Van Albada Mirjam E, Loot Frederieke G, Fokkema Rebecca, Roofthooft Marcus T R, Berger Rolf M F
Department of Pediatrics, Division of Pediatric Cardiology, Beatrix Children's Hospital/University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands.
Pediatr Res. 2008 Mar;63(3):321-7. doi: 10.1203/PDR.0b013e318163a2e7.
Appropriate parameters are needed for the monitoring of children with pulmonary arterial hypertension (PAH). Various biologic markers seem to be of use in adults with PAH. No data are available on their value in children with PAH. In this study, the relation between serum markers, functional parameters, and hemodynamic variables in pediatric PAH and their ability to predict survival is determined. Serum N-terminal pro brain natriuretic peptide (NT-proBNP), uric acid, norepinephrine, and epinephrine were measured and correlated with invasive hemodynamics, functional parameters, and outcome in 29 pediatric patients with PAH who visited a tertiary reference center for pediatric PAH between 1997 and 2005. NT-proBNP correlated with functional class (R = 0.36; p = 0.03) and 6-min walking distance (6MWD) (R = -0.53; p < 0.001). Uric acid correlated with mean pulmonary arterial pressure, pulmonary vascular resistance, and cardiac index (R = 0.63, p = 0.01; R = 0.71, p = 0.03, and R = -0.65, p = 0.007, respectively). After initiation of treatment, NT-proBNP decreased. This decrease correlated with an increased 6MWD. Finally, norepinephrine and NT-proBNP levels were highly predictive for mortality. In this series of children with PAH, biologic markers were correlated with hemodynamics and functional capacity, as parameters of disease severity. The data indicate that these markers can be used to monitor treatment effects and predict mortality in pediatric PAH.
监测肺动脉高压（PAH）患儿需要合适的参数。各种生物标志物似乎对成人PAH有用。关于它们在儿童PAH中的价值尚无数据。在本研究中，确定了小儿PAH中血清标志物、功能参数和血流动力学变量之间的关系及其预测生存的能力。对1997年至2005年间到一家三级小儿PAH参考中心就诊的29例小儿PAH患者测量了血清N末端脑钠肽前体（NT-proBNP）、尿酸、去甲肾上腺素和肾上腺素，并将其与有创血流动力学、功能参数及预后进行关联分析。NT-proBNP与功能分级（R = 0.36；p = 0.03）和6分钟步行距离（6MWD）（R = -0.53；p < 0.001）相关。尿酸与平均肺动脉压、肺血管阻力和心脏指数相关（分别为R = 0.63，p = 0.01；R = 0.71，p = 0.03；R = -0.65，p = 0.007）。开始治疗后，NT-proBNP下降。这种下降与6MWD增加相关。最后，去甲肾上腺素和NT-proBNP水平对死亡率具有高度预测性。在这组小儿PAH患者中，生物标志物与血流动力学和功能能力相关，可作为疾病严重程度的参数。数据表明这些标志物可用于监测小儿PAH的治疗效果并预测死亡率。
