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

被引:19
作者
Lai, Silvia [1 ]
Mazzaferro, Sandro [1 ]
Mitterhofer, Anna Paola [1 ]
Bonci, Enea [2 ]
Marotta, Paolo Giangreco [1 ]
Pelligra, Francesco [1 ]
Murciano, Manuel [3 ]
Celani, Camilla [4 ]
Troiani, Patrizia [3 ]
Cimino, Giuseppe [3 ]
Palange, Paolo [3 ]
机构
[1] Sapienza Univ Rome, Nephrol & Dialysis Unit, Dept Translat & Precis Med, Viale Univ 37, I-00185 Rome, Italy
[2] Sapienza Univ Rome, Dept Expt Med, Rome, Italy
[3] Sapienza Univ Rome, Dept Publ Hlth & Infect Dis, Special Unit Cyst Fibrosis, Rome, Italy
[4] Bambino Gesu Pediat Hosp, Dept Specialist Pediat, Rheumatol Unit, Rome, Italy
关键词
Cystic fibrosis; Chronic kidney disease; Cardiovascular disease; Endothelial dysfunction; MOLECULAR-MECHANISMS; DISEASE; INFLAMMATION;
D O I
10.1186/s12967-019-02139-4
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
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.
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页数:7
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共 31 条
  • [1] Comparison Among Recommendations for the Management of Arterial Hypertension Issued by Last US, Canadian, British and European Guidelines
    Antza C.
    Doundoulakis I.
    Stabouli S.
    Kotsis V.
    [J]. High Blood Pressure & Cardiovascular Prevention, 2018, 25 (1) : 9 - 16
  • [2] Bianchi S, 2016, GIORNALE ITALIANO NE, V33, P78
  • [3] Screening for Cystic Fibrosis-Related Diabetes: Matching Pathophysiology and Addressing Current Challenges
    Boudreau, Valerie
    Reynaud, Quitterie
    Dubois, Catherine Lehoux
    Coriati, Adele
    Desjardins, Katherine
    Durieu, Isabelle
    Rabasa-Lhoret, Remi
    [J]. CANADIAN JOURNAL OF DIABETES, 2016, 40 (05) : 466 - 470
  • [4] Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy
    Cantin, Andre M.
    Hartl, Dominik
    Konstan, Michael W.
    Chmiel, James F.
    [J]. JOURNAL OF CYSTIC FIBROSIS, 2015, 14 (04) : 419 - 430
  • [5] Cystic fibrosis: a clinical view
    Castellani, Carlo
    Assael, Baroukh M.
    [J]. CELLULAR AND MOLECULAR LIFE SCIENCES, 2017, 74 (01) : 129 - 140
  • [6] Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications
    Cohen-Cymberknoh, Malena
    Kerem, Eitan
    Ferkol, Thomas
    Elizur, Arnon
    [J]. THORAX, 2013, 68 (12) : 1157 - 1162
  • [7] Predictive performance of different kidney function estimation equations in lung transplant patients
    Degen, Dov A.
    Janardan, Jyotsna
    Barraclough, Katherine A.
    Schneider, Hans G.
    Barber, Thomas
    Barton, Howard
    Snell, Gregory
    Levvey, Bronwyn
    Walker, Rowan G.
    [J]. CLINICAL BIOCHEMISTRY, 2017, 50 (7-8) : 385 - 393
  • [8] Treatment of lung infection in patients with cystic fibrosis: Current and future strategies
    Doering, Gerd
    Flume, Patrick
    Heijerman, Harry
    Elborn, J. Stuart
    Angyalosi, G.
    Assael, B.
    Bell, S.
    Bilton, D.
    De Boeck, K.
    Bush, A.
    Campbell, P. W., III
    Cattaneo, A.
    Dembowsky, K.
    Drevinek, P.
    Dubois, C.
    Eichler, I.
    Elborn, J. S.
    Flume, P. A.
    Foweraker, J. E.
    Gallagher, C.
    Gartner, S.
    Geller, D. E.
    Goldman, M.
    Goss, C. H.
    Gupta, R.
    Heijerman, H. G.
    Henig, N.
    Higgins, M.
    Hjelte, L.
    Hoiby, N.
    Jongejan, R.
    Knoch, M.
    Konstan, M. W.
    Muhlebach, M. S.
    Nieuwenhuizen, P. W.
    Parkins, M. D.
    Pressler, T.
    Quittner, A. L.
    Ratjen, F.
    Ramsey, B. W.
    Smyth, A.
    Thieroff-Ekerdt, R.
    Tullis, E.
    van der Ent, C. K.
    Vazquez, C.
    Wainwright, C. E.
    [J]. JOURNAL OF CYSTIC FIBROSIS, 2012, 11 (06) : 461 - 479
  • [9] Cystic fibrosis
    Elborn, J. Stuart
    [J]. LANCET, 2016, 388 (10059) : 2519 - 2531
  • [10] Long-term effect of chronic intravenous and inhaled nephrotoxic antibiotic treatment on the renal function of patients with cystic fibrosis
    Florescu, Marius C.
    Lyden, Elizabeth
    Murphy, Peter J.
    Florescu, Diana F.
    Fillaus, Jennifer
    [J]. HEMODIALYSIS INTERNATIONAL, 2012, 16 (03) : 414 - 419