The Impact of Sickle Cell Disease on Exercise Capacity in Children

The Impact of Sickle Cell Disease on Exercise Capacity in Children
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DOI:
10.1378/chest.12-0611
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发表时间:
2013-02-01
期刊:
影响因子:
9.6
通讯作者:
Crowley, Suzanne
Crowley, Suzanne
中科院分区:
医学1区
文献类型:
--
作者:
Chaudry, Rifat A.;Bush, Andrew;Crowley, Suzanne

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背景:对镰状细胞病(SCD)患儿的肺血管并发症知之甚少。我们假设,转移因子(肺弥散量的一氧化碳[DLCO])可作为替代的肺血管床的大小和肺血管异常的儿童SCD可能会限制运动capability.Methods:50个稳定的SCD患者年龄在10至18岁和50名健康对照组相匹配的种族和年龄被招募。使用呼吸质谱法进行递增式测力计心肺运动试验,用于呼出气体分析。一个再呼吸动作被用来测量功能残气量,有效肺血流量(Qpeff),DLCO,和氦稀释被用来计算每分钟通气量,耗氧量,和CO2 production.Results:在89个可评估的主题,有SCD和对照组之间没有明显的差异。在所有阶段,SCD受试者的Qpeff始终比对照受试者高15%至20%,但在所有阶段,SCD受试者的表面积和血红蛋白校正DLCO仅高约7%至10%。因此,在SCD的所有阶段,DLCO/Qpeff比都相当低。动静脉氧含量差约三分之一,在SCD在所有stages.Conclusions:与我们的假设相反,未能保持足够的Qpeff,以弥补贫血导致运动限制。肺毛细血管血容量与血流量的比率始终降低,这意味着轻微的肺血管疾病;然而,这不是限制运动的因素。胸部2013; 143(2):478-484
Background: Little is known about pulmonary vascular complications in children with sickle cell disease (SCD). We hypothesized that transfer factor (diffusing capacity of the lung for carbon monoxide [DLCO]) may be used as a surrogate for the size of the pulmonary vascular bed and that pulmonary vascular abnormalities in children with SCD may limit exercise capacity.Methods: Fifty stable patients with SCD aged 10 to 18 years and 50 healthy control subjects matched for race and age were recruited. Incremental ergometer cardiopulmonary exercise testing was performed using respiratory mass spectrometry for exhaled gas analysis. A rebreathing maneuver was used to measure functional residual capacity, effective pulmonary blood flow (Qpeff), and DLCO, and helium dilution was used to calculate minute ventilation, oxygen consumption, and CO2 production.Results: In the 89 evaluable subjects, there were no ventilatory differences between SCD and control subjects. Qpeff was consistently 15% to 20% greater in subjects with SCD than control subjects at all stages, but DLCO corrected for both surface area and hemoglobin was only about 7% to 10% greater in subjects with SCD at all stages. As a result, the DLCO/Qpeff ratio was considerably lower in SCD at all stages. Arteriovenous oxygen content difference was about one-third less in SCD at all stages.Conclusions: Contrary to our hypothesis, failure to maintain a sufficient Qpeff to compensate for anemia led to exercise limitation. The ratio of pulmonary capillary blood volume to flow is reduced throughout, implying subtle pulmonary vascular disease; however, this was not a factor limiting exercise. CHEST 2013; 143(2):478-484