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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

Pancreatic Elastase-1 Quick Test™ proves to be a rapid and reliable option to qualitatively evaluate pancreatic function for diagnostic purposes in a clinical setting of CF care.

Since Folling's discovery of "imbecillitas phenylpyruvica" in 1934, knowledge of the nature of phenylketonuria (PKU) has broadened. In untreated patients, an accumulation of phenylalanine and its metabolites results in toxic damage of central nervous system due to an impairment of phenylalanine hydroxylase (PAH) activity. Through early diagnosis with neonatal screening, PKU might be eff ectively treated by a lowphenylalanine diet and/or tetrahydrobiopterin (sapropterin dihydrochloride, BH4) supplementation for PAH PHENYLKETONURIA IS NOT A RISK FACTOR FOR CHANGES OF INFLAMMATION STATUS AS ASSESSED BY INTERLEUKIN 6 AND INTERLEUKIN 8 CONCENTRATIONS ABSTRACTBackground. High oxidative stress and a reduced potential for free radical scavenging in phenylketonuria (PKU) patients, a phenomenon confi rmed in a few studies, may lead to systemic chronic infl ammation. The aim of this study was to compare the infl ammation status, as assessed by interleukin 6 and interleukin 8 concentrations, in patients with PKU and in healthy controls. Material and methods. Twenty patients with classical PKU, aged 18-34 years and under dietary control, were enrolled in the study. The control group comprised of 20 healthy subjects matched for age and sex. Interleukin 6 and 8 levels were measured by enzyme-linked immunosorbent assay (ELISA) kits in all study participants. Results. IL-6 concentrations in the study group ranged from 0.74 pg/ml to 1.34 pg/ml. No signifi cant differences were found between IL-6 concentration between the study group and the control group (p = 0.989). IL-8 concentrations ranged from 17.56 pg/ml to 20.87 pg/ml. The obtained results of IL-8 levels did not diff er signifi cantly between the study group and control group (p = 0.192). No signifi cant correlation was observed between Phe blood levels and IL-6 or IL-8 concentrations in the study group (ρ respectively: -0.225, 0.177). In a multivariate analysis, neither IL-6 nor IL-8 concentrations were correlated with sex, age, BMI and Phe levels. Conclusions. Phenylketonuria is not a risk factor for changes of infl ammation status as assessed by IL-6 and IL-8 concentrations.