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Background: With the rapid development of perinatal medicine and neonatal resuscitation technology, neonatal mortality gradually reduces, but sepsis is still a neonatal critical illness and an important cause of death. The onset of sepsis in premature infants is insidious, and the clinical symptoms lack of specificity. The available laboratory tests exhibit limited sensitivity and specificity for diagnosis, and there is a certain degree of time lag. Therefore, it is particularly important to find a method for early detection of severe infection in premature infants. Main body: Sepsis is the third leading cause of death with poor prognosis in infants. Early and accurate identification of sepsis are particularly important. Heart rate variability may present before clinical symptoms of sepsis. The study is to summarize the available data on the relationship between heart rate variability and development of sepsis in early infants. We searched six database, PubMed, Web of Science, Embase, Cochrane Library, Wanfang, and CNKI, using the following terms for our search strategy (Infants, Newborn) OR (Newborn Infant) OR (Newborn Infants) OR (Newborns) OR (Newborn) OR (Neonate) OR (Neonates) AND (Sepsis) OR (Bloodstream Infection) OR (Bloodstream Infections) OR (Pyemia) OR (Pyemias) OR (Pyohemia) OR (Pyohemias) OR (Pyaemia) OR (Pyaemias) OR (Septicemia) OR (Septicemias) OR (Blood Poisoning) OR (Blood Poisonings) OR (Severe Sepsis) OR (Sepsis, Severe) AND (Heart Rate Variability). Premature infants would experience the reduction in heart rate variability before clinical symptoms of sepsis present. Conclusion: The application of heart rate variability, a noninvasive monitoring method, to the identification and diagnosis of sepsis in premature infants can enable clinicians to identify, diagnose, and treat children with sepsis early, thereby reducing the mortality of premature infants and neurological damage. [ABSTRACT FROM AUTHOR]

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 < 0.001 . The FGF-21 level was independently associated with aortic stiffness (odds ratio (OR): 1.008; 95% CI: 1.003–1.012; P = 0.001) after adjusting for diabetes mellitus, age, hypertension, C-reactive protein, and body weight in multivariable logistic regression analysis. Multivariable forward stepwise linear regression analysis also confirmed that the logarithmically transformed FGF-21 level (β = 3.245, 95% CI: 1.593–4.987, P < 0.001) was an independent predictor of cfPWV values. The area under the receiver operating characteristic (ROC) curve predicting aortic stiffness by the serum FGF-21 level was 0.693 (95% CI: 0.606–0.771, P < 0.001). Conclusions. Serum FGF-21 level positively correlates with cfPWV and is also an independent predictor of aortic stiffness in maintenance HD patients. [ABSTRACT FROM AUTHOR]

Objective: Kawasaki disease (KD) is a systemic vasculitis of unknown etiology and primarily affects children less than 5 years of age. Cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) has been suggested as a candidate gene for conferring susceptibility to autoimmunity. This study examined the correlation of CTLA-4 gene polymorphisms in KD with and without coronary artery lesions (CAL). Materials and methods: A total of 233 KD patients and 644 controls were subjected to determination of CTLA-4 polymorphisms at (−318) C/T and (+49) A/G positions by restriction fragment length polymorphism. Susceptibility, CAL, and intravenous immunoglobulin treatment response of KD were then analyzed with genetic variants. Results: Polymorphisms of CTLA-4 (+49 A/G) and (−318 C/T) were not significantly different between normal children and patients with KD. The CTLA-4 (+49) A allele (AA+AG genotype), however, was significantly associated with CAL formation, especially in female patients. Conclusions: This study provides the first evidence supporting the association of CTLA-4 (+49) A/G polymorphism with the CAL formation of KD particularly in female patients. [ABSTRACT FROM AUTHOR]

ent pulmonary valve syndrome is rare. We report the case of a child with absent pulmonary valve syndrome noted after birth. Progressive dilatation of the main pulmonary artery and ascending aorta were noted by echocardiography and confirmed by heart computed tomography. He underwent ascending aorta graft replacement and pulmonary artery reduction angioplasty at the age of 6. Good outcome was achieved at 18-month follow-up where subsequent computed tomography revealed a normal size ascending aorta and main pulmonary artery. [ABSTRACT FROM AUTHOR]

Objectives: Kawasaki disease (KD) is a systemic vasculitis primarily affecting children who are <5 years old. Intravenous immunoglobulin (IVIG) is the standard therapy for KD. However, many patients with KD still show poor response to initial IVIG treatment. This study was conducted to investigate the risk factors for initial IVIG treatment failure in KD. Methods: Children who met KD diagnosis criteria and were admitted for IVIG treatment were retrospectively enrolled for analysis. Patients were divided into IVIG-responsive and IVIG-resistant groups. Initial laboratory data before IVIG treatment were collected for analysis. Results: A total of 131 patients were enrolled during the study period. At 48 h after completion of initial IVIG treatment, 20 patients (15.3%) had an elevated body temperature. Univariate analysis showed that patients who had initial findings of high neutrophil count, abnormal liver function, low serum albumin level (≤2.9 g/dL) and pericardial effusion were at risk for IVIG treatment failure. Multivariate analysis with a logistic regression procedure showed that serum albumin level was considered the independent predicting factor of IVIG resistance in patients with KD (p = 0.006, OR = 40, 95% CI: 52.8–562). There was no significant correlation between age, gender, fever duration before IVIG treatment, haemoglobin level, total leucocyte and platelet counts, C-reactive protein level, or sterile pyuria and initial IVIG treatment failure. The specificity and sensitivity for prediction of IVIG treatment failure in this study were 96% and 34%, respectively. Conclusion: Pre-IVIG treatment serum albumin levels are a useful predictor of IVIG resistance in patients with KD. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the impact of mild and moderate intrapulmonary shunting (IPS) in children with end-stage liver disease (ESLD) undergoing liver transplantation. A total of 73 patients (38 male, 35 female; mean age 31.5 ± 35.2 months, range 6–180 months) with ESLD and subsequent liver transplantation were enrolled. Based on contrast echocardiography, patients without IPS were assigned to group 1 ( n = 57), and patients with mild or moderate IPS were assigned to group 2 ( n = 17). The preoperative age, body weight, O2 saturation, length of hospital stay, duration of mechanical ventilation, postoperative complications, and 1-year survival rate were compared between groups. The overall incidence of IPS and 1-year survival rate were 23.3% (17/73) and 96% (70/73), respectively. There were significant differences between group 1 and group 2 patients regarding age at transplant (35.9 vs. 16.6 months, p = 0.002) and body weight (12.6 vs. 8.5 kg, p = 0.002). There were no other statistically significant differences between the two groups. Although children with mild and moderate IPS were younger at the time of transplantation and had significantly lower body weight than those without IPS, the presence of mild and moderate IPS in children with ESLD did not affect the overall outcome of liver transplantation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The article presents a case study of an 2-year-old girl who was hospitalized due to high fever, cough and dyspnea for seven days in Taiwan. She was pronounced with bronchiolitis obliterans (BO) after the follow-up computed tomography (CT) that shows multiple hypoattenuated areas with mosaic perfusion in both lungs. The article discusses the BO as a rare disease caused by epithelial injury to the small airway resulting to the progressive obliteration and obstruction of the patients.

The article presents a case on the retrieval of a fractured peripherally inserted central catheter (PICC) remnant in the right femoral vein of a 56-day-old female infant using cardiac catheter. It offers information on the possible post-insertion complications of PICC, several factors that might have contributed to the spontaneous breakage of the PICC inside the infant and the risks involved in the retrieval of the PICC.

Kawasaki disease (KD) is a systemic febrile vasculitis particular coronary artery involvement. Eosinophilia has been found in our and other studies in KD. This study further investigates whether eosinophil-related T helper 2 (Th2) cytokines or the activation marker (eosinophil cationic protein – ECP) is involved in KD with coronary artery lesions (CAL). A total of 95 KD patients were enrolled for this study. Plasma samples were subjected to the measurement of interleukin (IL)-4, IL-5, and eotaxin by Luminex-Bedalyte multiplex beadmates system and to the measurement of ECP by fluoroimmunoassay. Patients with KD had higher eosinophils than controls. Eosinophil-related mediators: IL-4, IL-5, eotaxin, and ECP levels were also higher in KD patients than controls before intravenous immunoglobulin (IVIG) treatment. After IVIG treatment, ECP decreased but IL-4, IL-5, and eotaxin increased significantly. The higher the IL-5 and eosinophil levels after IVIG treatment, the lower rate of CAL was found. Changes of eosinophils after IVIG treatment were positively correlated to changes of IL-5 levels but not ECP levels. An increase of eosinophils and IL-5, but not ECP levels after IVIG treatment, was inversely correlated with CAL formation in KD. [ABSTRACT FROM AUTHOR]

To investigate the role of eosinophils in Kawasaki disease (KD) and the relationship to initial intravenous immunoglobulin (IVIG) treatment failure. A retrospective analysis of all children who were admitted and met the criteria of KD between 1999 and 2005. The patients were divided into IVIG-responsive and IVIG-resistant groups. A total of 185 patients were enrolled during the study period. A series of blood eosinophils and biochemistry studies were correlated to the effectiveness of IVIG. The neutrophils percentage before IVIG treatment (pre-IVIG), leukocyte counts within 3 days after IVIG treatment (post-IVIG), liver enzyme, albumin levels, and post-IVIG eosinophils percentage were all significantly different between the two groups in univariate analysis. Under multivariate analysis with logistic regression, post-IVIG eosinophilia [peripheral blood (PB) eosinophils ≥4%] had an inverse correlation to KD patients with IVIG-resistance (p = 0.003). Also, pre-IVIG hypoalbuminemia (albumin ≤3.0 g/dl) was positively correlated to IVIG-resistance (p = 0.018). Further analysis showed that the PB eosinophils was markedly increased in the acute stage and returned to normal 3 weeks after IVIG treatment (p < 0.001). Eosinophil levels are highly elevated in the acute stage of KD both before and after the IVIG treatment. Post-IVIG treatment eosinophilia has an inverse correlation to KD patients with IVIG-resistance and may indicate IVIG-responsive. This may be a valuable factor to survey for the necessity of a second dose IVIG treatment. [ABSTRACT FROM AUTHOR]

Background In this study, we investig1ated whether microRNA let-7i regulates dendric cell maturation targeting interleukin-10 (IL-10) via the Janus kinase 1–signal transducer and activator of transcription 3 (JAK1-STAT3) signal pathway subsequently prolongs rat cardiac allograft survival. Methods Quantitative real-time reverse transcriptase polymerase chain reaction, enzyme linked immunosorbent assay, and dual-luciferase assay were performed to verify whether IL-10 was the target of let-7i, and regulatory T cells were assessed by flow cytometry and immunohistochemical study. Western blot was performed to detect JAK1, STAT3, and phosphorylated STAT3 expression. Lewis recipients of Dark Agouti hearts were transfused with phosphate-buffered saline, lipopolysaccharide (LPS)-mature dendritic cells (mDCs), or let-7i–inhibitor-mDCs. Allograft survival times were recorded, and histologic studies were performed. Results Expression of IL-10 messenger RNA level and production of IL-10 were increased in let-7i–inhibitor-mDCs compared with LPS-mDCs. Luciferase activity showed that the translational level of the IL-10 luciferase reporter was decreased by let-7i mimic but increased by let-7i-inhibitor. MicroRNA let-7i inhibitor suppressed DC maturation; however, pretreatment of IL-10 small interfering RNA attenuated the suppression. Expression of JAK1, STAT3, and phosphorylated STAT3 in mDCs were suppressed by let-7i mimic, and pre-treatment of IL-10 small interfering RNA, however, were upregulated by let-7i inhibitor. Lewis recipients transfused with let-7i–inhibitor-mDCs significantly prolonged Dark Agouti cardiac allograft survival. The allografts transfused with let-7i–inhibitor-mDCs showed slight cell infiltration and significantly preserved graft structure. Inhibition of let-7i increased CD4 + CD25 + forkhead box P3 + regulatory T cells and modulated cytokine profiles in vivo and in vitro. Conclusions MicroRNA let-7i regulated DC maturation and function targeting IL-10 through the JAK1-STAT3 pathway. Moreover, transfusion of LPS-induced mDCs transfected with let-7i inhibitor induced prolonged cardiac allograft survival and generated regulatory T cells. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 post-operative tricuspid regurgitation in both groups. In applying our novel criterion (last post-operative 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract: 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 (β-actin, β-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 β-actin and β-tubulin were much lower in cord blood leukocytes than in adult leukocytes, and this expression pattern persisted in children up to 3years old. Further study revealed that the β-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 β-actin messenger RNA (mRNA). Further experiments showed that posttranslational regulation was responsible for the low β-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. [Copyright &y& Elsevier]

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 Callele of ITPKC SNP rs28493229 is associated with the susceptibility and aneurysm formation in KD patients in a Taiwanese population. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is characterized by systemic vasculitis of unknown etiology. A study from Japan reported that G to A substitution of a single-nucleotide polymorphism (SNP) located in the 5′-untranslated region of caspase 3 (CASP3) (rs72689236), which was associated with nuclear factor of activated T cell-mediated T-cell activation, is responsible for susceptibility to KD. This study was conducted to investigate whether the polymorphism of CASP3 is responsible for susceptibility and coronary artery lesion (CAL) formation in KD in the Taiwanese population. A total of 1092 subjects (341 KD patients and 751 controls) were investigated to identify an SNP of rs72689236 using Invader assays (Third Wave Technologies). Our data provided a borderline significant association between the genotypes and allele frequency of rs72689236 in control subjects and KD patients (P=0.0535 under the dominant model; P=0.0575 under the allelic model). The A allele of rs72689236 in KD patients and in patients with CAL and intravenous immunoglobulin resistance was seen in a higher frequency. Importantly, a significant association was obtained between rs72689236 and KD patients with aneurysm formation (P=0.009, under the recessive model). The A allele of rs72689236 is very likely to be a risk allele in the development of aneurysm in patients with KD. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) is the leading cause of acquired heart disease during childhood in the developed countries. Coronary artery lesions (CAL) are the major complications of KD. A unique proteomic profiling with increased or decreased fibrinogen, alpha-1-antitrypsin, clusterin, and immunoglobulin free light chains were noted in KD in our previous study. The purpose of this study was to evaluate relations between these biomarkers and CAL in KD and to establish within the markers the appropriate cut-off value with which to predict the occurrence of CAL. A total of 47 KD patients were enrolled, including 14 with CAL and 33 without CAL. Plasma samples from patients with KD before intravenous immunoglobulin administration were indicated for measurement of these biomarkers. A potential relation among CAL, clinical characteristics, and these biomarkers was investigated, and a receiver operating characteristic curve was used to identify a cut-off value of the significant marker that best predicated the occurrence of CAL. Among these biomarkers, only plasma clusterin level was associated with the occurrence of CAL. Using a cut-off value of clusterin <12.0 mg/l, the relative risk for CAL was 4.53-fold (95% confidence interval [CI] 1.060-19.347%, P = 0.014). Results from this study suggest that plasma clusterin level <12.0 mg/l in KD is significantly associated with the occurrence of CAL. Results from this study provide a potential biomarker of KD that may help predict the occurrence of CAL. [ABSTRACT FROM AUTHOR]