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Aim This study aimed to investigate sensitive factors involved in left ventricular mass reduction in children with end-stage renal disease (ESRD) undergoing peritoneal dialysis. Methods Thirty-five subjects on peritoneal dialysis were enrolled. Two successive echocardiographic and clinical data for each subject were obtained. Blood pressure and left ventricular mass index (LVMI) were indexed through a division with the normal 95th percentile value. Differences in numeric data between two datasets were calculated. Results The mean age was 12.9 ± 4.6 years. Predictors of left ventricular hypertrophy and its persistence were systolic blood pressure index (P = 0.019 and P = 0.046) and E' velocity (P = 0.035 and P = 0.031) in univariate analysis. However, differences in these predictors between the datasets were not related to the change in indexed LVMI. Reduction in indexed LVMI was correlated to a reduction of indexed left atrial volume (R = 0.638, P = 0.001), trans-mitral A velocity (R = 0.443, P = 0.011), and serum blood urea nitrogen level (R = 0.372, P = 0.028) and an elevation of haemoglobin level (R = -0.374, P = 0.027). Conclusion The extent of circulating volume expansion is potentially the main predictive factor for change of LVMI, because the volume dependent diastolic functional variables correlate to the change of LVMI. Further study with a large number of ESRD children including a group under fluid volume control is needed to investigate the role of volume expansion on the change of LVMI.

Kawasaki disease (KD) is an acute pediatric vasculitis that affects genetically susceptible infants and children. To identify coding variants that influence susceptibility to KD, we conducted whole exome sequencing of 159 patients with KD and 902 controls, and performed a replication study in an independent 586 cases and 732 controls. We identified five rare coding variants in five genes (FCRLA, PTGER4, IL17F, CARD11, and SIGLEC10) associated with KD (odds ratio [OR], 1.18–4.41; p = 0.0027–0.031). We also performed association analysis in 26 KD patients with coronary artery aneurysms (CAAs; diameter > 5 mm) and 124 patients without CAAs (diameter < 3 mm), and identified another five rare coding variants in five genes (FGFR4, IL31RA, FNDC1, MMP8, and FOXN1), which may be associated with CAA (OR, 3.89–37.3; p = 0.0058–0.0261). These results provide insights into new candidate genes and genetic variants potentially involved in the development of KD and CAA.

Background and Objectives Kawasaki disease (KD) is an acute systemic vasculitis that affects the coronary arteries. Abnormal immune reactions are thought to contribute to disease pathogenesis. The effect of immunoglobulin (Ig) isotype (IgG, IgA, IgM, and IgE) on inflammatory data and clinical outcomes of patients with KD was examined. Methods Ig levels in 241 patients with KD were measured during the acute, subacute, convalescent, and normal phases of the disease. Results Compared with reference Ig values, IgG, IgA, and IgM levels were significantly higher in the subacute phase, while IgE levels were elevated in 73.9% (178/241) of patients with KD in all clinical phases. However, high IgE levels were not associated with clinical outcomes, including intravenous immunoglobulin unresponsiveness and coronary artery lesions (CALs). Significantly more CALs were observed in the high IgA group than in the normal IgA group (44.7% vs. 20.8%, respectively; p

Introduction Stiff person syndrome is a rare autoimmune, neurological disorder characterized by progressive rigidity and episodic painful spasms, predominantly affecting the proximal limbs and axial muscles, and leading to progressive disability. We report the case of a child who developed symptoms compatible with stiff person syndrome during treatment for pleuropulmonary blastoma. Patient Description A 3-year, 5-month-old girl was admitted for gradually worsening postural tremor, painful spasms, and generalized stiffness. Since the age of 3 years, she had been on adjuvant chemotherapy for pleuropulmonary blastoma before surgical resection. Brain magnetic resonance imaging and electroencephalographic findings were normal. Although serologic tests for autoimmune disease, including paraneoplastic antibodies and antiglutamic acid decarboxylase antibodies, were unremarkable, her findings were attributed to a paraneoplastic syndrome based on her clinical features and medical history. However, following the planned pulmonary lobectomy, her symptoms were paradoxically aggravated, with continuous motor unit potential at rest on electromyography, which occurs in stiff person syndrome. She gradually improved during postadjuvant chemotherapy with simultaneous immunotherapy including intravenous immunoglobulins and methylprednisolone, and she had recovered completely when evaluated at the 22-month follow-up visit after completion of her treatment for pleuropulmonary blastoma. Conclusion We present the first documented child with stiff person syndrome associated with pleuropulmonary blastoma. The marked clinical improvement following chemotherapy for pleuropulmonary blastoma was yet more proof of the pleuropulmonary blastoma-related stiff person syndrome. In children with a malignancy and stiff person syndrome, a paraneoplastic syndrome should be considered and the treatment for the malignancy must be undertaken.

Purpose: In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. Methods: We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, labo­ ratory testing was performed more than twice. Results: The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequ­ ency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391–2.821; P

This study aimed to investigate sensitive factors involved in left ventricular mass reduction in children with end-stage renal disease (ESRD) undergoing peritoneal dialysis.Thirty-five subjects on peritoneal dialysis were enrolled. Two successive echocardiographic and clinical data for each subject were obtained. Blood pressure and left ventricular mass index (LVMI) were indexed through a division with the normal 95The mean age was 12.9 ± 4.6 years. Predictors of left ventricular hypertrophy and its persistence were systolic blood pressure index (P = 0.019 and P = 0.046) and E' velocity (P = 0.035 and P = 0.031) in univariate analysis. However, differences in these predictors between the datasets were not related to the change in indexed LVMI. Reduction in indexed LVMI was correlated to a reduction of indexed left atrial volume (R = 0.638, P = 0.001), trans-mitral A velocity (R = 0.443, P = 0.011), and serum blood urea nitrogen level (R = 0.372, P = 0.028) and an elevation of haemoglobin level (R = -0.374, P = 0.027).The extent of circulating volume expansion is potentially the main predictive factor for change of LVMI, because the volume dependent diastolic functional variables correlate to the change of LVMI. Further study with a large number of ESRD children including a group under fluid volume control is needed to investigate the role of volume expansion on the change of LVMI.

Background: Recently, several studies to predict the unresponsiveness to initial intravenous immunoglobulin (IVIG) treatment during the acute phase of Kawasaki disease have been conducted. However, reported predictors are varied in those studies. The racial difference was assumed as the cause of such diversity. The aim of this study is to find predictors of unresponsiveness to initial IVIG in Korean children with Kawasaki disease. Subjects and methods: Subjects were 424 patients with Kawasaki disease who had been admitted to Asan Medical Center from January 2006 to December 2012. Demographic, clinical, and laboratory data were investigated through a review of medical records. Laboratory data before initial IVIG treatment and within 10 days after fever onset were selected. Forty one patients (9.7%) were non responders to initial IVIG treatment. Univariate and multivariate logistic regression analyses were performed to find predictors of unresponsiveness to initial IVIG treatment. Results: Median age was 30.0 months (range 1.6-186.0 months) in IVIG-responders, and 30.9 months (range 2.9-107.8 months) in IVIG-nonresponders (P=0.819). Number of females was 156 (40.7%) and 15 (36.6%) respectively (P=0.607). No other demographic and clinical variables were different between two groups. Univariate analyses showed that neutrophil percentage (P Conclusion: The results of this study were inconsistent with the results of previously conducted studies with the same purpose in other countries. Therefore future study with more number of subjects is needed for confirmative identification of predictors of unresponsiveness to initial IVIG treatment in patients with Kawasaki disease.

Introduction: Hemophagocytic lymphohistiocytosis(HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis and up-regulation of inflammatory cytokines. Thus, both HLH and Kawasaki disease(KD) are characterized by prolonged fever, and are diagnosed by a clinical and laboratory scoring system, concurrent manifestation of HLH and KD has been described in the literature. We describe two cases of children who diagnosed as KD initially, but after intravenous gamma globulin(IVIG) failed to produce clinical response, were found to have HLH. Case report: A 3-year-old boy who had previous KD history 5 months ago was admitted for 9day fever and skin rash. His symptoms were fulfilled KD criteria, and echocardiography showed dilated right coronary artery of 4.2mm. He was treated with 2 cycles of IVIG until fever subsided. However, 2 days later, he got fever again and cytopenia(Hb Comment: There is considerable overlap between the clinical syndromes of KD and HLH and early recognition and treatment of these two disease entity is imperative to avoid fatal outcomes in severe cases. Thus, these should both be considered and excluded in any child with unremitting fever and rash.

목적: 가와사키병이 있는 소아에서 진단 및 결과 예측에 있어서 Bacille Calmette-Guerin (BCG) 접종 부위 반응의 임상적 중요성을 알아보고자 하였다. 방법: 2006년 1월부터 2017년 2월까지 가와사키병으로 서울아산병원에 입원한 1,058명의 환자를 대상으로 각 연령대에서 BCG 부위 반응의 발생 빈도를 조사하였다. 이 중 생후 18개월 미만인 416명의 환자를 BCG 부위 반응과 실험실 및 임상 소견들과의 연관성을 분석하기 위한 대상자들로 선정하였다. 분석은 완전형과 불완전형 발현 두 그룹으로 구분하여 수행되었다. 결과: 1,058명의 환자들을 관찰한 결과, BCG 부위 반응의 발생률은 생후 6-12개월(83%) 연령의 환자에게 가장 많았고, 12개월 후 연령이 증가함에 따라 감소했다(P

Purpose The aim of this study was to investigate the statistical properties of four previously developed pediatric coronary artery z score models in healthy Korean children. Methods The study subjects were 181 healthy Korean children, whose age ranged from 1 month to 15 years. The diameter of each coronary artery was measured using 2-dimensional echocardiography and converted to the z score in the four models (McCrindle, Olivieri, Dallaire, and Japanese model). Descriptive statistical analyses and 1-sample t tests were performed. Results All calculated z scores had P values of ≥0.050 using the Kolmogorov-Smirnov test. The one sample t test showed that the mean z scores did not converge to zero except in 1 model, and the mean right coronary artery (RCA) z score was less than zero in all 4 models. The smaller RCA diameter in this study could be associated with the more distal measuring point used to avoid the conal branch. The percentage of subjects with extreme z score values (≥2.0 and ≥2.5) for the left main coronary artery (LMCA) seems to be higher in the Dallaire (4.9% and 3.3%) and Japanese models (7.1% and 3.8%). Conclusion All 4 models showed statistical feasibility of normal distribution. More precise instructions would be needed for the measurement of the RCA. The higher percentage of extreme z scores for the LMCA is compatible with the basic understanding of anatomic variation in the LMCA.