Kawasaki disease (KD) or Kawasaki syndrome is known as a vasculitis of small to medium-sized vessels, and coronary arteries are predominantly involved in childhood. Generally, 20–25% of untreated with IVIG and 3–5% of treated KD patients have been developed coronary artery lesions (CALs), such as dilatation and aneurysm. Understanding how coronary artery aneurysms (CAAs) are established and maintained in KD patients is therefore of great importance. Upon our previous genotyping data of 157 valid KD subjects, a genome-wide association study (GWAS) has been conducted among 11 (7%) CAA-developed KD patients to reveal five significant genetic variants passed pre-defined thresholds and resulted in two novel susceptibility protein-coding genes, which are NEBL (rs16921209 (P = 7.44 × 10−9; OR = 32.22) and rs7922552 (P = 8.43 × 10−9; OR = 32.0)) and TUBA3C (rs17076896 (P = 8.04 × 10−9; OR = 21.03)). Their known functions have been reported to associate with cardiac muscle and tubulin, respectively. As a result, this might imply their putative roles of establishing CAAs during KD progression. Additionally, various model analyses have been utilized to determine dominant and recessive inheritance patterns of identified susceptibility mutations. Finally, all susceptibility genes hit by significant genetic variants were further investigated and the top three representative gene-ontology (GO) clusters were regulation of cell projection organization, neuron recognition, and peptidyl-threonine phosphorylation. Our results help to depict the potential routes of the pathogenesis of CAAs in KD patients and will facilitate researchers to improve the diagnosis and prognosis of KD in personalized medicine.
Kawasaki disease (KD) is a childhood vasculitides associated with serious coronary artery lesions. It is the most common cause of pediatric acquired heart disease in developed countries, and is increasingly reported from many rapidly industrializing developing countries. The incidence varies widely among different nations and is highest in North-East Asian countries, where almost 1 in 100 children in Japan having the disease by age of 5, where the lowest incidence reported in sub-Saharan Africa. The etiology of KD is still uncertain; interaction between a genetic predisposition and several environmental and immunological factors has been hypothesized. Several susceptibility genes were identified to be associated with the development of KD and increased risk of coronary artery lesions. Gene-gene associations and alteration of deoxyribonucleic acid (DNA) methylation are also found to play key roles in the pathogenesis and prognosis of KD. This article will focus on the global epidemiological patterns of KD, and the currently known genetic predisposition.
Global EpidemiologyKD has been documented in more than 60 countries and cross all ethnicities [4,17] (Fig. 1, [2]). The incidence of KD is in-
ObjectiveTo evaluate retrospectively the relationship between meteorological factors in Shenmu County, Yulin City, Shaanxi Province, China and the incidence of lower respiratory tract infections in children.MethodsMeteorological data (air temperature, atmospheric pressure, rainfall, hours of sunlight, wind speed and relative humidity) for Shenmu County and medical data from hospitalized patients aged ≤16 years were collected between January 2009 and December 2012. The association between meteorological factors and rate of hospitalization due to lower respiratory tract infections was investigated; the total hospitalization rate was compared with the rate of lower respiratory tract disease-related hospitalizations.ResultsThe leading bacterial causes of lower respiratory tract infections were Streptococcus pneumoniae and Haemophilus influenzae type B; the main viral cause was respiratory syncytial virus. Lower respiratory tract infection hospitalization rate was significantly correlated with air temperature (R = −0.651), atmospheric pressure (R = 0.560), rainfall (R = −0.614) and relative humidity (R = −0.470), but not with hours of sunlight (R = −0.210) or wind speed (R = 0.258). Using multiple linear regression, lower respiratory tract infection hospitalization rate decreased with a gradual increase in air temperature (F = 38.30) and relative humidity (F = 15.58).ConclusionAir temperature and relative humidity were major influencing meteorological factors for hospital admissions in children due to lower respiratory tract infections.
The Chinese developmental curves obtained from the GDS-C showed similarities and differences to the developmental curves from the British GMDS-ER. The development of urban Chinese children should be assessed with the culturally appropriate GDS-C.
Kawasaki disease (KD) is a common vasculitis in children and is the commonest cause of pediatric acquired heart disease in children in Japan and countries in North America and the European Union. It is now being increasingly reported from several developing countries, including China and India. Diagnosis of KD is based on a set of clinical criteria, none of which is individually pathognomonic for this condition. Further, these clinical features appear sequentially over a few days and all findings may not be present at a given point of time. Like many other vasculitides, there is no confirmatory laboratory test for KD. Treatment of KD involves use of intravenous immunoglobulin (IVIg) and aspirin. IVIg is an expensive product and poses several difficulties for patients in developing countries who may find it difficult to access therapy even if a diagnosis of KD has been made in time. In this review, the authors discuss some of these challenges that pediatricians have to face while managing KD in resource constrained settings.
Our intent was to explore the predictive value of body mass index (BMI) in differentiating between vasovagal syncope (VVS) and postural tachycardia syndrome (POTS) in children and adolescents. A total of 111 children and adolescents with POTS and 154 children and adolescents with VVS were included in our study. The control group included 82 healthy children and adolescents. Height and weight were measured in all participants. The headup tilt test was performed in participants in all groups (POTS, VVS, and control). BMI was significantly lower in children and adolescents with POTS (18.3±3.4) than in children and adolescents with VVS (20.3±4.2) and the control group (20.5±2.9). The receiver operating characteristic curve was performed to determine the predictive value of BMI differentiation between POTS and VVS and showed that a BMI of 19.30 was the cutoff value for the probability of distinction. However, the results (BMI of 19.30) produced unsatisfactory sensitivity (57.1%) and specificity (28.8%) rates of correctly discriminating between patients with POTS and patients with VVS. Children and adolescents with POTS have a lower BMI compared with healthy peers or children and adolescents with VVS.
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