Background: The interest in cystic fibrosis (CF) dyslipidaemia as a potential risk factor for cardiovascular disease is increasing with patients' survival. This study aimed to investigate CF dyslipidaemia, its clinical correlates and links to oxidized low-density lipoprotein (oxLDL), adiponectin, and apolipoprotein E (APOE). Methods: This cross-sectional study assessed clinical characteristics of CF, as well as the serum lipid profile, oxLDL, adiponectin, and APOE. Results: In total, 108 CF subjects were enrolled in this study, with a median age of 22 years, BMI of 20.5 kg/m 2 , FEV1% of 61%, of which 81% were pancreatic insufficient (PI). Healthy subjects (HS; n = 51) were in similar age. Hypocholesterolaemia occurred in 31% of CF subjects and in no HS. Hypertriglyceridaemia concerned 21% of patients (HS: 8%, p = .04), and low HDL-C 45% (HS: 6%, p b .0001). At least one of these three CF dyslipidaemia disturbances was present in 62% of CF subjects, but there were no significant differences in oxLDL, oxLDL/LDL-C ratio, adiponectin, and APOE between CF and HS groups. PI was independently associated with low total cholesterol, LDL-C, and non-high density lipoprotein cholesterol, with age and sex also modifying lipid levels. In CF (n = 42), triglycerides did not correlate with serum tumour necrosis factor α (TNF-α). Conclusions: CF dyslipidaemia is highly prevalent and heterogenous. The lipid profile weakly associates with the clinical characteristics of CF as well as oxLDL, adiponectin, and APOE. Further research is needed, especially regarding HDL function in CF, the causes of hypertriglyceridaemia, and the value of essential fatty acid supplementation for CF dyslipidaemia.

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PurposeAs life expectancy in cystic fibrosis (CF) increases, questions regarding its potential impact on cardiovascular health arise. Soluble vascular cell adhesion molecule 1 (sVCAM-1), P-selectin (sP-selectin) are proposed as biomarkers of cardiovascular disease. We aimed to: compare their concentrations in clinically stable CF patients and healthy subjects (HS) and verify whether they independently correlate with CF characteristics.MethodsSerum sVCAM-1 and sP-selectin levels were measured using ELISA. CF was characterized using: forced expiratory volume in 1 s, exocrine pancreatic and CF-related liver disease status, Pseudomonas aeruginosa colonization, serum high-sensitivity C-reactive protein, and body mass index (BMI). CFTR genotypes were classified as severe (classes I and II) or other.Results108 CF patients and 51 healthy subjects volunteered for the study. In the CF group BMI was lower (median [IQR]: 20.5 kg/m2 [18.4–22.2] vs. 21.6 kg/m2 [19.9–23.4], p = 0.02) and hsCRP levels were higher (3.6 mg/L [1.1–7.1] vs. 0.5 mg/dL [0.3–1.0], p < 10−10). While sVCAM-1 concentrations were greater in CF patients (1018 ng/mL [851–1279] vs. 861 ng/mL [806–979], p < 10−4), sP-selectin levels did not differ (155 ng/mL [129–188] vs. 156 ng/mL [144–177], p = 0.48). None of the multivariable regression models was valid for the prediction of sVCAM-1 and sP-selectin in CF.ConclusionsWe found higher sVCAM-1 concentrations in CF patients than in healthy subjects, which were not explained by CF characteristics. Further research is required to check whether sVCAM-1 is a marker of microangiopathy in CF.

Introduction: Cystic fibrosis (CF) involves chronic inflammation and decreased pulmonary function, which increase caloric demand. Yet, sufficient energy provision is hindered by reduced appetite and fat malabsorption. Brain-derived neurotrophic factor (BDNF), leptin, and neuropeptide Y (NPY) belong to energy balance-regulating factors. We aimed to assess their concentrations in CF patients in order to search for potential clinical correlates. Material and methods: This was an exploratory, cross-sectional study. Patients' weight and height Z-scores, forced expiratory volume in 1 s (FEV 1 %), exocrine pancreatic status (fecal elastase-1), genotypes, and other characteristics were assessed. Serum concentrations of BDNF, leptin, NPY, IL-6, and TNF-α were measured using ELISA. Results: The study enrolled 56 patients, of whom 29 (52%) were female and 17 (30%) were younger than 16 years. Median (1 st-3 rd quartile) mass Z-score was-0.85 (-1.56-(-0.36)); median FEV 1 was 70.5% (45.0-89.5); 48 (86%) patients had exocrine pancreatic insufficiency and 8 (14%) diabetes. Overall, median concentrations were: BDNF: 33.91 ng/ml (26.40-40.43), leptin: 12.05 ng/ml (8.93-17.77), NPY: 2.86 ng/ml (1.75-4.42). None of these factors correlated with mass Z-score, FEV 1 %, IL-6 or TNF-α. Leptin and NPY correlated negatively (ρ =-0.62, p = 3 × 10-7); BDNF/NPY ratio was associated with leptin (ρ = 0.54, p = 2 × 10-5), BDNF/leptin ratio correlated with NPY (ρ = 0.60, p = 1 × 10-6). In a multivariable regression analysis NPY was weakly, but independently, associated with FEV 1 %, and leptin with age. Conclusions: BDNF and leptin were not associated with weight Z-score or FEV 1 %. Serum NPY concentrations seemed to be lower in CF patients with reduced pulmonary function independently of malnutrition and inflammation.

Background. Routine administration of vitamin A, recommended in CF patients, can help to prevent its deficiency. However, high vitamin A supplementation may lead to its excessive level and possible toxicity. Therefore, the aim of the present study was to assess the status of vitamin A and the determinants of its body resources in CF patients. Material and methods. In 196 CF patients aged from 4 months to 47 years, the following parameters were analysed: nutritional status (standardized body weight and height, serum albumin concentration) and clinical expression of disease (lung function − spirometry; biochemical markers of liver function − ALT, AST, GGT; respiratory tract colonization by Pseudomonas aeruginosa; diabetes; cirrhosis, non-cirrhotic liver disease; exocrine pancreatic function − fecal elastase-1 concentration; blood clotting -INR and vitamin A supplementation). Results. Median vitamin A concentration in the study group was 383.0 ng/ml (1

Background: Butyric acid’s effectiveness has not yet been assessed in the pediatric inflammatory bowel disease (IBD) population. This study aimed to evaluate the effectiveness of oral sodium butyrate as an add-on to standard therapy in children and adolescents with newly diagnosed IBD. Methods: This was a prospective, randomized, placebo-controlled multicenter study. Patients aged 6–18 years with colonic Crohn’s disease or ulcerative colitis, who received standard therapy depending on the disease’s severity, were randomized to receive 150 mg sodium butyrate twice a day (group A) or placebo (group B). The primary outcome was the difference in disease activity and fecal calprotectin concentration between the two study groups measured at 12 weeks of the study. Results: In total, 72 patients with initially active disease completed the study, 29 patients in group A and 43 in group B. At week 12 of the study, the majority of patients achieved remission. No difference in remission rate or median disease activity was found between the two groups (p = 0.37 and 0.31, respectively). None of the patients reported adverse events. Conclusions: A 12-week supplementation with sodium butyrate, as adjunctive therapy, did not show efficacy in newly diagnosed children and adolescents with IBD.

Background/Aims: Cystic fibrosis (CF) liver disease is the third most frequent cause of death in CF patients. Although it alters fatty acid (FA) metabolism, data concerning the profile of FA in CF patients with liver cirrhosis is lacking. This study aimed to assess the FA composition of serum phospholipids in CF patients with and without liver cirrhosis. Methods: The study comprised 25 CF patients with liver cirrhosis and 25 without it. We assessed Z-scores for body height and weight, lung function, exocrine pancreatic sufficiency and colonization with Pseudomonas aeruginosa. FAs' profile of serum glycerophospholipids was quantified by gas chromatography mass spectrometry. Results: In CF patients with liver cirrhosis, the levels of C16:0 were higher and the amounts of C20:2n-6, C20:3n-6, C20:4n-6, and all the n-3 polyunsaturated FAs (PUFAs) (C18:3n-3, C20:5n-3, C22:5n-3, C22:6n-3) were lower than those in CF subjects without liver cirrhosis. The n-6/n-3, C20:4n-6/C18:2n-6, total n-6/C18:2n-6, C20:5n-3/C18:3n-3 and total n-3/C18:3n-3 ratios did not differ between the 2 groups. Conclusions: Liver cirrhosis may associate with profound abnormalities in the composition of serum glycerophospholipids FAs in CF patients. None of the analyzed clinical factors could explain the greater prevalence of low levels of PUFAs in this CF subgroup.

Aim of the study: to evaluate the immune response rate in children with inflammatory bowel disease (IBD) who received the full hepatitis B vaccination course in infancy. we also evaluated rates of response to booster doses. Material and methods: participants were 1-to 18-year-old children with IBD, who received 3 doses of the hepatitis B vaccine in infancy. the study subjects were on no immunosuppressive therapy, on immunomodulators, on biological therapy, or received combo therapy. anti-hepatitis B surface antibody (anti-hBs) level ≥ 10 mIu/ml was considered to be seroprotective. patients with anti-hBs level < 10 mIu/ml received 1 or 3 doses of hepatitis B vaccine, and their post-vaccination anti-hBs levels were evaluated. Results: In total, we included 157 subjects, with a median age of 14.5 years. anti-hBs levels ≥ 10 miu/ml were found in 84/157 (53.5%) patients and were not associated with age (p = 0.3), sex (p = 0.7), or IBD type (p = 0.9). there was no significant difference in the rate of seroconversion between IBD patients treated with no immunosuppressive drugs, immunomodulators, biologicals, and combo therapy (30.4% vs. 39.3% vs. 2.7% vs. 7.1%, respectively, p = 0.3). after the first and third dose of booster vaccine, anti-hBs levels ≥ 10 mIu/ml were as follows: 92% and 100%, respectively. Conclusions: the immune response in children with IBD, who received the full series of hepatitis B vaccinations in infancy was inadequate and did not depend on the type of therapy. the booster dose(s) of vaccine could help to protect this group of patients from hepatitis B virus.