Initiating transitional care for adolescents with cystic fibrosis at the age of 12 is both feasible and promising

Acta Paediatrica, EarlyView.
Source: Acta Paediatrica - Category: Pediatrics Authors: Source Type: research

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Conclusions: The TAI was high particularly among children younger than 14 years. There was a positive correlation between the self-referenced TA and the one presumed by the Psychology (p=0.032) and the nutrition (p=0.012) professionals.RESUMO Objetivo: Avaliar o grau de ades ão ao tratamento (AT) autorreferida e pesquisar sua associação com variáveis clínicas e sociodemográficas em pacientes com fibrose cística (FC) de um centro de referência e comparar o grau de AT autorreferida com o presumido pela equipe multidisciplinar. Métodos: Estudo transversal, inclu in...
Source: Revista Paulista de Pediatria - Category: Pediatrics Source Type: research
(University of M ü nster) Cystic fibrosis is the most frequent severe inherited disorder worldwide. Researchers have now discovered a novel disease that might lead to a better understanding of cystic fibrosis and new treatment options in the future. The results have been published in the scientific journal " Journal of Medical Genetics " .
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news
Human neutrophil elastase (HNE) is a major cause of the destruction of tissues in cases of several different chronic andinflammatory diseases. Overexpression of the elastase enzyme plays a significant role in the pathogenesis of various diseases including chronic obstructive pulmonary disease (COPD), acute respiratory distress syndrome, rheumatoid arthritis, the rare disease cyclic hematopoiesis (or cyclic neutropenia), infections, sepsis, cystic fibrosis, myocardial ischemia/reperfusion injury and asthma, inflammation, and atherosclerosis. Human neutrophil elastase is secreted by human neutrophils due to different stimuli...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
AbstractCystic fibrosis (CF), an autosomal recessive disorder, occurs due to mutations inCFTR gene resulting in impaired cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel function in various epithelia. In addition to the well-known pulmonary and pancreatic morbidities, CF is characterized by electrolyte and acid-base abnormalities- hypochloremia, hyponatremia, hypokalemia and metabolic alkalosis. These are collectively known as Pseudo-Bartter syndrome, as similar abnormalities are seen in Bartter syndrome- an inherited tubulopathy affecting thick ascending limb of loop of Henle. There may be a sig...
Source: Indian Journal of Pediatrics - Category: Pediatrics Source Type: research
Condition:   Cystic Fibrosis in Children Interventions:   Behavioral: Exercise;   Behavioral: Unsupervised exercise;   Drug: CFTR Modulators Sponsor:   Universidad Europea de Madrid Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
rodon Identification of the cystic fibrosis transmembrane conductance regulator (CFTR) gene and its numerous variants opened the way to fantastic breakthroughs in diagnosis, research and treatment of cystic fibrosis (CF). The current and future challenges of molecular diagnosis of CF and CFTR-related disorders and of genetic counseling are here reviewed. Technological advances have enabled to make a diagnosis of CF with a sensitivity of 99% by using next generation sequencing in a single step. The detection of heretofore unidentified variants and ethnic-specific variants remains challenging, especially for newborn scre...
Source: Genes - Category: Genetics & Stem Cells Authors: Tags: Review Source Type: research
People with CF are prone to airway bacterial infection and experience pulmonary exacerbations (PEx), periods of acute increases in respiratory signs and symptoms often coupled with an acute lung function drop, throughout their lives. [1,2] PEx are considered important clinical events; [1-3] guidelines recommend treatment with increased airway clearance, antimicrobial therapy, and nutritional and psychosocial support, [4-5] with antimicrobials chosen by in vitro antimicrobial susceptibility testing (AST) of patient bacterial isolates.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
Condition:   Cystic Fibrosis in Children Interventions:   Behavioral: Exercise;   Behavioral: Unsupervised exercise;   Drug: CFTR Modulators Sponsor:   Universidad Europea de Madrid Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
(Van Andel Research Institute) Van Andel Institute scientists have revealed the first known atomic structure of a 'molecular machine' responsible for installing critical signaling proteins into cellular membranes. The findings, published today in Nature, shed new light on how this process works, and lay the foundation for potential future therapies for diseases like cancer, Alzheimer's and cystic fibrosis.
Source: EurekAlert! - Biology - Category: Biology Source Type: news
Cystic fibrosis (CF) is a common autosomal recessive disorder in Caucasian populations, caused by pathogenic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Currently, more than 2000 CFTR variants have already been identified [1]. CFTR variant LRG_663t1: c.350G>A (p.Arg117His, traditional name: R117H) is a missense variant in exon 4, leading to conductance (class IV) as well as CFTR gating (class III) defects [2,3]. It has an estimated allele frequency of 0.3% in Caucasian populations [1].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
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