Influence of pancreatic status on circulating plasma sterols in patients with cystic fibrosis

Monica Gelzo 1 , 2 , Paola Iacotucci 3 , Concetta Sica 2 , Renato Liguori 4 , Marika Comegna 1 , 2 , Vincenzo Carnovale 3 , Antonio Dello Russo 2 , Gaetano Corso 5 , and Giuseppe Castaldo 1 , 2
  • 1 CEINGE Advanced Biotechnologies s.c.a.r.l., Naples, Italy
  • 2 Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy
  • 3 Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy
  • 4 Department of Science and Technology, University of Naples Parthenope, Naples, Italy
  • 5 Department of Clinical and Experimental Medicine, University of Foggia, Viale L. Pinto 1, 71122 Foggia, Italy
Monica Gelzo
  • CEINGE Advanced Biotechnologies s.c.a.r.l., Naples, Italy
  • Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy
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, Paola Iacotucci
  • Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy
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, Concetta Sica
  • Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy
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, Renato Liguori
  • Department of Science and Technology, University of Naples Parthenope, Naples, Italy
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, Marika Comegna
  • CEINGE Advanced Biotechnologies s.c.a.r.l., Naples, Italy
  • Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy
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, Vincenzo Carnovale
  • Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy
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, Antonio Dello Russo
  • Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy
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, Gaetano Corso
  • Corresponding author
  • Department of Clinical and Experimental Medicine, University of Foggia, Viale L. Pinto 1, 71122 Foggia, Italy
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and Giuseppe Castaldo
  • CEINGE Advanced Biotechnologies s.c.a.r.l., Naples, Italy
  • Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy
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Abstract

Background

Patients with cystic fibrosis (CF) have a reduced intestinal absorption of cholesterol and in a preliminary study we observed differences in plasma sterol profile between patients with pancreatic sufficiency (PS) and those with pancreatic insufficiency (PI). Therefore, we hypothesized that the sterol analysis may contribute to study the digestion and absorption state of lipids in patients with CF. To this aim we evaluated plasma sterols in a significant number of adult patients with CF in relation to the pancreatic status.

Methods

Beside cholesterol, we measured phytosterols and lathosterol as markers of intestinal absorption and hepatic biosynthesis, respectively, by gas-chromatography in plasma of adult CF patients with pancreatic sufficiency (PS-CF, n = 57), insufficiency (PI-CF, n = 97) and healthy subjects (control group, CT, n = 71).

Results

PI-CF patients had cholesterol and phytosterols levels significantly lower than PS-CF and CT (p < 5 × 10−10) suggesting a reduced intestinal absorption of sterols related to PI. Instead, lathosterol was significantly higher in PI-CF patients than PS-CF and CT (p < 0.0003) indicating an enhanced cholesterol biosynthesis. In PI-CF patients, phytosterols positively correlate with vitamin E (p = 0.004). Both the classes of molecules need cholesterol esterase for the intestinal digestion, thus the reduced levels of such lipids in serum from PI-CF patients may depend on a reduced enzyme activity, despite the pancreatic enzyme supplementation in all PI-CF patients.

Conclusions

A plasma sterols profile may be useful to evaluate the metabolic status of lipids in adult patients with CF and could help to manage the pancreatic enzyme supplementation therapy.

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