Abnormal pulmonary function in adults with sickle cell anemia
Abnormal pulmonary function in adults with sickle cell anemia
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DOI：
10.1164/rccm.200601-125oc
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发表时间：
2006-06-01
影响因子：
24.7
通讯作者：
Steinberg, Martin H.
中科院分区：
文献类型：
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作者：
Klings, Elizabeth S.;Wyszynski, Diego F.;Steinberg, Martin H.
理由：镰状细胞性贫血 (Hb-SS) 的肺部并发症通常会导致发病，但针对该人群肺功能测试 (PFT) 的大型研究却鲜有报道。目的：分析 310 名 Hb-SS 成人的 PFT（肺量测定、肺容量和一氧化碳扩散能力 [DLCO]），以确定肺功能障碍的模式及其与镰状细胞病其他全身并发症的关联。方法：将原始 PFT 数据与预测值进行比较。每个受试者被分为五组之一：阻塞性生理学、限制性生理学、混合阻塞性/限制性生理学、孤立性低 DLCO 或正常。通过多元线性回归评估 DLCO 降低或生理限制性患者的实验室数据与正常受试者的实验室数据之间的关联。测量和主要结果：310 名患者中，仅 31 名患者 (10%) 存在正常 PFT。总体而言，患有 Hb-SS 的成年人的特点是总肺活量（预测值 70.2 +/- 14.7%）和 DLco（64.5 +/- 19.9%）下降。最常见的 PFT 模式是限制性生理 (74%) 和孤立的低 DLCO (13%)。 DLco 降低与血小板增多（p = 0.05）、肝功能障碍（丙氨酸氨基转移酶升高；p = 0.07）以及肾功能障碍趋势（血尿素氮和肌酐升高；分别为 p = 0.05 和 0.07）相关。结论：90% 的 Hb-SS 成年患者肺功能异常。常见异常包括限制性生理和 DLCO 减少。 DLCO 降低可能表明更严重的镰状血管病，其特征是肝肾功能受损。
Rationale: Pulmonary complications of sickle cell anemia (Hb-SS) commonly cause morbidity, yet few large studies of pulmonary function tests (PFTs) in this population have been reported. Objectives: PFTs (spirometry, lung volumes, and diffusion capacity for carbon monoxide [DLCO]) from 310 adults with Hb-SS were analyzed to determine the pattern of pulmonary dysfunction and their association with other systemic complications of sickle cell disease.Methods: Raw PFT data were compared with predicted values. Each subject was subclassified into one of five groups: obstructive physiology, restrictive physiology, mixed obstructive/restrictive physiology, isolated low DLCO, or normal. The association between laboratory data of patients with decreased DLCO or restrictive physiology and those of normal subjects was assessed by multivariate linear regression.Measurements and Main Results: Normal PFTs were present in only 31 of 310 (10%) patients. Overall, adults with Hb-SS were characterized by decreased total lung capacities (70.2 +/- 14.7% predicted) and DLco (64.5 +/- 19.9%). The most common PFT patterns were restrictive physiology (74%) and isolated low DLCO (13%). Decreased DLco was associated with thrombocytosis (p = 0.05), with hepatic dysfunction (elevated alanine aminotransferase; p = 0.07), and a trend toward renal dysfunction (elevated blood urea nitrogen and creatinine; p = 0.05 and 0.07, respectively).Conclusions: Pulmonary function is abnormal in 90% of adult patients with Hb-SS. Common abnormalities include restrictive physiology and decreased DLCO. Decreased DLCO may indicate more severe sickle vasculopathy characterized by impaired hepatic and renal function.