作者: Rifat A. Chaudry , Andrew Bush , Mark Rosenthal , Suzanne Crowley
关键词: Internal medicine 、 DLCO 、 Functional residual capacity 、 Sickle cell anemia 、 Lung 、 Vascular disease 、 Cardiology 、 Surgery 、 Medicine 、 Diffusing capacity 、 Anemia 、 Respiratory minute volume 、 Critical Care and Intensive Care Medicine 、 Pulmonary and Respiratory Medicine 、 Cardiology and Cardiovascular Medicine
摘要: 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 size bed and abnormalities SCD limit exercise capacity. Methods Fifty stable patients aged 10 to 18 years 50 healthy control subjects matched race age were recruited. Incremental ergometer cardiopulmonary testing was performed using respiratory mass spectrometry exhaled gas analysis. A rebreathing maneuver measure functional residual capacity, effective blood flow (Qpeff), DLCO, helium dilution calculate minute ventilation, oxygen consumption, CO 2 production. Results In 89 evaluable subjects, there no ventilatory differences between subjects. Qpeff consistently 15% 20% greater than at all stages, but DLCO corrected both surface area hemoglobin only 7% 10% stages. As result, DLCO/Qpeff ratio considerably lower Arteriovenous content difference one-third less Conclusions Contrary our hypothesis, failure maintain sufficient compensate anemia led limitation. The capillary volume reduced throughout, implying subtle disease; however, this not limiting exercise.