Yasuhiko Mori

Association of Uric Acid with Obesity and Endothelial Dysfunction in Children and Early Adolescents

Background: Hyperuricemia in adults is known to be associated with hypertension, the metabolic syndrome and cardiovascular disease. The purpose of this study was to elucidate the factors associated with hyperuricemia in obese children and early adolescents and to investigate the threshold serum level of uric acid (UA) for the metabolic syndrome in children. Methods: We assessed anthropometric measurements, blood pressure, body composition and biochemical data in 1,559 obese children. To assess endothelial dysfunction, flow-mediated dilatation (FMD) was measured in 92 children. The correlations between serum UA levels and various parameters were examined. The threshold serum UA level for the metabolic syndrome was calculated by receiver-operating characteristic (ROC) curve analysis. Results: Serum UA levels were positively correlated with lipids in both boys and girls, and they were inversely correlated with FMD in the boys but not the girls. The threshold serum UA level for the metabolic syndrome was 5.25 mg/dl in boys and 5.05 mg/dl in girls. However, the specificity and sensitivity of ROC curve analysis are not so striking. Conclusion: The correlation between UA and FMD showed gender differences and might be affected by the hormonal status. The cutoff level of serum UA as a marker of the metabolic syndrome in obese children was affected by both age and gender.

Repair of persistent truncus arteriosus without a conduit: sleeve resection of the pulmonary trunk from the aorta and direct right ventricle-pulmonary artery anastomosis §

OBJECTIVE Establishing a new continuity between the right ventricle and the pulmonary artery is the mainstay of repair for persistent truncus arteriosus. We used the Tran Viet-Neveux technique without a Lecomte maneuver to construct the connection without a conduit. Here, we retrospectively review the mid-term surgical results to examine the effectiveness of this approach. METHODS A cylindrical segment incorporating both pulmonary artery branches was sleeve-resected from the truncal artery. The cylindrical segment was cut in the middle and two truncal arterial flaps were combined to form the posterior floor of the new pulmonary arterial trunk. The edge of the floor was attached directly to the superior margin of an oblique incision made in the left-anterior wall of the right ventricle. A polytetrafluoroethylene monocusp was attached to the lower half margin of the right ventricular incision. A large glutaraldehyde-treated pericardial patch was used to form the anterior hood of the new right ventricular outflow tract. Both great arteries were located in a normal spiral configuration. RESULTS Ten babies (range: 3 days to 9 months of age) underwent this procedure. The Collett-Edwards classification of persistent truncus arteriosus was type I in five cases and type II in five others. There was one hospital death due to severe respiratory distress. During follow-up (36-60 months, median 54 months), only one re-operation was required to enlarge a left branch pulmonary artery stenosis. Follow-up echocardiography showed pulmonary regurgitation (mild two, moderate seven, and severe one) and mild flow acceleration in the left pulmonary artery branch and right ventricle-pulmonary artery connection in one case. CONCLUSION This simple modification for surgical correction of persistent truncus arteriosus may be an effective alternative that overcomes conduit-related problems.

Meconium Aspiration Delays Normal Decline of Pulmonary Vascular Resistance Shortly after Birth through Lung Parenchymal Injury

Background: Persistent pulmonary hypertension of the newborn is often associated with meconium aspiration syndrome (MAS) or perinatal asphyxia. Objective: To determine the effect of meconium or asphyxia on pulmonary arterial pressure and circulating levels of vasoactive substances, we conducted a prospective study of 54 term infants, including infants with meconium-stained amniotic fluid with normal (MSAF) or abnormal (MAS) chest X-ray findings, infants with perinatal asphyxia, and controls. The purpose of this study was to determine the group most likely to have elevated pulmonary arterial pressure and a disturbed balance between vasoactive substances. Methods: To estimate the pulmonary arterial pressure by echocardiography, we used the ratio of the right to left systolic ventricular pressure (RVP/LVP ratio). We measured the plasma concentrations of endothelin-1 (ET-1), cyclic guanosine monophosphate (cGMP) as an indicator of nitric oxide (NO) production, and 6-keto-prostaglandin F1α (6-keto-PGF1α) for the estimation of prostacyclin concentration. We also measured KL-6 as a marker of lung injury. Results:The RVP/LVP ratio was significantly higher in the MAS group than the other groups on day 0. Although ET-1 and 6-keto-PGF1α levels were comparable among all groups, the cGMP level on days 3–5 and the KL-6 level throughout the first postnatal week were significantly higher in the MAS group. Conclusions: It is possible that meconium aspiration delays normal decline of pulmonary vascular resistance shortly after birth through lung parenchymal injury. The subsequent increase of cGMP in MAS may be an adaptive response to prevent further elevation of pulmonary arterial pressure by inducing NO.

Persistent high fever for more than 10 days during acute phase is a risk factor for endothelial dysfunction in children with a history of Kawasaki disease

BACKGROUND Endothelial dysfunction has previously been reported in children with a history of Kawasaki disease, but the determinants of endothelial function in Kawasaki disease patients are still unknown. In this study, we investigated endothelial function in Kawasaki disease patients and attempted to identify risk factors for persistent endothelial dysfunction. METHODS Using high-resolution ultrasound, we measured the percent flow-mediated dilatation, an arterial response to reactive hyperemia, to evaluate endothelial function in 67 patients with a history of Kawasaki disease and 28 age- and sex-matched control subjects. We divided the Kawasaki disease patients into a group with impaired endothelial function (the percent flow-mediated dilatation below -2 standard deviations of the control group) and a group with normal endothelial function (the percent flow-mediated dilatation more than -2 standard deviations of control). Logistic multiple regression analysis was performed to identify independent predictors of impaired endothelial function. RESULTS In Kawasaki disease patients, the percent flow-mediated dilatation was significantly lower than in the control subjects (9.8±3.6%, compared with 13.1±3.4%, p<0.01). In 13 Kawasaki disease patients (3 patients with coronary artery lesions and 10 patients without coronary artery lesions), the percent flow-mediated dilatation was below -2 standard deviations of control. Logistic multiple regression analysis showed that a febrile period of longer than 10 days during the acute phase was the significant risk factor for endothelial dysfunction (odds ratio: 8.562; 95% confidence interval: 1.366-53.68). Presence of coronary artery lesions was not a determinant of endothelial dysfunction. CONCLUSIONS Systemic endothelial dysfunction exists in children with a history of Kawasaki disease, and a febrile period of longer than 10 days during the acute phase is an independent predictor of endothelial dysfunction irrespective of coronary artery involvement.

Contents Vol. 99, 2011

S. Andersson, Helsinki E. Bancalari, Miami, Fla. J. Bhatia, Augusta, Ga. G. Buonocore, Siena W. Carlo, Birmingham, Ala. I. Choonara, Derby T. Curstedt, Stockholm C. Dani, Florence B. Darlow, Christchurch M. Fujimura, Osaka M. Hallman, Oulu W.W. Hay, Jr., Aurora, Colo. S.E. Juul, Seattle, Wash. M. Kaplan, Jerusalem B. Kramer, Maastricht R.J. Martin, Cleveland, Ohio C.J. Morley, Cambridge J. Neu, Gainesville, Fla. P.C. Ng, Hong Kong M.W. Obladen, Berlin A.G.S. Philip, Sebastopol, Calif. M. Post, Toronto E. Saliba, Tours O.D. Saugstad, Oslo M.S. Schimmel, Jerusalem B. Schmidt, Philadelphia, Pa. M.P. Sherman, Columbia, Mo. E.S. Shinwell, Rehovot K. Simmer, Perth, W.A. J. Smith, Tygerberg B. Sun, Shanghai N. Vain, Buenos Aires F. van Bel, Utrecht J.N. van den Anker, Washington, D.C. M. Vento Torres, Valencia M. Weindling, Liverpool J.A. Widness, Iowa City, Iowa Fetal and Neonatal Research

A Case of Kawasaki Disease with Re-dilatation of the Coronary Artery Five Years after the Onset

We experienced a case of Kawasaki disease with re-dilatation of coronary artery aneurysm five years after the onset. [Patient profile] The patient is a 12-year-old boy. He suffered from Kawasaki disease at the age of four years. Intravenous gamma-globulin (IVGG) therapy with 300 mg/kg/day was administered for 5 days, and additional IVGG with 1g/kg/day was administered furthermore. However, in spite of IVGG therapy, the aneurysms were developed on the both coronary arteries, which were confirmed by the cardiac catheterization one year later. (AHA Committee Report: Segment 2: two aneurysms; 4.8mm, 5.0mm in diameter, Segment 6: 4,3mm, Segment 11: 4.1mm). Thereafter, the coronary aneurysm on LAD regressed gradually. We depicted this regression of LAD aneurysm by the echocardiography at 4 years after the onset. (Segment 6: 2.4mm in diameter). However, further follow-up echocardiography revealed re-dilatation of the aneurysm on the same region of LAD one year later. (Segment 6: 7.4mm in diameter). We also confirmed this re-dilatation of the aneurysm without stenotic lesions by cardiac catheterization. [Discussion] There have been reported that the regressed aneurysms sometimes develop to the stenotic lesions late after the onset. However, there are a few reports in which a newly developed aneurysm emerged late after the onset of Kawasaki disease. [Conclusions] Re-dilatation of the regressed aneurysm is a very unique clinical course as a long-term Kawasaki disease. While the mechanism of the re-dilatation of this patient is still un-known, we must be careful to follow him up.