RESUMO
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. METHODS: Serum 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. RESULTS: 108 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. CONCLUSIONS: We 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.
Assuntos
Fibrose Cística/sangue , Selectina-P/sangue , Molécula 1 de Adesão de Célula Vascular/sangue , Adulto , Biomarcadores/sangue , Estudos de Casos e Controles , Estudos Transversais , Fibrose Cística/diagnóstico , Fibrose Cística/microbiologia , Fibrose Cística/fisiopatologia , Ensaio de Imunoadsorção Enzimática , Insuficiência Pancreática Exócrina/sangue , Insuficiência Pancreática Exócrina/diagnóstico , Feminino , Volume Expiratório Forçado , Humanos , Modelos Lineares , Pulmão/microbiologia , Pulmão/fisiopatologia , Masculino , Análise Multivariada , Polônia , Valor Preditivo dos Testes , Prognóstico , Infecções por Pseudomonas/sangue , Infecções por Pseudomonas/diagnóstico , Infecções por Pseudomonas/microbiologia , Pseudomonas aeruginosa/isolamento & purificação , Infecções Respiratórias/sangue , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/microbiologia , Regulação para Cima , Adulto JovemRESUMO
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. 5-15% of CF patients develop allergic bronchopulmonary aspergillosis. The aim of the study was to analyse the occurrence of yeast and filamentous fungi of the respiratory infections in CF patients and evaluation of drug resistance. MATERIAL AND METHODS: Between 2006 and 2014, mycological evaluation of 42 patients hospitalized at the National Institute of Tuberculosis and Lung Diseases was carried out. RESULTS: 217 specimens from pulmonary tract were collected from 42 patients with cystic fibrosis. 205 (68%) strains of yeast and 96 (32%) filamentous fungi strains were cultured. The most common mould strain was A. fumigatus - 22,2% (67 species). All isolates of filamentous fungi were in vitro 100% susceptible to itraconazole, voriconazole, posaconazole and amphotericin B. CONCLUSIONS: A. fumigatus and C. albicans were the most common etiological agents of fungal respiratory pathogens associated with CF patients. A. fumigatus strains were in vitro 100% susceptible to azole and amphotericin B. Two strains of C. albicans and one strain of C. tropicalis were non-susceptible to azole (fluconazole, itraconazole and voriconazole). Scedosporium apiospermum was resistant to amphotericin B (MIC > 32 mg/l) and susceptible to voriconazole (MIC 0.094 mg/l).
Assuntos
Fibrose Cística/complicações , Fibrose Cística/microbiologia , Fungos/isolamento & purificação , Micoses/complicações , Micoses/microbiologia , Leveduras/isolamento & purificação , Anfotericina B/farmacologia , Antifúngicos/farmacologia , Aspergilose Broncopulmonar Alérgica/microbiologia , Aspergillus fumigatus/efeitos dos fármacos , Aspergillus fumigatus/isolamento & purificação , Aspergillus fumigatus/ultraestrutura , Candida albicans/efeitos dos fármacos , Candida albicans/isolamento & purificação , Candida tropicalis/efeitos dos fármacos , Candida tropicalis/isolamento & purificação , Farmacorresistência Fúngica Múltipla , Fungos/efeitos dos fármacos , Fungos/ultraestrutura , Humanos , Itraconazol/farmacologia , Testes de Sensibilidade Microbiana , Scedosporium/efeitos dos fármacos , Scedosporium/isolamento & purificação , Triazóis/farmacologia , Voriconazol/farmacologia , Leveduras/efeitos dos fármacosRESUMO
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. The life expectancy of cystic fibrosis patients has been increasing over the past years, so there is a need to verify usefulness of existing or create new methods of nutritional status assessment. The aim of this paper was presentation current data on the methods of assessment and monitoring of nutritional status. Particular attention has been paid to appropriate nutritional support in prevention and treatment of malnutrition patients with cystic fibrosis. On the basis of recent literature we can conclude that the advanced nutritional status assessment is recommended in patient with CF by using anthropometrical methods, body composition analysis and biochemical data. Good nutritional status is connected with pulmonary functions, quality and life length.
Assuntos
Fibrose Cística/complicações , Fibrose Cística/fisiopatologia , Desnutrição/etiologia , Desnutrição/prevenção & controle , Estado Nutricional , Adulto , HumanosRESUMO
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. Some patients with cystic fibrosis (CF) have disease progression due to NTM, others can have NTM cultured intermittently from respiratory specimens without a significant decline in lung function. Identifying which patients will worsen from NTM and therefore need treatment remains difficult because of the similarity of symptoms in CF and NTM lung disease. The most common species of NTM isolated in CF patients are Mycobacterium avium complex (MAC) and Mycobacterium abscessus. In this paper, we present three different cases of mycobacterial lung disease in patients with cystic fibrosis.
Assuntos
Fibrose Cística/complicações , Fibrose Cística/microbiologia , Infecções por Mycobacterium não Tuberculosas/microbiologia , Adulto , Antibacterianos/uso terapêutico , Fibrose Cística/tratamento farmacológico , Progressão da Doença , Humanos , Masculino , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Micobactérias não Tuberculosas/isolamento & purificaçãoRESUMO
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. MK-4 concentrations were higher (median <1st-3rd quartile>: 0.778 <0.589-1.086> vs. 0.349 <0.256-0.469>, p < 0.0001) and MK-7 levels lower (0.150 <0.094-0.259> vs. 0.231 <0.191-0.315>, p = 0.0007) in CF patients than in HS. MK-7 concentrations were higher in CF patients receiving K1 and MK-7 supplementation than in those receiving vitamin K1 alone or no supplementation. Moreover, vitamin K1 concentrations depended on the supplementation regime. Based on multivariate logistic regression analysis, we have found that MK-7 supplementation dose has been the only predictive factor for MK-7 levels. In conclusion, vitamin K1 levels in CF are low if not currently supplemented. MK-4 concentrations in CF patients supplemented with large doses of vitamin K1 are higher than in HS. MK-7 levels in CF subjects not receiving MK-7 supplementation, with no regard to vitamin K1 supplementation, are low. There do not seem to be any good clinical predictive factors for vitamin K status.
Assuntos
Fibrose Cística , Protrombina , Vitamina K 1 , Vitamina K 2 , Humanos , Fibrose Cística/sangue , Feminino , Masculino , Vitamina K 2/sangue , Vitamina K 2/análogos & derivados , Estudos Transversais , Protrombina/análise , Adolescente , Adulto , Vitamina K 1/administração & dosagem , Vitamina K 1/sangue , Adulto Jovem , Estado Nutricional , Suplementos Nutricionais , Deficiência de Vitamina K/sangue , Vitamina K/sangueRESUMO
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. We describe a patient with follicular lymphoma treated with R-CHOP scheme (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) who had pulmonary symptoms during chemotherapy which were diagnosed as rituximab-induced interstitial pneumonia. Symptoms and radiological changes resolved with prednisone therapy.
Assuntos
Anticorpos Monoclonais Murinos/efeitos adversos , Doenças Pulmonares Intersticiais/induzido quimicamente , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Linfoma Folicular/tratamento farmacológico , Anticorpos Monoclonais Murinos/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Feminino , Humanos , Pessoa de Meia-Idade , Radiografia , Fatores de Risco , RituximabRESUMO
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. Pathogens that commonly infect the airways of adult CF patients include Staphylococcus aureus and Pseudomonas aeruginosa. The aim of this study was to analyse the microorganisms cultured from the airways of adult CF patients and to test the antimicrobial resistance of the most frequently isolated bacteria. MATERIAL AND METHODS: In this study, 1422 isolates of 89 CF patients were collected during a 4-year period. The microorganisms were cultured and identified according to standard microbiological procedures. Identification and drug susceptibility were performed in an automatic system Phoenix (BD), Vitek2Compact (bioMérieux), and disk-diffusion method by Kirby-Bauer. RESULTS: Among the 1422 strains the most frequent pathogens were Pseudomonas aeruginosa (55.6%) and Staphylococcus aureus (37.8%). A total of 482 (61.0%) strains of 790 isolates of P. aeruginosa, were identified as P. aeruginosa mucoid phenotype. The isolates with mucoid phenotypes were more susceptible to antibiotics than non-mucoid. Eighty-six strains of S. aureus showed resistance to methicillin (MRSA), which accounted for 16.0% of all strains of S. aureus. CONCLUSIONS: The analysis of microbiological materials from adult CF patients treated in IGiChP allowed the determination of the prevalence of potentially pathogenic microorganisms. The data obtained are consistent with the literature.
Assuntos
Fibrose Cística/tratamento farmacológico , Fibrose Cística/microbiologia , Resistência a Meticilina , Staphylococcus aureus Resistente à Meticilina/isolamento & purificação , Pseudomonas aeruginosa/isolamento & purificação , Centros Médicos Acadêmicos , Academias e Institutos , Adulto , Idoso , Fibrose Cística/epidemiologia , Feminino , Nível de Saúde , Humanos , Pacientes Internados/estatística & dados numéricos , Masculino , Staphylococcus aureus Resistente à Meticilina/patogenicidade , Pessoa de Meia-Idade , Polônia/epidemiologia , Pseudomonas aeruginosa/patogenicidade , Estudos RetrospectivosRESUMO
BACKGROUND: Pseudomonas aeruginosa (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 (CFTR) gene was F508del (76.3%), followed by 3849+10kbC>T (26.3%). A total of 39.4% of the PA isolates showed multiple resistances. In patients with parameters pointing to a decline in lung function, there was a statistically significant moderate correlation with ß-lactam resistance and a weak correlation between hospital frequency and colistin resistance. The mucoidity did not correlate with the biofilm formation ability, which showed 41.9% of the isolates. Proteolytic activity, observed in 60.8% of the clinical isolates, was weakly associated with motility detected in 78.4% of the strains. The genetic profiles of the PA were highly heterogeneous, and a weak positive correlation was established between cluster group and biofilm formation. CONCLUSION: The findings suggest that there is a high variety in P. aeruginosa populations in adult CF patients. There is a need to monitor PA strains in groups of patients with cystic fibrosis, in particular, in terms of the occurrence of antibiotic resistance related to a decline in lung function.
RESUMO
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. AIM: The aim of the present study was to assess the frequency of NTM isolations in CF patients hospitalized in the pulmonary department, serving as a hospital CF center, and to describe challenges concerning the recognition of NTMLD (nontuberculous mycobacterial lung disease) in those patients. METHODS: Consecutive CF patients, who were hospitalized due to pulmonary exacerbations (PEX), in a single CF center, between 2010 and 2020, were retrospectively assessed for the presence of NTM in respiratory specimens. Clinical and radiological data were retrospectively reviewed. RESULTS: Positive respiratory specimen cultures for NTM were obtained in 11 out of 151 patients (7%), mean age-35.7 years, mean BMI-20.2 kg/m2, mean FEV1-58.6% pred. Cultures and phenotyping revealed the presence of Mycobacterium avium (M. avium)-in six patients, Mycobacterium chimaera (M. chimaera) in two, Mycobacterium kansasii (M. kansasii)-in one, Mycobacterium abscessus (M. abscessus)-in one, Mycobacterium lentifavum (M. lentiflavum)-in one. Simultaneously, respiratory cultures were positive for fungi in 91% of patients: Candida albicans (C. albicans)-in 82%, Aspergillus fumigatus (A. fumigatus)-in 45%. Clinical signs of NTMLD were non-specific, chest CT indicated NTMLD in five patients only. CONCLUSION: Due to non-specific clinical presentation, frequent sputum cultures for NTM and analysis of serial chest CT examinations are crucial for NTMLD recognition in CF patients. Further studies concerning the predictive role of fungal pathogens for NTMLD development in CF patients are needed.
RESUMO
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). It was compared with the standard vitamin preparations in the closest possible doses (2500 IU, 1428 IU, 4000 IU, 150 IU, 2.14 mg, respectively; no vitamin K2) over 3 months. RESULTS: Forty-two patients finished the trial in the liposomal and 49 in the control group (overall 91 pts: 22.6 ± 7.6 years, 62.6% female, BMI 19.9 ± 2.8 kg/m2, FEV1% 70% ± 30%). The main outcome was the change of vitamin status in the serum during the study (liposomal vs. standard): all-trans-retinol (+1.48 ± 95.9 vs. -43.1 ± 121.4 ng/mL, p = 0.054), 25-hydroxyvitamin D3 (+9.7 ± 13.4 vs. +2.0 ± 9.8 ng/mL, p = 0.004), α-tocopherol (+1.5 ± 2.5 vs. -0.2 ± 1.6 µg/mL, p < 0.001), %undercarboxylated osteocalcin (-17.2 ± 24.8% vs. -8.3 ± 18.5%, p = 0.061). The secondary outcome was the vitamin status at the trial end: all-trans-retinol (370.0 ± 116.5 vs. 323.1 ± 100.6 ng/mL, p = 0.045), 25-hydroxyvitamin D3 (43.2 ± 16.6 vs. 32.7 ± 11.5 ng/mL, p < 0.001), α-tocopherol (9.0 ± 3.1 vs. 7.7 ± 3.0 µg/mL, p = 0.037), %undercarboxylated osteocalcin (13.0 ± 11.2% vs. 22.7 ± 22.0%, p = 0.008). CONCLUSION: The liposomal fat-soluble vitamin supplement containing vitamin K2 was superior to the standard form in delivering vitamin D3 and E in pancreatic-insufficient patients with CF. The supplement was also more effective in strengthening vitamin K-dependent carboxylation, and could improve vitamin A status.
RESUMO
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. AIM OF THE GUIDELINES: A large variety of available drugs that can be used in the form of aerosols (bronchodilators, mucolytics, antibiotics), a wide range of devices for their delivery, and a different approach to the practical aspect related to the use of inhalation, makes it necessary to systematize knowledge in order to optimize nebulisation therapy. The paper presents an overview of inhaled drugs used in cystic fibrosis and their administration devices. RESULTS: The principles of inhalation antibiotic therapy, which constitute the basis for the treatment of primary and chronic respiratory tract infections of Pseudomonas aeruginosa etiology, are discussed in detail. A very important issue was raised related to the proper selection of devices and their proper operation. In the context of the key role of nebulisation therapy in cystic fibrosis, a huge problem is the limited availability of inhaled antibiotics in Poland. CONCLUSIONS: The possibility of choosing an antibiotic and using alternating therapy increases the effectiveness of inhalation treatment, which results in slowing down the progress of bronchopulmonary disease and extending the life of patients.
RESUMO
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. All participants received 4 mg of ß-carotene and 1.07 mg of vitamin K1 to ensure compliance with the guidelines. The primary outcome was the change from the baseline of all-trans-retinol and 25-hydroxyvitamin D3 concentrations and the percentage of undercarboxylated osteocalcin. Out of 75 randomized patients (n = 28 liposomes, n = 22 cyclodextrins, and n = 25 MCT), 67 completed the trial (89%; n = 26 liposomes, n = 18 cyclodextrins, and n = 23 MCT) and had a median age of 22 years (IQR 19-28), body mass index of 20.6 kg/m2 [18.4-22.0], and forced expiratory volume in 1 s of 65% (44-84%). The liposomal formulation of vitamin A was associated with the improved evolution of serum all-trans-retinol compared to the control (median +1.7 ng/mL (IQR -44.3-86.1) vs. -38.8 ng/mL (-71.2-6.8), p = 0.028). Cyclodextrins enhanced the bioavailability of vitamin D3 (+9.0 ng/mL (1.0-17.0) vs. +3.0 ng/mL (-4.0-7.0), p = 0.012) and vitamin E (+4.34 µg/mL (0.33-6.52) vs. -0.34 µg/mL (-1.71-2.15), p = 0.010). Liposomes may augment the bioavailability of vitamin A and cyclodextrins may strengthen the supplementation of vitamins D3 and E relative to MCT in pancreatic-insufficient CF but further studies are required to assess liposomal vitamin E (German Clinical Trial Register number DRKS00014295, funded from EU and Norsa Pharma).
Assuntos
Ciclodextrinas/química , Fibrose Cística/dietoterapia , Lipossomos/química , Triglicerídeos/química , Vitaminas/administração & dosagem , Adolescente , Adulto , Calcifediol/sangue , Colecalciferol/administração & dosagem , Colecalciferol/sangue , Suplementos Nutricionais , Insuficiência Pancreática Exócrina/dietoterapia , Feminino , Humanos , Masculino , Resultado do Tratamento , Vitamina A/administração & dosagem , Vitamina A/sangue , Vitamina D/administração & dosagem , Vitamina D/sangue , Vitamina E/administração & dosagem , Vitamina E/sangue , Vitamina K 2/administração & dosagem , Vitamina K 2/análogos & derivados , Vitaminas/sangue , Vitaminas/química , Adulto Jovem , beta Caroteno/administração & dosagemRESUMO
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 22â¯years, BMI of 20.5â¯kg/m2, FEV1% of 61%, of which 81% were pancreatic insufficient (PI). Healthy subjects (HS; nâ¯=â¯51) were in similar age. Hypocholesterolaemia occurred in 31% of CF subjects and in no HS. Hypertriglyceridaemia concerned 21% of patients (HS: 8%, pâ¯=â¯.04), and low HDL-C 45% (HS: 6%, pâ¯<â¯.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 =â¯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.
Assuntos
Fibrose Cística/complicações , Dislipidemias/etiologia , Adiponectina/sangue , Adulto , Apolipoproteínas E/sangue , Correlação de Dados , Estudos Transversais , Fibrose Cística/sangue , Dislipidemias/sangue , Feminino , Humanos , Lipoproteínas LDL/sangue , Masculino , Adulto JovemAssuntos
Pneumopatias/diagnóstico , Doenças Pleurais/diagnóstico , Pneumologia/tendências , Humanos , Pneumopatias/epidemiologia , Pneumopatias/terapia , Publicações Periódicas como Assunto , Doenças Pleurais/epidemiologia , Doenças Pleurais/terapia , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Pesquisa , Projetos de Pesquisa , Tuberculose PulmonarRESUMO
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. Moreover, we also aimed to establish determinants of its body resources in cystic fibrosis patients. MATERIAL AND METHODS: The study group comprised 211 cystic fibrosis patients aged from 1 month to 48 years. In all of them serum α-tocopherol concentration was analyzed using high-performance liquid chromatography. RESULTS: Median vitamin E concentration was 9.9⯵g/ml (1st-3rd quartile: 7.5-13.5). Vitamin E deficiency was found in 17 (8.0%) and high levels were documented in 24 (11.4%) participants. Patients with and without vitamin E deficiency did not differ significantly with respect to age, standardized body weight and height, FEV1, albumin concentration and vitamin E supplementation dose. However, vitamin E deficiency appeared more frequently in participants without vitamin E supplementation. Moreover, in multiple linear regression analysis pancreatic insufficiency, severe CFTR gene mutation and vitamin E dose, were potentially defined as determinants of vitamin E concentration. CONCLUSIONS: Vitamin E deficiency in cystic fibrosis patients is rather rare nowadays. Excessive vitamin E levels seem to be more frequent. Vitamin E status wasn't documented to be strictly related to clinical determinants. Beyond vitamin E supplementation, exocrine pancreatic function and CFTR gene mutations may have had an impact on the vitamin E body resources in cystic fibrosis patients.
Assuntos
Fibrose Cística/sangue , Deficiência de Vitamina E/complicações , Vitamina E/sangue , Adolescente , Adulto , Criança , Pré-Escolar , Fibrose Cística/complicações , Feminino , Humanos , Recém-Nascido , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Deficiência de Vitamina E/sangueRESUMO
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. 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 (1st-3rd quartile: 316.5-457.0). Vitamin A deficiency was found in 32 (16.3%) subjects studied. Vitamin A concentrations above the reference range were observed only in 3 (1.5%) CF patients. CF patients with vitamin A deficiency were significantly older and had lower values of FEV1 compared to CF subjects with normal vitamin A status. Moreover, vitamin A deficiency occurred more frequently in CF patients with diabetes, Pseudomonas aeruginosa colo- nization, worse lung function and in those without vitamin A supplementation. However, in multiple linear regression analyses, none of the independent variables was documented to be important for predicting vita- min A status. CONCLUSIONS: Vitamin A body resources in CF patients are mostly normal. Moreover, there are no good de- terminants of vitamin A status in these patients. Further studies targeted at exploring potential toxicity and deficiencies of vitamin A in CF patients are needed.
Assuntos
Fibrose Cística/metabolismo , Estado Nutricional , Deficiência de Vitamina A/sangue , Vitamina A/administração & dosagem , Vitamina A/metabolismo , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Adulto JovemRESUMO
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. Vitamin K status was assessed using prothrombin induced by vitamin K absence (PIVKA-II) and the percentage of undercarboxylated osteocalcin (u-OC). PIVKA-II concentrations were higher in cirrhotic than in non-cirrhotic CF patients (median [1st-3rd quartile]: 3.2ng/ml [1.0-10.0] vs. 1.3ng/ml [0.2-2.6], p=0.0029). However, the differences in u-OC percentages between the studied groups did not reach the level of significance (49.4% [7.0-73.8] vs. 8.0% [2.6-59.1], p=0.0501). Based on multiple linear regression analysis the dose of vitamin K and F508del mutation were potentially defined as determinants of vitamin K deficiency. Liver cirrhosis was not documented to be an independent risk factor. In CF patients with liver cirrhosis vitamin K deficiency is not only more frequent, but also more severe. However, not liver cirrhosis, but the presence of a F508del CFTR mutation constitutes an independent risk factor for vitamin K deficiency.
Assuntos
Biomarcadores/sangue , Fibrose Cística/complicações , Cirrose Hepática/complicações , Precursores de Proteínas/sangue , Deficiência de Vitamina K/tratamento farmacológico , Vitamina K/administração & dosagem , Adolescente , Criança , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Feminino , Humanos , Modelos Lineares , Masculino , Osteocalcina/análise , Polônia , Estudos Prospectivos , Protrombina , Fatores de Risco , Deficiência de Vitamina K/complicações , Deficiência de Vitamina K/epidemiologia , Adulto JovemRESUMO
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. 16 pregnancies have been reported (4 patients had two pregnancies); there were 16 live births of which 11 (69%) were premature, 10 (62%) of newborns had low weight. There was one neonatal death. The mean prepregnancy FEV1 (% predicted) and BMI (kg/m2) were 56,6 +/- 14,8% (range 35-95) and 19,9 +/- 1,5 (range 18-23) respectively. The mean FEV1 during pregnancy was 47,7 +/- 15,1% (range 23-87), mean maternal weight gain during pregnancy was 5,5kg. There was a significant decline in FEV1 during pregnancy (16.6 +/- 6,4%, p<0.05) as compared to prepregnancy values. Two women had diabetes mellitus and two developed gestational diabetes. Mean postpregnancy (12 months after delivery) FEV1 was 54.1 +/- 18.8% (range 22-103) and mean BMI was 18.7 +/- 1.8 kg/m2 (range 15-22). There was a significant postpregnancy decline in FEV1 (6.2 +/- 10.6%, p=0.027) and in BMI (1 +/- 1.14 kg/m2, p<0.01) as compared to prepregnancy values. Microbiological status remains unchanged. 11 patients are alive; one patient died 11 years after delivery. The mean follow up time was 8.5 +/- 3.6 years (range 2-23). CONCLUSIONS: Pregnancy was well tolerated in our population of CF women, although affected the rate of yearly decline in FEV1 and BMI. Increased risk of premature and low newborn weight were observed. All pregnancies in CF women should be planned and monitored by CF team.
Assuntos
Fibrose Cística/epidemiologia , Doenças do Recém-Nascido/epidemiologia , Complicações na Gravidez/epidemiologia , Resultado da Gravidez/epidemiologia , Adolescente , Adulto , Índice de Massa Corporal , Fibrose Cística/diagnóstico , Feminino , Volume Expiratório Forçado , Humanos , Bem-Estar do Lactente , Recém-Nascido , Bem-Estar Materno , Polônia/epidemiologia , Gravidez , Complicações na Gravidez/diagnóstico , Gravidez de Alto Risco , Estudos Retrospectivos , Inquéritos e QuestionáriosRESUMO
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. The clinical picture was very heterogenous. We described these cases to show that the use of the mineral oils may increase the risk of lipoid pnemonia.
Assuntos
Óleo Mineral/efeitos adversos , Parafina/efeitos adversos , Pneumonia Lipoide/diagnóstico por imagem , Pneumonia Lipoide/etiologia , Diagnóstico Diferencial , Feminino , Humanos , Pneumopatias/diagnóstico por imagem , Pneumopatias/etiologia , Masculino , Pessoa de Meia-Idade , Pneumonia Aspirativa/diagnóstico por imagem , Pneumonia Aspirativa/etiologia , Tomografia Computadorizada por Raios XRESUMO
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 (86.9%) and non-supplemented - 22 (13.1%) CF patients. Vitamin K status was assessed using prothrombin inducted by vitamin K absence (PIVKA-II) and undercarboxylated osteocalcin (u-OC). The pathological PIVKA-II concentration (≥ 2 ng/ml) and abnormal percentage of osteocalcin (≥ 20%) were found in 72 (42.8%) and 60 (35.7%) subjects, respectively. We found that liver involvement, diabetes, and glucocorticoid therapy were potential risk factors for vitamin K deficiency. Pathological concentrations of PIVKA-II occurred more frequently in patients with pancreatic insufficiency and those who have two severe mutations in both alleles of the CFTR gene. Pathological percentage of u-OC was found more frequently in adult CF patients and those not receiving vitamin K. However, it seems that there are no good predictive factors of vitamin K deficiency in CF patients in everyday clinical care. Early vitamin K supplementation in CF patients seems to be warranted. It is impossible to clearly determine the supplementation dose. Therefore, constant monitoring of vitamin K status seems to be justified.