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Volume 11
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Article

Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis

by
Sławomira Drzymała-Czyż
1,2,*,
Patrycja Krzyżanowska-Jankowska
1,
Krzysztof Dziedzic
1,3,
Aleksandra Lisowska
1,
Szymon Kurek
1,
Joanna Goździk-Spychalska
4,
Victoria Kononets
5,
Dagmara Woźniak
2,
Edyta Mądry
6 and
Jarosław Walkowiak
1
1
Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, 60-572 Poznań, Poland
2
Department of Bromatology, Poznan University of Medical Sciences, 60-354 Poznań, Poland
3
Department of Food Science and Nutrition, Institute of Food Technology of Plant Origin, Poznań University of Life Sciences, 60-637 Poznań, Poland
4
Department of Pulmonology, Allergology and Respiratory Oncology, Poznan University of Medical Sciences, 60-569 Poznań, Poland
5
Department of Natural Sciences Disciplines, West Kazakhstan Marat Ospanov Medical University, Aktobe 030012, Kazakhstan
6
Department of Physiology, Poznan University of Medical Sciences, 61-781 Poznań, Poland
*
Author to whom correspondence should be addressed.
Biomolecules 2021, 11(2), 313; https://doi.org/10.3390/biom11020313
Submission received: 5 January 2021 / Revised: 13 February 2021 / Accepted: 16 February 2021 / Published: 19 February 2021
(This article belongs to the Section Molecular Genetics)
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Abstract

Background: Several factors could lead to lipid disturbances observed in cystic fibrosis (CF). This study aimed to assess sterol homeostasis in CF and define potential exogenous and endogenous determinants of lipid dysregulation. Methods: The study involved 55 CF patients and 45 healthy subjects (HS). Sterol concentrations (μg/dL) were measured by gas chromatography/mass spectrometry. CF was characterised by lung function, pancreatic status, liver disease and diabetes coexistence, Pseudomonas aeruginosa colonisation and BMI. CFTR genotypes were classified as severe or other. Results: Campesterol and β-sitosterol concentrations were lower (p = 0.0028 and p < 0.0001, respectively) and lathosterol levels (reflecting endogenous cholesterol biosynthesis) were higher (p = 0.0016) in CF patients than in HS. Campesterol and β-sitosterol concentrations were lower in patients with a severe CFTR genotype, pancreatic insufficiency and lower pancreatic enzyme dose (lipase units/gram of fat). In multiple regression analyses, β-sitosterol and campesterol concentrations were predicted by genotype and pancreatic insufficiency, whereas cholesterol and its fractions were predicted by phytosterol concentrations, age, dose of pancreatic enzymes, nutritional status and genotype. Conclusions: Independent determinants of lipid status suggest that malabsorption and pancreatic enzyme supplementation play a significant role in sterol abnormalities. The measurement of campesterol and β-sitosterol concentrations in CF patients may serve for the assessment of the effectiveness of pancreatic enzyme replacement therapy and/or compliance, but further research is required.
Keywords: campesterol; β-sitosterol; stigmasterol; cholesterol; lathosterol campesterol; β-sitosterol; stigmasterol; cholesterol; lathosterol

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MDPI and ACS Style

Drzymała-Czyż, S.; Krzyżanowska-Jankowska, P.; Dziedzic, K.; Lisowska, A.; Kurek, S.; Goździk-Spychalska, J.; Kononets, V.; Woźniak, D.; Mądry, E.; Walkowiak, J. Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis. Biomolecules 2021, 11, 313. https://doi.org/10.3390/biom11020313

AMA Style

Drzymała-Czyż S, Krzyżanowska-Jankowska P, Dziedzic K, Lisowska A, Kurek S, Goździk-Spychalska J, Kononets V, Woźniak D, Mądry E, Walkowiak J. Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis. Biomolecules. 2021; 11(2):313. https://doi.org/10.3390/biom11020313

Chicago/Turabian Style

Drzymała-Czyż, Sławomira, Patrycja Krzyżanowska-Jankowska, Krzysztof Dziedzic, Aleksandra Lisowska, Szymon Kurek, Joanna Goździk-Spychalska, Victoria Kononets, Dagmara Woźniak, Edyta Mądry, and Jarosław Walkowiak. 2021. "Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis" Biomolecules 11, no. 2: 313. https://doi.org/10.3390/biom11020313

APA Style

Drzymała-Czyż, S., Krzyżanowska-Jankowska, P., Dziedzic, K., Lisowska, A., Kurek, S., Goździk-Spychalska, J., Kononets, V., Woźniak, D., Mądry, E., & Walkowiak, J. (2021). Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis. Biomolecules, 11(2), 313. https://doi.org/10.3390/biom11020313

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