Inhaled corticosteroids for cystic fibrosis : a review of clinical effectiveness / authors, Kwakye Peprah, Suzanne McCormack

Cystic fibrosis (CF) is a rare chronic genetic disease that affects multiple systems in the body including the respiratory tract, pancreas, gastro-intestinal tract and liver. The disorder is caused by mutations of the CF transmembrane conductance regulator (CFTR) gene. Approximately, one in 25 Canadians carries an abnormal CFTR gene, and CF occurs in children who inherit two abnormal genes, one from each parent. It is estimated that one in every 3,600 children born in Canada has CF, with more than 4,300 Canadian children, adolescents, and adults with CF attending specialized CF clinics. The disease has no cure at present, and it is the most common fatal genetic disease affecting Canadian children and young adults. Lung disease is the most prominent manifestation of CF, and it is reported to account for nearly 85% of deaths. Respiratory tract abnormalities in CF patients cause mucus plugging of the airways, bronchial wall thickening due to inflammation, increased susceptibility to respiratory tract infection, and airway destruction. Much of the pulmonary damage begins with lung inflammation. Although a normal inflammatory response is beneficial to host defence mechanisms, and helps to prevent the spread of infection, the excessive inflammation seen in CF patients is harmful as it contributes to the disease and associated death. One of the goals in the treatment of cystic fibrosis is to reduce lung inflammation. Corticosteroids are potent anti-inflammatory drugs which have been widely used in the treatment of a variety of diseases with underlying inflammation including asthma and chronic obstructive pulmonary disease (COPD). They exert direct inhibitory effects on many inflammatory cells, and regular use of inhaled corticosteroids (ICS) has been reported to reduce the number of mast cells within the airways, decrease airway microvascular leakage, and lessen mucus production. Although benefit of its use in CF has not been proven, ICS has been widely prescribed as anti-inflammatory agents to treat children and adults with CF empirically. The objective of this report is to summarize the evidence regarding the clinical effectiveness of ICS for the treatment of CF..

Medienart:	E-Book

Erscheinungsjahr:	March 12, 2019
Erschienen:	Ottawa: CADTH ; March 12, 2019
Ausgabe:	Version 1.0.

Reihe:	CADTH rapid response report: summary with critical appraisal

Sprache:	Englisch

Beteiligte Personen:	Peprah, Kwakye [VerfasserIn] McCormack, Suzanne [VerfasserIn]

Links:	www.ncbi.nlm.nih.gov [teilw. kostenfrei]

Themen:	Administration, Inhalation Adrenal Cortex Hormones Anti-Inflammatory Agents Canada Cystic Fibrosis Lung Diseases, Obstructive Review Tables Treatment Outcome

Anmerkungen:	Includes bibliographical references. - Description based on online resource; title from PDF title page (viewed February 6, 2020)

Umfang:	1 online resource (1 PDF file (25 pages)) ; illustrations.

Förderinstitution / Projekttitel:

PPN (Katalog-ID):	1773188763