Williams Sophia N, Nussbaum Eliezer, Yoonessi Leila, Morphew Tricia, Randhawa Inderpal
Pediatric Pulmonology/Allergy/Immunology, Miller Children's Hospital, 2801 Atlantic Avenue, Long Beach, CA, 90806, USA.
Lung. 2014 Jun;192(3):385-93. doi: 10.1007/s00408-014-9572-y. Epub 2014 Apr 4.
The pulmonary complications of sickle cell disease (SCD) are a leading cause of morbidity and mortality (MacLean et al. Am J Respir Crit Care Med 178:1055-1059, 2008; Klings et al. Am J Respir Crit Care Med 173:1264-1269, 2006; National Heart, Lung, and Blood Institute, 2009). Despite this recognition, predictive markers of lung dysfunction progression remain elusive (Klings et al. Am J Respir Crit Care Med 173:1264-1269, 2006; Platt et al. N Engl J Med 330:1639-1644, 1994; Caboot et al. Curr Opin Pediatr 20:279-287, 2008; Field et al. Am J Hematol 83:574-576, 2008; Shirlo et al. Peadiatr Respir Review 12:78-82, 2011). This study was designed describe the longitudinal progression and identify specific markers that influence bronchial disease in SCD.
A retrospective, chart review of 89 patients with SCD was conducted. All patients underwent spirometry in conjunction with body plethysmography as part of routine care. Eleven lung function variables were assessed, five of which were selected to establish patterns of normal, obstructive, restrictive, or mixed obstructive-restrictive physiology (Klings et al. Am J Respir Crit Care Med 173:1264-1269, 2006; Field et al. Am J Hematol 83:574-576, 2008).
In the unadjusted model, forced expiratory volume in one second (FEV1)% of predicted trended downward with age, while total lung capacity (TLC)% of predicted showed a bimodal distribution and carbon monoxide diffusion capacity corrected for hemoglobin (DLCOcor)% of predicted remained stable. Adjusting for acute chest syndrome (ACS) episodes, medication status, and growth velocity (GV), the final model demonstrated that the downward trend between FEV1% of predicted with age was further influenced by the latter two factors.
Initial decline in FEV1% of predicted is associated with worsening pulmonary dysfunction over time. Independent of ACS episodes, the factors most influential on the progression of FEV1% predicted include the introduction of medications as well as the promotion of adequate prepubertal growth. Efforts to ensure normal prepubertal GV and treatment with bronchodilators, such as short-acting beta(2) agonists and inhaled corticosteroids (ICS), should be considered at an early age to delay progression of pulmonary dysfunction.
镰状细胞病（SCD）的肺部并发症是发病和死亡的主要原因（MacLean等人，《美国呼吸与重症医学杂志》178:1055 - 1059，2008年；Klings等人，《美国呼吸与重症医学杂志》173:1264 - 1269，2006年；美国国立心肺血液研究所，2009年）。尽管人们已经认识到这一点，但肺功能障碍进展的预测标志物仍然难以捉摸（Klings等人，《美国呼吸与重症医学杂志》173:1264 - 1269，2006年；Platt等人，《新英格兰医学杂志》330:1639 - 1644，1994年；Caboot等人，《儿科学当前观点》20:279 - 287，2008年；Field等人，《美国血液学杂志》83:574 - 576，2008年；Shirlo等人，《儿科呼吸综述》12:78 - 82，2011年）。本研究旨在描述其纵向进展并确定影响SCD支气管疾病的特定标志物。
对89例SCD患者进行回顾性病历审查。作为常规护理的一部分，所有患者均接受了肺活量测定和体容积描记法。评估了11项肺功能变量，其中5项被选来确定正常、阻塞性、限制性或混合性阻塞 - 限制性生理模式（Klings等人，《美国呼吸与重症医学杂志》173:1264 - 1269，2006年；Field等人，《美国血液学杂志》83:574 - 576，2008年）。
在未调整模型中，预测的一秒用力呼气量（FEV1）%随年龄呈下降趋势，而预测的肺总量（TLC）%呈双峰分布，预测的血红蛋白校正一氧化碳弥散量（DLCOcor）%保持稳定。在对急性胸综合征（ACS）发作、用药状况和生长速度（GV）进行调整后，最终模型表明，预测的FEV1%与年龄之间的下降趋势还受到后两个因素的进一步影响。
预测的FEV1%的初始下降与肺功能障碍随时间恶化相关。独立于ACS发作，对预测的FEV1%进展最有影响的因素包括药物的使用以及青春期前适当生长的促进。应在早期考虑采取措施确保青春期前正常的GV，并使用支气管扩张剂进行治疗，如短效β2激动剂和吸入性糖皮质激素（ICS），以延缓肺功能障碍的进展。
