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Observational Study
. 2019 Nov 25;17(1):388.
doi: 10.1186/s12967-019-02139-4.

Renal involvement and metabolic alterations in adults patients affected by cystic fibrosis

Silvia Lai  1 , Sandro Mazzaferro  2 , Anna Paola Mitterhofer  2 , Enea Bonci  3 , Paolo Giangreco Marotta  2 , Francesco Pelligra  2 , Manuel Murciano  4 , Camilla Celani  5 , Patrizia Troiani  4 , Giuseppe Cimino  4 , Paolo Palange  4
Affiliations
Observational Study

Renal involvement and metabolic alterations in adults patients affected by cystic fibrosis

Silvia Lai et al. J Transl Med. .

Abstract

Background: 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.

Aim of the study: To evaluate the incidence, the manifestations of renal disease and the possible association with metabolic and endothelial dysfunction markers in the CF population.

Materials and methods: We performed a cross-sectional, observational study on 226 CF patients. Clinical and laboratory instrumental parameters (metabolic, inflammatory and endothelial dysfunction markers) were evaluated.

Results: 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.

Conclusions: 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.

Keywords: Cardiovascular disease; Chronic kidney disease; Cystic fibrosis; Endothelial dysfunction.

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Conflict of interest statement

The authors report no conflicts of interest. All authors must disclose any financial and personal relationships with other people or organisations that could inappropriately influence (bias) their work. The manuscript is not under consideration for publication elsewhere.

Figures

Fig. 1
Fig. 1
eGFR report and age in transplanted and non-transplanted patients. eGFR estimated Glomerular Filtration Rate
Fig. 2
Fig. 2
Relationship triglycerides and eGFR in transplanted and non-transplanted patients. eGFR estimated Glomerular Filtration Rate
See this image and copyright information in PMC

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