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1.
J Cyst Fibros ; 2024 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-38960841

RESUMO

BACKGROUND: Microbial dysbiosis has been linked to cystic fibrosis (CF); however, the composition of gut microbiota in adult CF patients in relation to severity of CF transmembrane conductance regulator (CFTR) gene mutation and nutritional status have not yet been explored. Study aimed to assess the gut microbiota composition in adults with CF, and its relationship with the severity of CFTR mutations, and BMI. METHODS: Gut microbiota of 41 adults with CF, and 26 non-CF controls were compared using whole 16S rRNA gene sequencing. Differences in the microbial community between groups of patients classified according to the severity of CFTR mutations, and BMI were assessed. The alpha diversity, beta diversity, and taxa abundance were identified to reflect gut microbiota composition. RESULTS: Results showed a significant decrease in alpha diversity of bacterial communities in CF compared to non-CF group, but no significant difference between the CF groups distinguished by the severity of CFTR mutations. However, more severe mutations were associated with the higher relative abundance of Bacteroides and Streptococcus and the lower relative abundance of Faecalibacterium and Blautia. Undernourished CF patients showed significantly lower alpha diversity compared to non-CF group and CF patients with BMI within the norm. Significant differences in the structure of the gut microbiota between CF and non-CF groups, as well as between BMI groups were also found. CONCLUSIONS: Our research indicates that CF is associated with alterations in gut microbiota in adults. Additionally, in adult CF patients, the composition of the gut microbiota is also related to BMI.

2.
Int J Mol Sci ; 25(12)2024 Jun 18.
Artigo em Inglês | MEDLINE | ID: mdl-38928397

RESUMO

Cystic fibrosis (CF), also known as mucoviscidosis, is the most common autosomal recessive genetic disease in the Caucasian population, with an estimated frequency of 1:2000-3000 live births. CF results from the mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene localized in the long arm of chromosome 7. The product of CFTR gene expression is CFTR protein, an adenosine triphosphate (ATP)-binding cassette (ABC) transporter that regulates the transport of chloride ions (Cl-) across the apical cell membrane. Primary manifestations of CF include chronic lung and pancreas function impairment secondary to the production of thick, sticky mucus resulting from dehydrated secretions. It is well known that CF can cause both anterior and posterior ocular abnormalities. Conjunctival and corneal xerosis and dry eye disease symptoms are the most characteristic manifestations in the anterior segment. In contrast, the most typical anatomical and functional changes relating to the posterior segment of the eye include defects in the retinal nerve fiber layer (RNFL), vascular abnormalities, and visual disturbances, such as reduced contrast sensitivity and abnormal dark adaptation. However, the complete background of ophthalmic manifestations in the course of CF has yet to be discovered. This review summarizes the current knowledge regarding ocular changes in cystic fibrosis.


Assuntos
Regulador de Condutância Transmembrana em Fibrose Cística , Fibrose Cística , Humanos , Fibrose Cística/metabolismo , Fibrose Cística/genética , Fibrose Cística/patologia , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/metabolismo , Oftalmopatias/etiologia , Oftalmopatias/metabolismo , Oftalmopatias/patologia , Mutação , Animais
3.
Adv Med Sci ; 68(2): 474-481, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37945440

RESUMO

PURPOSE: Bronchofiberoscopy (FOB) is a procedure routinely performed for: lung cancer, obstruction, interstitial diseases, foreign bodies' removal, airway clearance, and hemoptysis. It causes acute airway narrowing leading to respiratory and cardiovascular stress. Due to increasing number of ill patients with respiratory failure (RF), conventional oxygen therapy (COT) is frequently insufficient to assure accurate oxygenation and prevent RF in patients requiring FOB. In this clinical scenario, patients may be intubated and supported with invasive mechanical ventilation (IMV) with the specific aim of allowing a safe FOB. However, this invasive strategy is associated with an increased risk of IMV-associated complications. MATERIALS AND METHODS: Our study is a planned prospective multicenter three-arm randomized controlled trial (RCT). The target number of 300 patients was calculated based on the intubation risk in RF patients, which is 0.2-2%. The patients will be assigned to each arm based on Horowitz index. In each arm, the patients will be randomly assigned to one out of two dedicated respiratory support methods in each group i.e. COT/high flow nasal cannula (HFNC), HFNC/non-invasive ventilation (NIV) and NIV/IMV. In the manuscript the current state of art in the area of respiratory support is discussed. We have underlined knowledge gaps in medical evidence which we are planning to reveal with our results. RESULTS: The results of our study are clinically crucial, because they address current gaps concerning COT/HFNC/NIV/IMV. CONCLUSION: The expected findings of this study would allow for careful selection of respiratory support method to safely perform FOB in patients with hypoxemic RF.


Assuntos
Insuficiência Respiratória , Humanos , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Oxigênio , Oxigenoterapia/métodos , Pulmão , Respiração Artificial , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos Multicêntricos como Assunto
4.
Eur J Med Res ; 28(1): 293, 2023 Aug 22.
Artigo em Inglês | MEDLINE | ID: mdl-37608339

RESUMO

BACKGROUND: The management of oral health during severe symptoms of Covid-19 is still a challenge, especially in intensive care units under invasive/noninvasive ventilation in hospital. Understanding the cause-and-effect relationships may allow for individual adjustment of oral care recommendations during Covid-19 disease. The study's objective was to assess Covid-19 patients' oral health status under hospital treatment due to pulmonary adverse Covid-19 outcomes. MATERIAL AND METHODS: Covid-19 patients (mean age 74.4 ± 15.4; n = 120, male n = 50/female n = 70) were admitted to hospital in the acute phase of Covid-19 between January and March 2022 who required oxygen therapy due to pneumonia, rapid respiratory failure, low saturation. Blood and radiological tests were taken according to National Health Fund guidelines. The condition of teeth (Decayed, Missing, Filled teeth as DMFT index), dental hygiene (Plaque Control Record as PCR index), periodontal status (probing depth PD, clinical attachment CAL, bleeding on probing BOP) and oral mucosa (BRUSHED and Beck scores) were examined. RESULTS: Charateristics of the teeth (dental caries 35.2%, DMFT Median 22), plaque retention (83.4%), advanced periodontitis (48.3%), xerostomia (74.2%), oral mucosa inflammation (80.8%), angular cheilitis (53.3%), hemorrhagic (21.7%) showed a high incidence of harmful oral conditions. BRUSHED model and Beck score indicated moderate oral dysfunction and need for oral care every 8 h. Spearman's analysis revealed a significant positive correlation between pneumonia and neutrophile, interleukin-6 IL-6, C-reactive protein CRP (p = 0.01, p < 0.001, p < 0.001), negative to lymphocyte count (p < 0.001). Multiple and logistic regressions selected the following risk predictors for pneumonia as IL-6, CRP, obesity and for severe COVID-19 symptoms D-dimer level and a lack of targeted vaccination (p < 0.001). Among oral predictors, the PCR index and Beck score were significant for both outcomes (respectively p < 0.001, p < 0.012). Patients who received oxygen therapy with face masks had more often angular heilitis and debris (p = 0.025, p = 0.035). CONCLUSIONS: COVID-19 hospitalised patients with severe symptoms crossing with poor oral health-related conditions. This may exacerbate a response for COVID infection, and play a role in cytokine storm. For Covid-19 management, to inhibit extraoral/intraoral complications, it is recommended to adjust oral hygiene procedures, including antibacterial, protective, moisturising agents after individual oral health assessment.


Assuntos
COVID-19 , Cárie Dentária , Ventilação não Invasiva , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Ventilação não Invasiva/efeitos adversos , Prevalência , Interleucina-6 , COVID-19/complicações , COVID-19/epidemiologia , SARS-CoV-2 , Oxigênio
5.
Artigo em Inglês | MEDLINE | ID: mdl-36294222

RESUMO

Background: Non-adherence occurs in various groups of patients, including those with chronic diseases. One strategy to increase adherence among oncological patients is to individualise treatment and expand pharmaceutical care. Pharmaceutical labels that remind patients how they should take their medications are of great importance in this respect. Objective: The main objective of this study was to evaluate medication adherence in oncological patients, and to gather their opinions on the individual medication labelling system as an element of effective treatment. Methods: The study was conducted in 2021 among 82 patients of the oncological department of the Centre of Oncology in Radom. The research tool was a questionnaire consisting of personal data and two parts relating to the patient's disease and the medication labelling system. Results: Nearly half of the respondents reported that they forget to take medications and how they should take them. These problems increased with the age of the patient and the number of administered medications. Of the respondents, 89% stated that the labels with dosing information are helpful. Over 67% agreed that these labels should be affixed to all medications. Nearly 90% of the respondents believed the labels should be available in all pharmacies. Conclusions: Non-adherence is a common phenomenon among oncological patients. Pharmacists providing a labelling service for medicinal products can play a significant role in reducing this phenomenon.


Assuntos
Serviços Comunitários de Farmácia , Neoplasias , Humanos , Institutos de Câncer , Polônia , Farmacêuticos , Adesão à Medicação , Aconselhamento , Preparações Farmacêuticas , Neoplasias/tratamento farmacológico
6.
Artigo em Inglês | MEDLINE | ID: mdl-35564907

RESUMO

INTRODUCTION: Spiritual care is needed in a clinical setting to improve the patients' quality of life. Deep connection with another person and delight with the beauty of nature or art and (in some cases) with God are all transcendental experiences. They may enable patients to ascribe meaning to their life with a chronic illness, find hope and well-being despite burdening symptoms. The opposite situation: lack of inner peace, inability to accept what is happening, feeling disconnected from others is called spiritual distress. OBJECTIVES: The aim of this research is to assess spiritual distress and spiritual needs of a group of Polish chronically ill patients and find associations with independent variables in order to provide data for recommendations on spiritual care in Poland. PATIENTS AND METHODS: 204 patients treated at the University Hospital and the Cystic Fibrosis Clinic in Poznan were surveyed in 2017 and 2018 with an original questionnaire. RESULTS: Over half of the patients felt that their illness was life-threatening. A little more than half reported that faith was a resource to cope with suffering. Almost all patients showed signs of spiritual distress, and more than half expressed spiritual needs. The intensity of distress correlated only with the severity of the disease. The most important predictor of having spiritual needs was recognizing faith as a resource. CONCLUSIONS: Spiritual needs are associated with personal beliefs; however, spirituality spans beyond the religious context since spiritual distress is unrelated to the level of religious devotion. Therefore, any patient with a severe chronic disease needs basic spiritual care, which includes being treated with compassion.


Assuntos
Qualidade de Vida , Espiritualidade , Doença Crônica , Estudos Transversais , Humanos , Polônia
7.
J Clin Med ; 11(10)2022 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-35628882

RESUMO

Cystic fibrosis (CF) is a genetic disease that is caused by a defect in the gene coding for the transmembrane cystic fibrosis transmembrane conductance regulator (CFTR). Research papers published so far point out that despite the numerous dental treatment needs of CF patients, there are no oral care guidelines for this group of patients. The aim of the article is to propose standards of dental prophylactic and therapeutic procedures for CF patients in different age groups. Regardless of the CF patient's age, dental check-ups should be scheduled at least every 6 months. However, taking into account the actual condition of the individual CF patients, therapeutic visits may be scheduled for earlier dates, to provide well-fitting treatment, considering the level of risk of oral diseases. The described management standards may be helpful and may improve the quality of dental care provided to CF patients.

8.
Adv Respir Med ; 2021 Dec 09.
Artigo em Inglês | MEDLINE | ID: mdl-34881808

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.

9.
Artigo em Inglês | MEDLINE | ID: mdl-32560490

RESUMO

BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare, ciliopathic disorder. In many ciliopathies, dental anomalies are observed alongside other symptoms of the disease. To date, there are no published reports concerning the dental developmental problems that are associated with ciliary defects in PCD patients. METHODS: Patients suffering from PCD underwent dental clinical examination, which included the assessment of developmental disorders regarding the number and morphological structure of the teeth (size and shape) as well as developmental disorders of mineralised dental tissues. Then, three-dimensional radiographic examination was performed utilising Cone Beam Computed Tomography (CBCT). RESULTS: Four PCD patients, aged 31-54, agreed to enter the study. Dental examinations showed the presence of dental developmental disorders in three of them. Additionally, CBCT showed abnormalities in those patients. CONCLUSIONS: 1. The dental phenotype in PCD patients seems to be heterogeneous. Tooth developmental disorders resulting from abnormal odontogenesis may be a symptom of PCD that is concomitant with other developmental abnormalities resulting from malfunctioning primary cilia. 2. Patients with ciliopathies are likely to develop dental developmental defects. Therefore, beginning in early childhood, they should be included in a targeted specialised dental programme to enable early diagnosis and to ensure dedicated preventive and therapeutic measures.


Assuntos
Transtornos da Motilidade Ciliar , Anormalidades Dentárias , Adulto , Criança , Pré-Escolar , Transtornos da Motilidade Ciliar/complicações , Humanos , Pessoa de Meia-Idade , Dente/crescimento & desenvolvimento
10.
Pol J Microbiol ; 68(3): 377-382, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31880883

RESUMO

The aim of this study was to assess the periodontal status of cystic fibrosis (CF) adult patients and to evaluate whether there is a correlation between the bacterial population of the subgingival biofilm and the health status of the periodontal tissues in this group of adults. The study involved 22 cystic fibrosis adult patients. The periodontal condition was assessed using Plaque Index (PLI), Gingival Index (GI), and Probing Pocket Depth (PPD). The gingival sulcus samples were analyzed by the Real-Time PCR assay (RT-PCR). Majority of patients showed moderate or severe bacterial dental plaque accumulation, but none of them had clinical symptoms of periodontal diseases. RT-PCR showed the presence of periopathogens in 50% of patients. Red complex microorganisms were detected in 9.09%, orange complex in 27.27%, and green complex in 31.82% of the samples analyzed. In cystic fibrosis patients colonized by periopathogens, the periodontal markers were significantly higher in comparison to not colonized by periopathogens patients. Despite the widespread presence of bacterial dental deposits in the cystic fibrosis adult patients examined, none of them has clinical symptoms of periodontal disease; however, the presence of periodontal pathogens in subgingival biofilm may represent a possible risk factor of this disease in the future. An unsatisfactory level of oral hygiene in any patient with cystic fibrosis indicates a need to focus on standards of dental care for such patients.The aim of this study was to assess the periodontal status of cystic fibrosis (CF) adult patients and to evaluate whether there is a correlation between the bacterial population of the subgingival biofilm and the health status of the periodontal tissues in this group of adults. The study involved 22 cystic fibrosis adult patients. The periodontal condition was assessed using Plaque Index (PLI), Gingival Index (GI), and Probing Pocket Depth (PPD). The gingival sulcus samples were analyzed by the Real-Time PCR assay (RT-PCR). Majority of patients showed moderate or severe bacterial dental plaque accumulation, but none of them had clinical symptoms of periodontal diseases. RT-PCR showed the presence of periopathogens in 50% of patients. Red complex microorganisms were detected in 9.09%, orange complex in 27.27%, and green complex in 31.82% of the samples analyzed. In cystic fibrosis patients colonized by periopathogens, the periodontal markers were significantly higher in comparison to not colonized by periopathogens patients. Despite the widespread presence of bacterial dental deposits in the cystic fibrosis adult patients examined, none of them has clinical symptoms of periodontal disease; however, the presence of periodontal pathogens in subgingival biofilm may represent a possible risk factor of this disease in the future. An unsatisfactory level of oral hygiene in any patient with cystic fibrosis indicates a need to focus on standards of dental care for such patients.


Assuntos
Biofilmes , Fibrose Cística/microbiologia , Doenças Periodontais/microbiologia , Adulto , Bactérias/classificação , Bactérias/genética , Bactérias/isolamento & purificação , Fenômenos Fisiológicos Bacterianos , Feminino , Gengiva/microbiologia , Humanos , Masculino , Pessoa de Meia-Idade , Índice Periodontal , Periodonto/microbiologia , Adulto Jovem
11.
J Cyst Fibros ; 18(6): 762-771, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-30473190

RESUMO

BACKGROUND: The oral cavity is an important reservoir of microorganisms and can be a potential source of infection of the respiratory tract in CF patients. The literature on the oral health does not explicitly determine dental problems in these patients. The aim of the study was to systematically review the studies relating to oral status in CF patients. METHODS: A systematic review of papers published from 1997 to 2018 regarding oral status in CF children and adults, carried out in accordance with the Cochrane Collaboration guidelines. RESULTS: The search resulted in 143 studies. Thirteen publications were included in the systematic review.


Assuntos
Fibrose Cística/complicações , Doenças Periodontais/etiologia , Doenças Dentárias/etiologia , Adulto , Criança , Cárie Dentária/etiologia , Esmalte Dentário , Humanos
12.
Adv Respir Med ; 86(5): 220-226, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30378649

RESUMO

INTRODUCTION: Bronchial artery embolisation (BAE) is one of the methods used in massive and recurring haemoptysis. The aim of the study is to determine the effectiveness and complications of bronchial artery embolisation in recurring haemoptysis. MATERIAL AND METHODS: The analysis included 47 embolisation procedures performed on 30 patients treated between 2011 and 2017 in the Department of Respiratory Medicine, Allergology and Pulmonary Oncology due to haemoptysis. The patient's age ranged between 18 and 71 years, while mean age at the time of BAE was 33.5 years. Patients with tuberculosis constituted 73.33% (n = 22) of the sample and underwent 31 embolisation procedures in total. The remaining part of the sample (n = 8) collectively underwent 16 BAEs. The analysis was conducted by verifying the medical documentation, as well as carrying face-to-face and phone conversations. RESULTS: Immediate control due to the inhibition of bleeding was obtained in 95.75% of cases. Recurrence within 3 days of BAE was reported in 5 patients (10.63%), and 4 re-embolisation procedures were conducted. In 10 patients (33.33%), recurrence was observed during the first year post-BAE, while it was reported in 17 cases during the whole observation period (56.66% of patients). The subjects who underwent re-embolisation demonstrated recurrence-free periods lasting from 2 days to 63 months. In patients with recurrence but no re-embolisation, the shortest and longest haemoptysis-free time was 2 and 35 months, respectively. 11 patients (36.66%) required several embolisation procedures during the whole observation period. CONCLUSIONS: BAE is a highly successful procedure in treating haemoptysis. The risk of complications is low.


Assuntos
Artérias Brônquicas/fisiopatologia , Embolização Terapêutica/métodos , Hemoptise/terapia , Adulto , Idoso , Feminino , Hemoptise/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto Jovem
13.
World J Gastroenterol ; 24(36): 4164-4177, 2018 Sep 28.
Artigo em Inglês | MEDLINE | ID: mdl-30271081

RESUMO

AIM: To determine tissue expression (mRNA, protein) of two types of mucins [mucin 1 (MUC1) and mucin 2 (MUC2)] in patients with colorectal cancer (CRC). METHODS: Expression of membrane-bound mucin (MUC1) and secretory mucin (MUC2) in CRC (mRNA, protein) were analyzed in tissue material including fragments of tumors obtained from CRC patients (n = 34), and fragments of normal colorectal tissue from the same patients (control). The analysis was conducted using real-time quantitative polymerase chain reaction (RT-qPCR) (transcripts), immunohistochemistry (IHC) (apomucins), and the modern approach for morphometric analysis of IHC reaction (HSV filter software). Results on tissue expression of both mucins (mRNA, protein) were compared to histological alterations in colorectal cancer samples and correlated with selected clinical data in the patients. The statistical analysis was conducted using Statistica PL v. 12.0 software. RESULTS: Significantly higher expression of the MUC1 mRNA in the CRC, compared with the control and the borderline correlation of mRNA expression with MUC1 protein levels in colorectal samples was observed. The expression of apomucins concerned cell membranes (MUC1) and cytoplasm (MUC2) and occurred both in control tissues and in most cancerous samples. There were no significant relationships between MUC1 (mRNA, protein) and the clinicopathological data of patients. MUC2 protein expression was significantly lower as compared to the control, while MUC2 mRNA expression was comparable in both groups. The MUC1/MUC2 ratio was significantly higher in CRC tissues than in the control. The higher expression of MUC2 was a feature of mucinous CRC subtypes, and characterized higher histological stage of tumors. Negative correlations have been obtained between MUC2 and the Ki-67 antigen, as well as between MUC2 and p53 protein expressions in CRC. CONCLUSION: A combination of tissue overexpression of MUC1, reduced MUC2 expression, and high ratio of MUC1/MUC2 is a factor of poor prognosis in CRC patients. MUC2 tissue expression allows to differentiate mucinous and nonmucinous CRC subtypes.


Assuntos
Adenocarcinoma Mucinoso/patologia , Biomarcadores Tumorais/metabolismo , Neoplasias Colorretais/patologia , Mucina-1/metabolismo , Mucina-2/metabolismo , Adenocarcinoma Mucinoso/mortalidade , Adenocarcinoma Mucinoso/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , Neoplasias Colorretais/mortalidade , Neoplasias Colorretais/cirurgia , Feminino , Humanos , Intestino Grosso/patologia , Masculino , Pessoa de Meia-Idade , Mucina-1/análise , Mucina-1/genética , Mucina-2/análise , Mucina-2/genética , Estadiamento de Neoplasias , Prognóstico , RNA Mensageiro/metabolismo , Taxa de Sobrevida
14.
Adv Med Sci ; 63(2): 341-346, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30081288

RESUMO

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 µg/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/sangue
15.
Acta Sci Pol Technol Aliment ; 16(3): 345-354, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29055982

RESUMO

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 Jovem
16.
Adv Exp Med Biol ; 1020: 43-51, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28255913

RESUMO

The aim of this study was to elucidate body composition, anthropometric indices, and hydration status in obstructive sleep apnea (OSA) patients, taking into account different disease stages, gender, and the possibility of the presence of cachexia. There were 98 OSA patients and 23 control subjects enrolled into the study. All study participants underwent polysomnography examination. Body mass index (BMI), fat mass index (FMI), fat free mass, muscle mass, body cell mass, total body water, and extracellular and intracellular water were evaluated. The neck, abdominal, and waist circumference was measured. We found that overweight and obesity were present in 96% of patients. Cachexia was present in one OSA individual with comorbidities. Apnea-hypopnea index correlated with the neck and waist circumference, and with BMI in OSA patients. All muscle indices and water contents above outlined were significantly higher in severe OSA compared with control subjects. BMI, FMI, neck circumference, and extracellular water were greater in a subset of severe OSA compared with a moderate OSA stage. The female OSA patients had a higher FMI than that present in males at a comparable BMI. We conclude that the most body composition indices differed significantly between severe OSA patients and control subjects. A higher FMI in females at a comparable BMI could be due to a discordance between BMI and FMI. Cachexia occurs rarely in OSA and seems to coexist with comorbidities.


Assuntos
Composição Corporal , Caquexia/complicações , Obesidade/complicações , Apneia Obstrutiva do Sono/complicações , Índice de Massa Corporal , Feminino , Humanos , Masculino , Polissonografia , Fatores de Risco
17.
Adv Respir Med ; 84(6): 307-309, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-28009029
18.
Pol Arch Med Wewn ; 126(5): 330-9, 2016 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-27230560

RESUMO

INTRODUCTION    Obstructive sleep apnea (OSA) is considered to be one of the major causes of resistant arterial hypertension (RAH). Apnea episodes cause hypoxia, which triggers the activation of the renin-angiotensin-aldosterone system. This leads to water retention and swelling in the neck region, exacerbating OSA symptoms. It is assumed that the use of eplerenone may reduce the swelling and thus alleviate the severity of OSA. OBJECTIVES    We aimed to prospectively assess the impact of eplerenone on the severity of OSA and arterial stiffness in patients with RAH. PATIENTS AND METHODS    The study included 31 patients with RAH and OSA. The exclusion criteria were as follows: secondary hypertension, myocardial infarction, stroke 6 months prior to the study, congestive heart failure, chronic kidney failure, alcohol or drug addiction, and active cancer. In all patients, the following tests were performed: blood pressure (BP) measurement (traditionally and using ambulatory BP measuring [ABPM]), applanation tonometry, polysomnography, and the apnea-hypopnea index (AHI) calculation. The tests were done before and after 3 months of eplerenone therapy. Patients received 50 mg of oral eplerenone daily, along with other hypertensive drugs. RESULTS    The mean age of participants was 57.76 ±6.16 years. After 3 months of eplerenone therapy, we observed a significant reduction in the AHI, neck circumference, BP, aortic pulse wave, and arterial wall stiffness. There were significant correlations between the AHI and mean BP measured by ABPM and between the AHI and arterial stiffness parameters. CONCLUSIONS    Our results provide evidence for the clinical significance of eplerenone, not only as an antihypertensive medication but also as a drug that may reduce the severity of OSA and arterial stiffness in patients with RAH and OSA.


Assuntos
Hipertensão/etiologia , Apneia Obstrutiva do Sono/tratamento farmacológico , Espironolactona/análogos & derivados , Rigidez Vascular/efeitos dos fármacos , Idoso , Eplerenona , Feminino , Humanos , Hipertensão/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Apneia Obstrutiva do Sono/complicações , Apneia Obstrutiva do Sono/patologia , Espironolactona/uso terapêutico
19.
Pneumonol Alergol Pol ; 83(5): 394-400, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26379003

RESUMO

The proper care of cystic fibrosis patients extends over their lifetime. More than half of the children with the disease die before adulthood. An important element in the patient's care is a time of transition from a paediatric to the care of an internist and the patient's acceptance of this necessity. Transition from paediatric care to an internist should be adequately prepared. It is not only a question of transfer of medical records, but also careful preparation of patients for such transition. The patients expect not only continuity of care but also the introduction to the management with the disease. The creation of a base for specialist hospital treatment for exacerbation of the disease at the adulthood is an important element in the care of these patients. The problem has been solved in the children group, but is still waiting for solution in adults with cystic fibrosis. It has been proven that care in the centres carried out by a specialized team ensures longer life and better quality of life of these patients. The paper is an overview of these two important elements of care of adults with cystic fibrosis.


Assuntos
Fibrose Cística , Transição para Assistência do Adulto , Adolescente , Adulto , Criança , Humanos , Qualidade de Vida , Adulto Jovem
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