Vitamin K Status Based on K1, MK-4, MK-7, and Undercarboxylated Prothrombin Levels in Adolescent and Adult Patients with Cystic Fibrosis: A Cross-Sectional Study.

The available evidence on vitamin K status in cystic fibrosis (CF) is scarce, lacking data on vitamin K2 (menaquinones-MK). Therefore, we assessed vitamin K1, MK-4 and MK-7 concentrations (LC-MS/MS) in 63 pancreatic insufficient and modulator naïve CF patients, and compared to 61 healthy subjects (HS). Vitamin K1 levels did not differ between studied groups.

The Impact of Infection in Adult Cystic Fibrosis Patients-A Single Polish Centre Study.

Background: (PA) is one of the most predominant pathogens of lung infections, often causing exacerbations in adult patients with cystic fibrosis (CF).

Materials And Methods: Microbiological characterization of 74 PA isolates and to evaluate the correlations between the bacterial features and 44 adult Polish CF cohort clinical parameters.

Results: The most common variant in the CF transmembrane conductance regulator () gene was F508del (76.

Nontuberculous Mycobacterial Lung Disease in the Patients with Cystic Fibrosis-A Challenging Diagnostic Problem.

Background: Cystic fibrosis (CF) is an autosomal, recessive genetic disorder, caused by a mutation in the cystic fibrosis transmembrane conductance receptor regulator (CFTR) gene. Dysregulated mucous production, and decreased bronchial mucociliary clearance, results in increased susceptibility to bacterial and fungal infections. Recently, nontuberculous mycobacteria (NTM) infections were identified as an emerging clinical problem in CF patients.

Fat-Soluble Vitamins in Standard vs. Liposomal Form Enriched with Vitamin K2 in Cystic Fibrosis: A Randomized Multi-Center Trial.

Background: We aimed to assess a liposomal fat-soluble vitamin formulation containing vitamin K2 with standard treatment in cystic fibrosis (CF).

Methods: A multi-center randomized controlled trial was carried out in 100 pancreatic-insufficient patients with CF. The liposomal formulation contained vitamin A as retinyl palmitate (2667 IU daily) and beta-carotene (1333 IU), D3 (4000 IU), E (150 IU), K1 (2 mg), and K2 as menaquinone-7 (400 µg).

Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial.

Fat-soluble vitamin deficiency remains a challenge in cystic fibrosis (CF), chronic pancreatitis, and biliary atresia. Liposomes and cyclodextrins can enhance their bioavailability, thus this multi-center randomized placebo-controlled trial compared three-month supplementation of fat-soluble vitamins in the form of liposomes or cyclodextrins to medium-chain triglycerides (MCT) in pancreatic-insufficient CF patients. The daily doses were as follows: 2000 IU of retinyl palmitate, 4000 IU of vitamin D3, 200 IU of RRR-α-tocopherol, and 200 µg of vitamin K2 as menaquinone-7, with vitamin E given in soybean oil instead of liposomes.

Nebulisation therapy in patients with cystic fibrosis - consensus of the Polish Cystic Fibrosis Society.

Introduction: Nebulisation therapy plays a key role in the treatment of cystic fibrosis (CF). Its effectiveness depends on obtaining a high concentration of drugs in the respiratory tract. Particle deposition is determined by many factors resulting, inter alia, from the essence of the lung disease (mucus, structural changes such as bronchiectasis, fibrous changes, cirrhosis) and the quality of the aerosol and breathing techniques during the procedure.

Cystic fibrosis dyslipidaemia: A cross-sectional study.

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.

Vitamin E status and its determinants in patients with cystic fibrosis.

Purpose: The risk of vitamin E deficiency is of primary concern in cystic fibrosis patients. However, early diagnosis and routine vitamin E supplementation can lead to its normal or even high levels. In the present study, we assessed vitamin E status in a large group of cystic fibrosis patients.

Vitamin A status and its determinants in patients with cystic fibrosis.

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.

Increased Soluble VCAM-1 and Normal P-Selectin in Cystic Fibrosis: a Cross-Sectional Study.

Purpose: As 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.

Vitamin K status in cystic fibrosis patients with liver cirrhosis.

The available data on the influence of liver cirrhosis on vitamin K status in CF patients is scarce. Therefore, the aims of the present study were to assess the prevalence of vitamin K deficiency in cirrhotic CF subjects and to determine whether it correlates with liver cirrhosis. The study group comprised of 27 CF patients with and 63 without liver cirrhosis.

Fungal infection of cystic fibrosis patients - single center experience.

Introduction: Cystic fibrosis (CF) is the most common monogenetic autosomal recessive disease in the human population. This systemic disease is characterized by changes in multiple organs, mainly in the lung tissue and digestive tract. More than 59% of CF patients become sensitized to fungal spores, mostly Aspergillus fumigatus.

[Nutritional status of adults with cystic fibrosis - current methods of assessment].

Cystic fibrosis (CF) is one of the most frequent monogenic disease in the Caucasian population, inherited in an autosomal recessive pattern. This is a multiple organ disease and its main manifestations include pulmonary and gastrointestinal dysfunction. The exocrine pancreatic deficiency results in impaired digestion and absorption what may lead to malnutrition and vitamins and minerals deficiencies.

Exogenous and endogenous determinants of vitamin K status in cystic fibrosis.

Cystic fibrosis (CF) patients are at high risk for vitamin K deficiency. The effects of vitamin K supplementation are very ambiguous. Therefore, we aimed to define the determinants of vitamin K deficiency in a large cohort of supplemented - 146 (86.

[Mycobacterial lung disease in patients with cystic fibrosis--report of three cases].

Mycobacterial lung disease is caused by nontuberculous mycobacteria (NTM), also known as atypical mycobacteria. NTM are widely distributed in the environment, particularly in soil and water. Although generally of low pathogenicity to humans, NTM can affect patients with underlying chronic lung diseases, such as cystic fibrosis, bronchiectasis, pneumoconiosis, or healed tuberculosis.

[Interstitial pneumonia connected with rituximab therapy--case report].

Rituximab (RTX) is a monoclonal antibody against the CD20 antigen found on the surface of B cells. RTX causes cell lysis and is therefore used to treat lymphomas, leukaemias, transplant rejection and certain autoimmune disorders. Pulmonary adverse events associated with RTX have been reported in literature.

[Analysis of the frequency of isolation and drug resistance of microorganisms isolated from the airways of adult CF patients treated in the Institute of Tuberculosis and Lung Disease during 2008-2011].

Introduction: Cystic fibrosis (CF) is the most common genetic autosomal recessive genetic disease. The most serious symptoms are observed in the lungs. Recurrent respiratory infections are the main causes of the hospitalizations and deaths of cystic fibrosis patients.

Oral antibiotic therapy improves fat absorption in cystic fibrosis patients with small intestine bacterial overgrowth.

Background: The aim of the present study was to assess the influence of antibiotic therapy on fat assimilation in cystic fibrosis (CF) patients with small intestine bacterial overgrowth (SIBO).

Materials And Methods: Twenty six pancreatic insufficient CF patients with bronchopulmonary exacerbation and diagnosed SIBO (positive hydrogen-methane breath test) entered the study. (13)C mixed triglyceride breath test was performed before and after antibiotic therapy.

Antibiotic therapy and fat digestion and absorption in cystic fibrosis.

Antibiotic therapy in the cystic fibrosis (CF) mouse model has been shown to result in reduced bacterial load of the intestine and significant body mass gain. The effect was suggested to be linked to the improvement of intestinal digestion and absorption. Therefore, we aimed to assess the influence of routinely applied antibiotic therapy in CF patients on fat assimilation.

[Vitamin K deficiency in patients with CF despite supplementation].

Introduction: Cystic fibrosis (CF) patients are at high risk for vitamin K deficiency. Vitamin K supplementation dose has not been clearly defined, and the effects of the supplementation are very ambiguous. Therefore, the aim of the present study was to assess body resources of vitamin K and determine the suitability of the coagulation parameters in the assessment of vitamin K deficiency in patients undergoing supplementation.

[Exogenous lipid pneumonia--a report of four cases].

Exogenous lipoid pneumonia (ELP) is a rare interstitial pulmonary disease caused by aspiration of the oily material. It is known to mimic many pulmonary diseases and causes many diagnostic difficulties. We present four patients with ELP.

[Pregnancy in cystic fibrosis patients--investigational study].

Aim: The purpose of this study was to investigate the consequences of pregnancy on women with CF and to clarify the impact of the disease on newborn health.

Methods: It was a retrospective study; a postal questionnaire was sent to 12 woman having a registered pregnancy in National Cystic Fibrosis Registry between 1984 and 2004 (2 to 23 years after delivery).

Results: There was a 100% response rate.