Poznan University of Medical Sciences, Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan, Poland.
Poznan University of Medical Sciences, Department of Pediatric Pneumonology, Allergology and Clinical Immunology, Poznan, Poland.
J Cyst Fibros. 2019 Jul;18(4):566-571. doi: 10.1016/j.jcf.2019.04.001. Epub 2019 Apr 10.
The interest in cystic fibrosis (CF) dyslipidaemia as a potential risk factor for cardiovascular disease is increasing with patients' survival. This study aimed to investigate CF dyslipidaemia, its clinical correlates and links to oxidized low-density lipoprotein (oxLDL), adiponectin, and apolipoprotein E (APOE).
This cross-sectional study assessed clinical characteristics of CF, as well as the serum lipid profile, oxLDL, adiponectin, and APOE.
In total, 108 CF subjects were enrolled in this study, with a median age of 22 years, BMI of 20.5 kg/m, FEV1% of 61%, of which 81% were pancreatic insufficient (PI). Healthy subjects (HS; n = 51) were in similar age. Hypocholesterolaemia occurred in 31% of CF subjects and in no HS. Hypertriglyceridaemia concerned 21% of patients (HS: 8%, p = .04), and low HDL-C 45% (HS: 6%, p < .0001). At least one of these three CF dyslipidaemia disturbances was present in 62% of CF subjects, but there were no significant differences in oxLDL, oxLDL/LDL-C ratio, adiponectin, and APOE between CF and HS groups. PI was independently associated with low total cholesterol, LDL-C, and non-high density lipoprotein cholesterol, with age and sex also modifying lipid levels. In CF (n = 42), triglycerides did not correlate with serum tumour necrosis factor α (TNF-α).
CF dyslipidaemia is highly prevalent and heterogenous. The lipid profile weakly associates with the clinical characteristics of CF as well as oxLDL, adiponectin, and APOE. Further research is needed, especially regarding HDL function in CF, the causes of hypertriglyceridaemia, and the value of essential fatty acid supplementation for CF dyslipidaemia.
随着患者生存时间的延长，囊性纤维化（CF）血脂异常作为心血管疾病潜在风险因素的重要性日益增加。本研究旨在探讨 CF 血脂异常及其临床相关性，并探讨其与氧化型低密度脂蛋白（oxLDL）、脂联素和载脂蛋白 E（APOE）的关系。
本横断面研究评估了 CF 的临床特征，以及血脂谱、oxLDL、脂联素和 APOE。
共纳入 108 例 CF 患者，中位年龄 22 岁，BMI 为 20.5kg/m，FEV1%为 61%，其中 81%为胰腺功能不全（PI）。健康对照者（HS；n=51）年龄相仿。CF 患者中 31%存在低胆固醇血症，而 HS 中则无。CF 患者中 21%存在高甘油三酯血症（HS：8%，p=0.04），45%存在低高密度脂蛋白胆固醇血症（HS：6%，p<0.0001）。CF 患者中至少存在上述三种血脂异常中的一种者占 62%，但 CF 和 HS 组间 oxLDL、oxLDL/LDL-C 比值、脂联素和 APOE 无显著差异。PI 与总胆固醇、LDL-C 和非高密度脂蛋白胆固醇降低独立相关，年龄和性别也可影响血脂水平。在 CF 患者（n=42）中，甘油三酯与血清肿瘤坏死因子-α（TNF-α）无关。
CF 血脂异常十分常见且具有异质性。血脂谱与 CF 的临床特征及 oxLDL、脂联素和 APOE 相关较弱。还需要进一步研究，尤其是关于 CF 中 HDL 功能、高甘油三酯血症的原因以及必需脂肪酸补充对 CF 血脂异常的价值。
