Department of Translational and Precision Medicine, Nephrology and Dialysis Unit, "Sapienza" University of Rome, Viale dell'Università 37, 00185, Rome, Italy.
Department of Experimental Medicine, "Sapienza" University of Rome, Rome, Italy.
J Transl Med. 2019 Nov 25;17(1):388. doi: 10.1186/s12967-019-02139-4.
Cystic fibrosis (CF) is one of the most frequent genetic diseases and the median survival of these patients has improved in the last few decades, therefore it becomes necessary to evaluate the long-term complications as renal and cardiovascular risk factors.
To evaluate the incidence, the manifestations of renal disease and the possible association with metabolic and endothelial dysfunction markers in the CF population.
We performed a cross-sectional, observational study on 226 CF patients. Clinical and laboratory instrumental parameters (metabolic, inflammatory and endothelial dysfunction markers) were evaluated.
We showed 65 patients with chronic kidney disease (CKD) and 158 patients with a reduced value of forced expiratory volume in 1 s (FEV1), of which 58 patients with a severe reduction of FEV1. Moreover 28 patients had undergone lung transplantation and them had a significant lower estimated Glomerular Filtration Rate (eGFR) with respect to the non-transplanted patients (p < 0.001). We reported also a significant association between lower eGFR value and serum triglycerides, total cholesterol and low-density lipoproteins (LDL) (p = 0.005, p < 0.001, p = 0.040; respectively), with a significant negative correlation between eGFR and serum triglycerides (r = - 0.28; p < 0.01). Moreover we found a significant association between lower eGFR value and serum uric acid (SUA) (p = 0.005), while we did not found an association with 25-hydroxy-vitamin-D value, serum glucose and hemoglobin A1c levels.
Our study showed a high prevalence of CKD in CF patients. Moreover we showed an increase of endothelial dysfunction and metabolic indexes in patients with reduced renal function, as SUA, serum triglycerides and LDL, suggesting the need for an early and complete screening of the main metabolic indexes to reduce cardiovascular risk and progression of renal damage, in particular in patients with lung transplant.
囊性纤维化（CF）是最常见的遗传疾病之一，在过去几十年中，这些患者的中位生存期有所提高，因此有必要评估肾脏和心血管危险因素等长期并发症。
评估囊性纤维化人群中肾病的发病率、表现以及与代谢和内皮功能障碍标志物的可能关联。
我们对 226 名 CF 患者进行了横断面、观察性研究。评估了临床和实验室仪器参数（代谢、炎症和内皮功能障碍标志物）。
我们发现 65 名患者患有慢性肾脏病（CKD），158 名患者的 1 秒用力呼气量（FEV1）值降低，其中 58 名患者的 FEV1 值严重降低。此外，28 名患者接受了肺移植，与未接受移植的患者相比，他们的估算肾小球滤过率（eGFR）显著降低（p<0.001）。我们还报告了较低的 eGFR 值与血清甘油三酯、总胆固醇和低密度脂蛋白（LDL）之间存在显著相关性（p=0.005，p<0.001，p=0.040；分别），eGFR 与血清甘油三酯之间存在显著负相关（r=-0.28；p<0.01）。此外，我们发现较低的 eGFR 值与血清尿酸（SUA）之间存在显著相关性（p=0.005），而与 25-羟维生素 D 值、血清葡萄糖和血红蛋白 A1c 水平之间没有相关性。
我们的研究表明，CF 患者的 CKD 患病率很高。此外，我们发现肾功能降低的患者内皮功能障碍和代谢指标增加，如 SUA、血清甘油三酯和 LDL，这表明需要早期和全面筛查主要代谢指标，以降低心血管风险和肾脏损害的进展，特别是在接受肺移植的患者中。
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