9. Gastrointestinal/Liver Disease/Metabolic Complications of CF/Nutrition S77 303 The effect of sunshine on vitamin D levels (25-OHD) in cystic fibrosis (CF) patients - a UK experience M. Gautam 1 , R. Menon 1 , S. Huq 1 , S. Pandya 1 , J. Greenwood 1 , M.J. Walshaw 1 . 1 Liverpool Heart and Chest Hospital, Liverpool, United Kingdom Introduction: Despite routine oral supplementation, levels of 25-OHD in CF patients are often unsatisfactory. Although the aetiology is multi-factorial, reduced skin exposure to sunlight is a contributory cause but there is no consensus as to when 25-OHD levels should be estimated. We therefore compared the variation in 25-OHD levels and monthly UK sunshine in our CF-patients, and also looked at any intra-patient variability in relation to the month in which testing was performed. Methods and Results: We compared 711 25-OHD estimations taken between 2004– 2010 from 240 adult CF patients (mean age 28 years, 173 [72%] receiving vitamin D supplementation, 133 male) with average monthly sunshine data obtained from the UK Meteorological Office. A positive correlation was observed between the average 25-OHD levels and average monthly UK sunshine (2004–2010) (Pearson’s coefficient r = 0.54, P < 0.03) (Table 1). 27 patients had >5 estimations of 25-OHD during the study period: same-patient variability expressed as the inter quartile difference (IQR), dependent upon the month of testing (Table 2). Table 1. Sunshine hours/25-OHD Months Jan-Feb Mar-Apr May-Jun Jul-Aug Sept-Oct Nov-Dec Average sunshine (h/mo) 54 119 177 170 108 49 25-OHD average level (ng/ml) 16 15 19 31 21 16 Table 2. IQR difference (DIQR) of vitamin D level per month Month Jan Feb Mar Apr May Jun Jul Aug Sept Oct Nov Dec DIQR 2 14 6 8 15 13 18 20 15 6 8 18 Conclusions: Although higher 25-OHD levels were found in our patients with in- creased sunshine during the UK summer months, overall levels of 25-OHD however were low despite supplementation: the UK has less sunshine hours (average 1,476 hrs/year) than other major European countries (1,776 hrs/year). Monthly variation of the IQR difference for the same patient suggests that measures should be taken to ensure that 25-OHD levels are checked at the same time each year. 304 Vitamin K status in young children with cystic fibrosis P. Krzyzanowska 1 , A. Lisowska 1 , H. Wos 2 , M. Trawi´ nska-Bartnicka 3 , B. Lyudmyla 4 , N. Rohovyk 4 , M. Rachel 5 , J. Walkowiak 1 . 1 Poznan University of Medical Sciences, Poznan, Poland; 2 Medical University of Silesia, Katowice, Poland; 3 Cystic Fibrosis Centre, Gdansk, Poland; 4 Lviv Cystic Fibrosis Centre, Lviv, Ukraine; 5 Medical University, Rzeszow, Poland Introduction: Cystic fibrosis (CF) patients are at risk of developing vitamin K deficiency. However, there is no reliable data in the youngest age group. Therefore, we aimed to assess vitamin K status in children aged up to 3 years and to correlate it with several clinical factors. Material and Methods: The study comprised 52 CF patients. In all subjects, the concentration of the undercarboxylated prothrombin (PIVKA-II), as a marker of vitamin K deficiency, was determined. Results: PIVKA-II concentrations were pathological in 24 (46.2%) CF children, in remaining 28 (53.8%) patients vitamin K status was found to be normal. No statistical differences in clinical parameters (Z-score for body height and weight, number of hospitalizations and sweat chloride concentrations) neither in distribution of Pseudomonas aeruginosa colonization nor in pancreatic status between selected subgroups with normal and abnormal PIVKA-II concentrations were documented. Normal vitamin K status was more frequent in patients receiving proper vitamin supplementation (p < 0.0078). However, vitamin K deficiency appeared in 5 out of 21 patients receiving at least 2.5 mg vitamin K/week. In logistic regression model, no clinical parameter was proven to be a risk factor for vitamin K deficiency. Conclusion: Vitamin K deficiency is frequent in CF infants and toddlers, and may also appear in those receiving recommended supplementation. There is no strong relationship between clinical expression of the disease and vitamin K status. 305 Lung function improves with high serum levels of vitamin A in patients with cystic fibrosis C. Bouso˜ no 1 , D. Gonz´ alez 1 , F. Rivas 1 , D. Acu˜ na 2 , S. Heredia 3 , A. Sojo 4 , A. L´ azaro 5 , J.J. D´ ıaz Mart´ ın 1 . 1 Hospital Universitario Central de Asturias, Pediatrics, Oviedo, Spain; 2 Hospital Universitario Ni˜ no Jes´ us, Pediatrics, Madrid, Spain; 3 Hospital Universitario Miguel Servet, Pediatrics, Zaragoza, Spain; 4 Hospital de Cruces, Pediatrics, Bilbao, Spain; 5 Hospital Cl´ ınico Universitario de Zaragoza, Pediatrics, Zaragoza, Spain Objectives: Pancreatic insufficiency and fat and bile malabsorption predisposes patients with cystic fibrosis (CF) to fat-soluble vitamin malabsorption, despite pancreatic enzyme replacement. Several studies have associated lung injury with deficit of antioxidants. The aim of our study was to analyze the relationship between lung function and vitamin A in children and young adults with CF. Methods: Multicenter descriptive and transversal study. During 2 consecutive visits during the same year, we checked serum levels of vitamins A, D and E in relation to functional pulmonary status obtained by espirometry in 94 CF patients − 6 to 24 years old − in absence of clinical exacerbation (no cough, fever or hemoptysis). Statistical analysis: Pearson and Spearman correlation coefficients. Multiple regression Analysis. Results: The average dose of vitamin A supplementation was 3204±1223 units/ day. Serum vitamin A levels were positively correlated with FEV 1 (r = 0.250 p = 0.015) and FEV25−75 (r = 0.250 p = 0.018). After adjusting for pancreatic status (13% were pancreatic sufficient), the same associations remained: FEV (B = 0.236 p = 0.018) and FEV25−75 (B = 0.324 p = 0.024). No correlations were found with other vitamins (D and E). Conclusion: Lung function in stable CF correlates and gradually improves as higher the serum levels of vitamin A are. This relationship is independent of pancreatic and vitamin D or E status. 306* Interaction of fat-soluble vitamins with immunosuppressant drugs in lung transplanted cystic fibrosis patients T. Pincikova 1 , L. Mared 2 , L. Hjelte 1 . 1 Karolinska Institutet, Karolinska Univ Hosp Huddinge, Stockholm CF Center, Stockholm, Sweden; 2 Sk˚ ane University Hospital, Heart and Lung Center, Lund, Sweden Objectives: Recent literature suggests that metabolism of fat-soluble vitamins undergoes changes in organ transplantated patients. Cyclosporine A was found to produce elevations in serum 1,25(OH) 2 D levels in animal models and kidney transplanted patients. Vit D supplementation might decrease the need for immuno- suppression. The role of vit D in the prevention of post-transplant osteoporosis remains unclear. Methods: Patient records of 41 lung transplanted CF patients at CF centres in Lund and Stockholm were used to investigate the metabolism of fat-soluble vitamins, importance of vit D for post-transplant bone health and its interactions with immunosuppressant drugs. S-retinol and a-tocoferol levels increase after lung transplantation (p < 0.001 and P < 0.005 respectively), whereas S-25OHD and 1,25(OH) 2 D levels do not change. Despite vit D supplementation, median S-25OHD is insufficient both before and after transplantation. Whole body, spinal and femoral BMD correlate with P-calcium (p < 0.05 in all). Vit D supplementation is not associated with higher P-calcium or BMD after transplantation. However, vit D sup- plement dose correlates negatively with PTH (r = −0.88; p < 0.05). S-1,25(OH) 2 D one year after transplantation is positively correlated with cumulative dose of cyclosporine A (r = 0.93; p < 0.01). Cumulative dose of hydrocortisone needed during the first week after transplantation is inversely related to the cumulative vit D supplement dose (r = −0.52;p < 0.05). Conclusion: The observed changes in vit A and E metabolism, and the interference of vitamin D with immunosuppressant drugs may have an impact on immune modulation and bone health in lung transplanted CF patients.