nucleotide polymorphisms and Kawasaki disease in Taiwanese children.

BACKGROUND: Kawasaki disease is characterized by systemic vasculitis of unknown etiology. Previous genetic studies have identified certain candidate genes associated with susceptibility to KD and coronary artery lesions. Host innate immune response factors are involved in modulating the disease outcome. The aim of this study was to investigate CLEC5A (C-type lectin domain family 5) genetic polymorphisms with regards to the susceptibility and outcome of KD. METHODS: A total of 1045 subjects (381 KD patients and 664 controls) were enrolled to identify 4 tagging single-nucleotide polymorphisms (tSNPs) of CLEC5A (rs1285968, rs11770855, rs1285935, rs1285933) by using the TaqMan Allelic Discrimination Assay. The Hardy-Weinberg equilibrium was assessed in cases and controls, and genetic effects were evaluated by the chi-square test. RESULTS: No significant associations were noted between the genotypes and allele frequency of the 4 CLEC5A tSNPs between controls and patients. In the patients, polymorphisms of CLEC5A showed no significant association with coronary artery lesion formation and intravenous immunoglobulin treatment response. CONCLUSIONS: This study showed for the first time that polymorphisms of CLEC5A are not associated with susceptibility to KD, coronary artery lesion formation, and intravenous immunoglobulin treatment response in a Taiwanese population.

Two new susceptibility loci for Kawasaki disease identified through genome-wide association analysis.

To 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.

Higher levels of soluble Fas ligand and transforming growth factor-ß after omalizumab treatment: a case report.

A skewed T-helper (T(h))1/T(h)2 immune response is considered to be the major cause of allergic disorders. Overproduction of T(h)2 cytokines, which promote recruitment and activation of mast cells and eosinophils, plays a key part in the pathogenesis of allergic asthma. The mechanisms by which omalizumab is effective in asthma treatment are not yet fully understood. A 16-year-old girl who was experiencing frequent asthma attacks in spite of daily administration of budesonide (640 µg) and montelukast (10mg) was given omalizumab (375 mg) at intervals of 2 weeks, to prevent a visit to the emergency room. Plasma levels of T(h)1 cytokines [interferon (IFN)-γ and interleukin (IL)-12p70], T(h)2 cytokines (IL-4 and IL-13), other proinflammatory and regulatory cytokines [IL-6, IL-10, IL-17, tumor necrosis factor (TNF)-α, and transforming growth factor (TGF)-ß], chemokines [monocyte chemotactic protein (MCP)-1, chemokine ligand (CCL)7, and CCL17], and soluble Fas ligand (sFasL) were measured before treatment and after treatment for 8 weeks. She showed a good clinical response to omalizumab: her lung function parameters improved and the use of ß2-agonist decreased. No emergency room visits were required after omalizumab treatment for 8 weeks. Plasma levels of sFasL and TGF-ß showed obvious increases after omalizumab therapy. IL-12p70 levels were decreased as compared to the corresponding baseline levels. These findings suggest that the effects of omalizumab in asthma treatment are not restricted to the regulation of the skewed T(h)1/T(h)2 cytokine immune response, and sFasL-mediated apoptosis and regulatory T-cell (Treg)-mediated TGF-ß seem to have important roles in the therapeutic effects of omalizumab.

Monoarticular septic arthritis in a patient with juvenile rheumatoid arthritis under etanercept treatment.

A 7-year-old girl with polyarticular type juvenile rheumatoid arthritis (JRA) presented with acute onset of right hip pain with limited range of motion and fever within the past two days. She had received etanercept for more than one year. Percutaneous arthrocentesis was performed and showed a white blood cell count of 84150/µL in the synovial fluid, although the culture showed negative results. The fever and right hip pain completely resolved after antibiotic treatment. Herein, we report the first case of septic monoarthritis of JRA under etanercept treatment.

Novel method for evaluating tricuspid valve function after tricuspid valve detachment in the repair of perimembranous ventricular septal defects.

Tricuspid valve detachment has been used for decades in the repair of type II ventricular septal defects (VSDs); however, the procedure can damage the tricuspid valve and conduction system. We retrospectively reviewed 177 consecutive type II VSD repairs performed at our hospital from 1997 through 2004. Patients were included if they had symptoms, pulmonary hypertension, or a Qp/Qs ratio>1.5: 86 underwent tricuspid valve detachment (TVD group) and 84 underwent VSD repair without this detachment (non-TVD group). There was no significant difference between groups in age, body weight, VSD size, Qp/Qs ratio, follow-up duration, or incidence of residual shunting. Cross-clamp times (109.6±42.6 vs 92.2±38.1 min) and cardiopulmonary bypass times (155.1±53.8 vs 137±47 min) were longer in the TVD group. No patients developed tricuspid stenosis or heart block. After excluding patients who underwent tricuspid repair, we found similar grades of postoperative tricuspid regurgitation in both groups. In applying our novel criterion (last postoperative regurgitation grade minus preoperative regurgitation grade) to evaluate changes between preoperative and postoperative tricuspid regurgitation, we found significant deterioration in the non-TVD group (P=0.018). Had conventional evaluation methods been used, severity in the groups would not have differed significantly. Our method enables additional evaluation of late tricuspid function in individual patients. Tricuspid valve detachment is safe for type II VSD repair and has no adverse effect on late tricuspid valve function. In addition, we recommend the interrupted-suture technique for leaflet reattachment.

Factors contributing to prolonged hospitalization of patients with infantile hypertrophic pyloric stenosis.

BACKGROUND: 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<0.001). Weight gain less than 800 g before admission (n=125) was related to longer hospital stay (p=0.026). CONCLUSION: The diagnostic method was changed from olive mass palpation to sonogram. Weight gain less than 800 g before admission and surgery time of 48 hours after admission were related to longer hospital stay.

Transpulmonary arterial repair of type 1 ventricular septal defect will induce pulmonary regurgitation: appraisal of subpulmonic approach.

OBJECTIVE: A transpulmonary arterial approach to repair type I ventricular septal defect is the standard. However, the possible adverse effect on the pulmonary valve by this technique has not been investigated. METHODS: A retrospective study reviewing type I ventricular septal defect repair from January 1991 to May 2010 was conducted. Of the 142 cases, the ventricular septal defect was repaired via the transpulmonary arterial route in 77 patients (54.2%, PA group) and via the subpulmonic route in 65 patients (45.8%, SP group). All patients received serial annual transthoracic color Doppler echocardiographic evaluation of pulmonary valve function. RESULTS: The age at operation ranged from 1.2 to 272 months (median, 35.0 months; mean, 50.4 months). The mean follow-up period was 96.2 months (range, 2-234 months). Between the PA and SP groups, there was no significant difference in age, body weight, ventricular septal defect size, left to right shunt amount, mean pulmonary arterial pressure, and preoperative pulmonary stenosis or regurgitation. Postoperatively, there was no significant difference in the ejection fraction or incidence of residual ventricular septal defect, right bundle branch block, and pulmonary stenosis. However, the incidence of postoperative pulmonary regurgitation of more than moderate and the total scale of postoperative pulmonary regurgitation were both significantly higher in the PA group (16.9% vs 4.6% and 1.7 ± 0.1 vs 1.4 ± 0.1, P = .031 and .019, respectively). CONCLUSIONS: Although the transpulmonary arterial approach for type I ventricular septal defect repair has been advocated for decades, considering the adverse effect on pulmonary valve competency, the subpulmonic approach may be an alternative.

Impact of transcatheter closure of atrial septal defects in pediatric patients on body weight.

BACKGROUND/PURPOSE: 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, <50(th) percentile (n = 39) and group B, ≥50(th) percentile (n = 21). Echocardiography and routine weight measurements were performed before the procedure and at 3, 6, and 12 months during follow-up. Clinical presentations, laboratory data, and outcomes were measured. RESULTS: Increased body weight percentile (41±4 vs. 48±4, p<0.01), lower heart rate (100±2 beats/min vs. 89±2beats/min, p<0.01), and resolution of right ventricular hypertrophy (59/60 vs. 1/60, p<0.01) were achieved after ASD closure at the 12-month follow-up. Patients in group A were significantly younger (4.6±0.5 years vs. 7.0±0.9 years, p = 0.016), had a higher pulmonary/systemic blood flow ratio (2.2±0.1 vs. 1.8±0.l, p = 0.044), a largerratio of ASD diameter/body surface area (25.0±1.4 vs. 16.4±1.9, p<0.01), and higher percentage of weight gain increase ≥ 5 percentile compared with patients in group B (22/39 vs. 6/21, p = 0.039). CONCLUSION: Transcatheter closure of ASD positively affects weight gain. An increase of 7 percentile weight was observed at 1 year of follow-up. Patients with a younger age, higher pulmonary/systemic blood flow ratio, and a larger ratio of ASD diameter/body surface area may have better weight gain after ASD closure.

ITPKC single nucleotide polymorphism associated with the Kawasaki disease in a Taiwanese population.

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.

Lack of association between ORAI1/CRACM1 gene polymorphisms and Kawasaki disease in the Taiwanese children.

OBJECTIVE: Kawasaki disease (KD) is characterized by systemic vasculitis of an unknown cause. A previous study has indicated that a polymorphism of the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene is involved in the susceptibility to KD. ORAI (also known as CRACM1) is one of the components of store-operated calcium channels involved in regulating immune and inflammatory reactions. This study was conducted to investigate if polymorphisms in ORAI1/CRACM1, a gene downstream from ITPKC, are associated with KD susceptibility and clinical outcomes. MATERIALS AND METHODS: A total of 1,056 subjects (341 KD patients and 715 controls) were investigated to identify five tagging single nucleotide polymorphisms (tSNPs) in ORAI1/CRACM1 (rs12313273, rs6486795, rs7135617, rs12320939, and rs712853) by using the TaqMan Allelic Discrimination assay. RESULTS: No significant associations between genotype and allele frequency of the five ORAI1/CRACM1 tSNPs were observed in the KD patients and controls. In KD patients, no significant associations between ORAI1/CRACM1 polymorphisms and coronary artery lesion (CAL) formation or intravenous immunoglobulin (IVIG) treatment response were observed. The results from haplotype analysis were insignificant. CONCLUSIONS: This study showed for the first time that ORAI1/CRACM1 polymorphisms are not associated with KD susceptibility, CAL formation, or IVIG treatment response in the Taiwanese population.

Glyceraldehyde-3-phosphate dehydrogenase is a reliable internal control in Western blot analysis of leukocyte subpopulations from children.

To study differences in the development of immunity, leukocytes from cord blood are often compared with those from adult peripheral blood. Western blot analysis is a common method for detecting proteins. In this study, we investigated the reliability of using different housekeeping proteins (ß-actin, ß-tubulin, and glyceraldehyde-3-phosphate dehydrogenase [GAPDH]) as internal controls for different leukocyte subpopulations from infants, children, and adults. Our results showed that the expression levels of ß-actin and ß-tubulin were much lower in cord blood leukocytes than in adult leukocytes, and this expression pattern persisted in children up to 3 years old. Further study revealed that the ß-actin expression level in newborns was especially lower in CD14-positive monocytes. However, cord blood and adult peripheral blood monocytes had similar expression levels of ß-actin messenger RNA (mRNA). Further experiments showed that posttranslational regulation was responsible for the low ß-actin expression level in neonatal monocytes. Thus, researchers should carefully assess the appropriate use of housekeeping gene-encoded proteins as internal standards to normalize samples for comparisons of different leukocyte populations from subjects of different ages. In this study, we determined that GAPDH was a more reliable internal control than others in Western blot analysis for comparing the development of immunity among infants, children, and adults.

Double precardinal anastomoses with interconnecting venous plexus hypothesis in the embryogenesis of anomalous brachiocephalic veins: computed tomography and magnetic resonance findings.

PURPOSE: The aim of this study was to assess the hypothesis of double precardinal transverse anastomoses with an interconnecting venous plexus in the embryogenesis of anomalous brachiocephalic veins (ABCV) based on computed tomography (CT) and magnetic resonance (MR) findings. MATERIALS AND METHODS: Between January 2000 and September 2009, of 25,940 thoracic CT or MR imaging studies, 27 patients with developmental ABCV were encountered in our institution. Among them, 15 pediatric patients underwent CT or MR studies due to suspected complex thoracic vascular anomalies that were not well defined on echocardiograms. In the other 12 adult patients, ABCVs were incidentally found during cancer workup or evaluation of mediastinal widening seen on the chest radiographs. The anatomic variations of ABCV demonstrated on CT or MR imaging were retrospectively reviewed, and the implications of ABCV variations in the embryogenesis of ABCV were assessed. RESULTS: Five types of ABCV were revealed: supraaortic retrovascular left ABCV (n=1), left subaortic ABCV (n=6), double superior venae cavae with hypoplastic bridging ABVC (n=5), double superior venae cavae with absence of the brachiocephalic vein (BCV) (n=14), and double left BCV (n=1). The 5 types of ABCV could be explained by the persistence and regression of different parts of the double precardinal transverse anastomoses and interconnecting venous plexus. CT also demonstrated interconnecting veins to the BCV in 1 case and nipple-like remnants of interconnecting veins on the BCV in 2 cases, providing direct imaging evidence of the presence of the interconnecting veins. CONCLUSION: The hypothesis of double precardinal anastomoses with interconnecting veins offers a concise but comprehensive explanation of various kinds of ABCV.