cystic fibrosis
Correction of the ion transport defect in Cystic Fibrosis by small molecules
... Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Assembly of f[r] ...
180
Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis.
... in cystic fibrosis (CF) is mainly related to chronic lung infection and inflammation, uncontrolled tissue rearrangements and fibrosis, and yet the underlying mechanisms remain largely ...and ...
10
Cystic fibrosis, are we missing in India?
... that Cystic fibrosis (CF), is now a pan ethnic ...cystic fibrosis. 90% do not have facilities for diagnosis of cystic fibrosis like sweat chloride test and ...of cystic ...
5
Medications used in pediatric cystic fibrosis population
... of cystic fibrosis ...with cystic fibrosis, patients are generally on several ...diagnosed. Cystic fibrosis patients often have difficulties in complying with treatment, ...
8
Hemoglobin oxygen affinity in patients with cystic fibrosis.
... Our results confirm former investigations that there is a small right shift of the standard ODC in most patients with cystic fibrosis probably caused by slightly increased intraerythrocytic concentra- tions ...
10
Structure and function of the cystic fibrosis transmembrane conductance regulator
... Cystic fibrosis (CF) is a lethal autosomal recessive genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR). Mutations in the CFTR gene may result in a defective processing ...
8
Avaliação da qualidade de vida de pacientes com fibrose cística por meio do Cystic Fibrosis Questionnaire.
... escore de Shwachman, enquanto a repercussão da doença no aparelho respiratório é avaliada por espirometria. Outros métodos também são utilizados, como o Cystic Fibrosis Clinical Score e o teste de caminhada ...
9
Plasma sCD14 as a biomarker to predict pulmonary exacerbations in cystic fibrosis.
... We enrolled 64 adult patients from the Adult Cystic Fibrosis (CF) Clinic at St. Paul’s Hospital (Vancouver, British Columbia, Canada) between April 2009 and June 2010 who were clinically stable at the time ...
7
Prevalência da mutação ΔF508 no gene cystic fibrosis transmembrane conductance regulator em pacientes com fibrose cística em um centro de referência no Brasil.
... the cystic fibrosis transmembrane conductance regulator gene among patients with cystic fibrosis diagnosed by the sweat test for sodium and chlorine and followed at the Pediatric Pneumology ...
4
Nutritional Assesment in Cystic Fibrosis Patients( Iran and Newzeland)
... - Compare and contrast the MacDonald Nutritional Screening tool with the Australasian guidelines for Nutrition in Cystic Fibrosis.. - Validate these results in comparison with patient’[r] ...
1
Multidrug-Resistant Nontuberculous Mycobacteria Isolated from Cystic Fibrosis Patients
... Worldwide, nontuberculous mycobacteria (NTM) have become emergent pathogens of pulmonary infections in cystic fibrosis (CF) patients, with an estimated prevalence ranging from 5 to 20%. This work ...
8
Heterogeneity of Pseudomonas aeruginosa in Brazilian cystic fibrosis patients
... The aim of this study was to assess the diversity and genomic variability of Pseudomonas aeruginosa isolates from cystic fibrosis (CF) patients being treated at a university hospital in Brazil. Ninety-seven ...
6
Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations.
... Pulmonary exacerbations (PEx), characterized by increased respiratory symptoms and worsening lung function, are the leading cause of morbidity and decreased quality of life in cystic fibrosis (CF) [1]. ...
13
The Cystic Fibrosis Transmembrane Regulator (CFTR) in the Kidney
... The cystic fibrosis transmembrane regulator (CFTR) is a Cl − channel. Mutations of this transporter lead to a defect of chloride secretion by epithelial cells causing the Cystic Fibrosis ...
8
Vitamin K status in cystic fibrosis patients
... Aris R.M., Merkel P.A., Bachrach L.K., Borowitz D.S., Boyle M.P., Elkin S.L., Guise T.A., Hardin D.S., Haworth C.S., Holick M.F., Joseph P.M., O’Brien K., Tullis E., Watts N.B., White T.B., 2005. Consensus statement: ...
5
Pseudomonas aeruginosa diversification during infection development in cystic fibrosis lungs
... Cystic Fibrosis (CF) is a genetic disorder in which a defect in the cystic fibrosis conductance regulator (CTFR) occurs. This disorder affects many organs, the airways being one of the most ...
108
Adeno-associated virus for cystic fibrosis gene therapy
... as cystic fibrosis. Cystic fibrosis is a severe autosomal recessive disease affecting one in 2500 live births in the white population, caused by mutation of the cystic fibrosis ...
9
Dyspnea perception in cystic fibrosis patients
... in cystic fibrosis patients compared with normal subjects, during an inspiratory resistive loading test and 6-min walk ...with cystic fibrosis (>15 years of age) and 31 age-, gender-, and ...
7
Calcium-activated chloride in cystic fibrosis
... Our present data also provide an explanation for the enhanced proliferation observed for CF epithelial cells. It has been shown earlier that cell proliferation in bronchial [r] ...
160
Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome.
... Methods: SP-A composition and function were assessed in both bronchoalveolar lavage (BAL) fluid and serum of 46 CF patients with mild disease, 25 patients with chronic bronchitis and 22 [r] ...
7