Sławomira Drzymała-Czyż1,2, Patrycja Krzyżanowska-Jankowska1, Krzysztof Dziedzic1,3, Aleksandra Lisowska1, Szymon Kurek1, Joanna Goździk-Spychalska4, Victoria Kononets5, Dagmara Woźniak2, Edyta Mądry6, Jarosław Walkowiak1. 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.
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.
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.
Authors: Aleksandra Lisowska; Andrzej Pogorzelski; Grzegorz Oracz; Katarzyna Siuda; Wojciech Skorupa; Marta Rachel; Szczepan Cofta; Tomasz Piorunek; Jarosław Walkowiak Journal: J Cyst Fibros Date: 2011-07-20 Impact factor: 5.482
Authors: Noël Peretti; Claude C Roy; Eric Drouin; Ernest Seidman; Pierre Brochu; Georges Casimir; Emile Levy Journal: Am J Physiol Gastrointest Liver Physiol Date: 2005-10-13 Impact factor: 4.052
Authors: V Terlizzi; A Tosco; R Tomaiuolo; A Sepe; N Amato; A Casale; C Mercogliano; F De Gregorio; F Improta; A Elce; G Castaldo; V Raia Journal: J Cyst Fibros Date: 2014-02-11 Impact factor: 5.482
Authors: Jaroslaw Walkowiak; Sanda Nousia-Arvanitakis; Alan Cade; Natasha Kashirskaya; Robert Piotrowski; Krystyna Strzykala; Maria Kouniou; Andrzej Pogorzelski; Dorota Sands; Nikolai Kapranov Journal: J Cyst Fibros Date: 2002-12 Impact factor: 5.482