This issue of Seminars in Respiratory and Critical Care Medicine reviews our current knowledge of key areas related to cystic fibrosis CF. Although Seminars has addressed CF previously, our knowledge of this condition has increased considerably and our treatment options are vastly improved. The discovery of the CF gene, coding for the cystic fibrosis transmembrane conductance regulator CFTRhas led to tremendous progress in therapeutics directed at the complications that result from the genetic mutations. The prognosis of a child born with CF has steadily improved dramatically over the last few decades, and today we are on the precipice of broad access to highly effective therapies with relatively low associated treatment burden that address the basic underlying defect. As survival has increased we have moved CF from being a pediatric illness to one that is merely pediatric in diagnosis, such that adulthood should be the expectation for all affected families. There remains a need to understand the etiology and progression of CF-associated complications. While CF diagnosis, whether by clinical symptoms or through newborn screening, has become routine as a result of improved diagnostic techniques and understanding of gene mutations, we are now more aware of CFTR-related complications which may not meet the diagnostic criteria of CF, but which can have real health consequences for affected individuals. In this Issue, we have included topics such as the basic pathophysiology associated with CFTR dysfunction, the complications of the condition including lung and pancreatic disease, what we have learned about the microbiologic aspects of sinus and lung disease, as well as endocrine and gastrointestinal or liver disease. Even with our newest therapies, patients who gain access to them after disease-associated complications have occurred will continue to suffer from irreversible pathologies, and so we have included a discussion of organ transplantation. We have invited international experts to write these up-to-date reviews based on cystic fibrosis dating research or clinical expertise and extensive experience within this field. In summary, this issue of Seminars will hopefully make clear the challenges of managing patients with cystic fibrosis, addressing the complicated diagnostic and treatment approaches required to manage and prevent progression of disease and serious complications. With the advance of our therapeutics, we anticipate a growing population of adult patients, for whom a workforce trained in the care of these patients will be needed. We would like to thank all the contributors for their enthusiasm and hard work. We also extend our thanks to Joseph P. Lynch, Editor-in-Chief of Seminars in Respiratory and Critical Care Medicineas well as all those at the Editorial Office for their expertise in producing this issue of Seminars in Respiratory and Critical Care Medicine. Jahre Archiv Ähnliche Zeitschriften. RSS-Feed cystic fibrosis dating Bitte kopieren Sie die angezeigte URL und fügen sie dann in Ihren RSS-Reader ein. PDF herunterladen. Semin Respir Crit Care Med ; 40 06 : DOI: Weitere Informationen Publikationsverlauf Publikationsdatum: Dezember online.
Drug therapies for reducing gastric acidity in people with cystic fibrosis
Thieme E-Journals - Seminars in Respiratory and Critical Care Medicine / Abstract Friday, October 20, Featured resource article. Study to Evaluate the Safety and Efficacy of Ciprofloxacin (Inhaled) in Patients With Cystic Fibrosis date) - Ability to perform reproducible pulmonary. Unlocking the Secrets of Cystic Fibrosis: A Genetic Study of people with CF in Germany Date. Drug therapies for reducing gastric acidity in people with cystic fibrosis - PMCStudienstandorte Toledo Children's Hospital Toledo, United States, Two authors independently selected the trials to be included in the review. This left 8 participants in each group. Although Seminars has addressed CF previously, our knowledge of this condition has increased considerably and our treatment options are vastly improved. Modern CFTRm have a positive effect on the clinical course of CF-CRS.
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Added value of this study. Up to 24 weeks of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious and safe in patients with cystic fibrosis ≥12 years of age who. Friday, October 20, Featured resource article. Study to Evaluate the Safety and Efficacy of Ciprofloxacin (Inhaled) in Patients With Cystic Fibrosis date) - Ability to perform reproducible pulmonary. Current CFTR modulator therapies, such as elexacaftor–tezacaftor–ivacaftor, have transformed cystic fibrosis care. Unlocking the Secrets of Cystic Fibrosis: A Genetic Study of people with CF in Germany Date.Risk of bias Bias Authors' judgement Support for judgement Random sequence generation selection bias Unclear risk Described as randomised, but no details of method given. In the Chalmers trial, participants received either cimetidine or placebo in addition to normal enzyme therapy Chalmers In six trials there were no participant withdrawals reported, but it was not explicitly stated that no participants withdrew DiMango ; Heijerman ; Heijerman ; Lubin ; Schoni ; Weber No trials have been identified assessing the effectiveness of these agents in improving quality of life, the complications of increased gastric acidity, or survival. In a third trial, participants received either esomeprazole 40 mg or placebo twice daily DiMango We included 17 trials in this review. Studienstandorte Rady Children's Hospital—San Diego San Diego, United States, Durie suggested that this meant that response to the single drug regimens were maximal Durie Oral pancreatic enzyme therapy to improve the nutritional status and decrease maldigestion due to pancreatic insufficiency in people with CF has been available for several decades. Concealment of allocation was adequate in only one trial in which the randomisation procedure was carried out at the pharmacy in the hospital Robinson In the trial by Schoni, the effectiveness of cimetidine compared to placebo was evaluated Schoni Studienstandorte Indiana University Indianapolis, United States, It can also increase acidity in the stomach, which may lead to heartburn and peptic ulcers. Könnte ich ein Scheinmedikament erhalten? We would like to thank all the contributors for their enthusiasm and hard work. Studienstandorte The Children's Hospital Aurora, United States, Participants 12 children with CF. We were therefore unable to identify any potential reporting biases. This is an update of a previously published review. Studienstandorte The Prince Charles Hospital Chermside, Australia, Koletzo Participants 10 children with CF 7 males, 5 females , mean age Synthetic prostaglandins can also be used to reduce gastric acid secretion and stimulate bicarbonate secretion from the small bowel. All trials stated that allocation was randomised, but no trials described the method of randomisation used. Stool collection from 1 participant was not complete, so stool data based on 7 participants only. Choose a collection Unable to load your collection due to an error Please try again. Trial Dates May From our review, no conclusive benefit of improved nutritional status and lung function can be deduced from the effects of agents that reduce gastric acidity. Studienstandorte Toledo Children's Hospital Toledo, United States, This trial contained 4 treatment periods, 3 of which used interventions that were not relevant to the review, therefore no comparisons could be made. The number of trials assessing different agents for reducing gastric acidity was small. Although a single new abstract was identified which was eligible for inclusion in the review, the limited narrative information provided has not led to any change in the conclusions of this review. Malabsorption of fat and protein contributes to poor nutritional status in people with cystic fibrosis. The included trial reported diarrhoea and abdominal discomfort as adverse effects in participants on the prostaglandin E2 analogue, enprostil treatment Heijerman Furthermore, nutritional status is also related to lung function and exercise performance in people with CF Neijens
