Ataga Kenneth I, Sood Namita, De Gent Guy, Kelly Eileen, Henderson Ashley G, Jones Susan, Strayhorn Dell, Lail Alice, Lieff Susan, Orringer Eugene P
Department of Medicine, University of North Carolina, Chapel Hill, North Carolina, USA.
Am J Med. 2004 Nov 1;117(9):665-9. doi: 10.1016/j.amjmed.2004.03.034.
Recurrent vaso-occlusive episodes lead to progressive end-organ damage in patients with sickle cell disease. We sought to determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease and to identify factors associated with this life-threatening complication.
Sixty patients (> or =18 years of age; mean [+/- SD] age, 37 +/- 13 years) followed at a University Medical Center were evaluated. They were selected by a systematic sampling of patients presenting to the clinic for routine follow-up visits. All enrolled subjects underwent a clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests during a single visit. Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram.
The prevalence of pulmonary hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 mm Hg), and 2 had severe pulmonary hypertension (> or =75 mm Hg). In a logistic regression model, both lower fetal hemoglobin level and lower systolic blood pressure were associated with the presence of pulmonary hypertension.
We found that the prevalence of pulmonary hypertension in adult patients with sickle cell disease was substantial, particularly in those with lower levels of fetal hemoglobin and lower systolic blood pressure.
复发性血管闭塞发作会导致镰状细胞病患者出现进行性终末器官损害。我们试图确定成年镰状细胞病患者中肺动脉高压的患病率，并确定与这种危及生命的并发症相关的因素。
对在大学医学中心接受随访的60例患者（年龄≥18岁；平均年龄[±标准差]为37±13岁）进行了评估。他们是通过对到诊所进行常规随访的患者进行系统抽样选取的。所有入选受试者在一次就诊期间均接受了临床检查、多普勒超声心动图检查、肺功能测试和血液学检查。使用年龄和体重指数调整后的列线图定义肺动脉高压。
肺动脉高压的患病率为30%（18/60）。10例患者患有轻度肺动脉高压（高达44 mmHg），5例患有中度肺动脉高压（45至74 mmHg），2例患有重度肺动脉高压（≥75 mmHg）。在逻辑回归模型中，较低的胎儿血红蛋白水平和较低的收缩压均与肺动脉高压的存在相关。
我们发现成年镰状细胞病患者中肺动脉高压的患病率很高，尤其是在胎儿血红蛋白水平较低和收缩压较低的患者中。
