The University of North Carolina at Chapel Hill School of Medicine, 130 Mason Farm Road, Campus Box 7020, Chapel Hill, NC 27599, USA.
The University of North Carolina at Chapel Hill School of Medicine, 130 Mason Farm Road, Campus Box 7020, Chapel Hill, NC 27599, USA.
J Cyst Fibros. 2023 Nov;22(6):1024-1026. doi: 10.1016/j.jcf.2023.10.002. Epub 2023 Oct 12.
Risk of cardiovascular disease (CVD) may be changing in people with cystic fibrosis (pwCF) with widespread use of highly effective modulator therapy (HEMT). We performed a retrospective analysis of patients who had lipids checked before and after initiation of ivacaftor or elexacaftor/tezacaftor/ivacaftor. We hypothesized that HEMT negatively impacts lipids (total cholesterol [TC], low-density lipoprotein [LDL], high-density lipoprotein [HDL], TC/HDL ratio). 41 adult patients were included. Paired t-tests showed statistically significant increases in TC (mean difference 16.3 mg/dL, p = 0.007, n = 40), LDL (mean difference 17.1 mg/dL, p < 0.001, n = 35), and TC/HDL ratio (mean difference 0.40, p = 0.014, n = 39) after HEMT initiation. HDL was unchanged (mean difference -1.5 mg/dL, p = 0.69, n = 39). Linear mixed models showed CF liver disease was associated with significantly blunted changes in TC and LDL. Family history of CVD risk factors was associated with significantly accentuated increases in TC and LDL. These data suggest a role for more lipid screening in pwCF.
心血管疾病（CVD）的风险可能会在广泛使用高效调节剂治疗（HEMT）的囊性纤维化（pwCF）患者中发生变化。我们对使用 ivacaftor 或 elexacaftor/tezacaftor/ivacaftor 治疗前和治疗后检查过血脂的患者进行了回顾性分析。我们假设 HEMT 会对血脂（总胆固醇[TC]、低密度脂蛋白[LDL]、高密度脂蛋白[HDL]、TC/HDL 比值）产生负面影响。共纳入 41 例成年患者。配对 t 检验显示 TC（平均差异 16.3mg/dL，p=0.007，n=40）、LDL（平均差异 17.1mg/dL，p<0.001，n=35）和 TC/HDL 比值（平均差异 0.40，p=0.014，n=39）在 HEMT 开始后有统计学意义的升高。HDL 没有变化（平均差异-1.5mg/dL，p=0.69，n=39）。线性混合模型显示 CF 肝病与 TC 和 LDL 变化明显减弱有关。CVD 危险因素家族史与 TC 和 LDL 的显著增加有关。这些数据表明，pwCF 需要更多的血脂筛查。
