Kanj N, Shamseddine A, Gharzeddine W, Kanj M, Nasr T A, Koussa S, Jibrail J, Taher A
American University of Beirut Medical Center, Lebanon.
Eur J Haematol. 2000 Jun;64(6):396-400. doi: 10.1034/j.1600-0609.2000.90106.x.
We studied 36 patients (17 males and 19 females) with thalassemia major by performing pulmonary function testing (PFT), arterial blood gas analysis (ABG), as well as determining the serum ferritin level. In addition, 19 of these patients were transfused with two units of packed cells, and a repeat ABG and PFT were performed. Twenty-three patients had normal PFTs, eleven patients (30.6%) showed a restrictive pattern (significant decrease in both TLC and DLCO), and only two patients (5.6%) showed an obstructive pattern. A significant negative correlation was found between serum ferritin and restrictive parameters, DLCO and TLC (p = 0.01 and p = 0.03, respectively). This correlation was even stronger after transfusion. Controlling for age, ferritin was still negatively correlated with DLCO (p = 0.04), but no longer with TLC. There was no correlation between age and DLCO or age and TLC; however, there was a statistically significant negative correlation between age and FVC (p = 0.003). Analysis of patients who were transfused revealed a significant decrease in forced vital capacity (89 +/- 4% vs. 74 +/- 5% of predicted; p-value < 0.001) and in maximum midexpiratory flow rate (79 +/- 4% vs. 67 +/- 5% of predicted; p-value = 0.004). For patients older than 15 yr of age, there was a statistically significant decrease in FEV1/FVC (84 +/- 2 vs. 83 +/- 2%; p-value = 0.04). The ABGs showed no significant change post-transfusion. In this study, PFT findings in thalassemia major were restrictive and correlated with serum ferritin level. Transfusion had an acute effect on the obstructive parameters of PFT.
我们对36例重型地中海贫血患者（17例男性和19例女性）进行了肺功能测试（PFT）、动脉血气分析（ABG），并测定了血清铁蛋白水平。此外，其中19例患者输注了两单位的浓缩红细胞，并再次进行了ABG和PFT。23例患者的PFT结果正常，11例患者（30.6%）表现为限制性模式（TLC和DLCO均显著降低），只有2例患者（5.6%）表现为阻塞性模式。血清铁蛋白与限制性参数DLCO和TLC之间存在显著的负相关（分别为p = 0.01和p = 0.03）。输血后这种相关性更强。在控制年龄后，铁蛋白仍与DLCO呈负相关（p = 0.04），但与TLC不再相关。年龄与DLCO或年龄与TLC之间无相关性；然而，年龄与FVC之间存在统计学上显著的负相关（p = 0.003）。对输血患者的分析显示，用力肺活量显著降低（预测值的89±4% vs. 74±5%；p值<0.001），最大呼气中期流速也显著降低（预测值的79±4% vs. 67±5%；p值 = 0.004）。对于年龄大于15岁的患者，FEV1/FVC有统计学上显著的降低（84±2 vs. 83±2%；p值 = 0.04）。ABG结果显示输血后无显著变化。在本研究中，重型地中海贫血患者的PFT结果为限制性，且与血清铁蛋白水平相关。输血对PFT的阻塞性参数有急性影响。
