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Cystic fibrosis in adults: diagnostic and therapeutic aspects.
J Bras Pneumol 2008; 34(2):107-17JB

Abstract

Once considered a childhood disease, cystic fibrosis is now also a disease of adults. Increased longevity has resulted in the aging of the cystic fibrosis population. The consequent age-related medical problems among adults with cystic fibrosis have increased medical care needs. These needs are being met by a growing number of non-pediatric pulmonologists and other non-pediatric specialists. The objective of this review was to summarize the current knowledge about diagnosis and treatment in adult cystic fibrosis. In most cases, the diagnosis is suggested by manifestations of chronic sinopulmonary disease and exocrine pancreatic insufficiency. The diagnosis is confirmed by a positive sweat test result. Adult patients may, however, present pancreatic sufficiency and atypical clinical features, sometimes in combination with normal or borderline sweat test results. In such cases, identifying cystic fibrosis mutations and measuring nasal potential difference can have diagnostic utility. The standard therapeutic approach to pulmonary disease includes the use of antibiotics, airway clearance, exercise, mucolytics, bronchodilators, oxygen therapy, anti-inflammatory agents and nutritional support. Appropriate application of these therapies results in most cystic fibrosis patients surviving into adulthood with an acceptable quality of life.

Authors+Show Affiliations

Department of Internal Medicine at the Universidade Federal do Rio Grande do Sul - UFRGS, Federal University of Rio Grande do Sul- School of Medicine, Porto Alegre, Brazil. pdalcin@terra.com.brNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng por

PubMed ID

18345455

Citation

Dalcin, Paulo de Tarso Roth, and Fernando Antônio de Abreu E Silva. "Cystic Fibrosis in Adults: Diagnostic and Therapeutic Aspects." Jornal Brasileiro De Pneumologia : Publicacao Oficial Da Sociedade Brasileira De Pneumologia E Tisilogia, vol. 34, no. 2, 2008, pp. 107-17.
Dalcin Pde T, Abreu E Silva FA. Cystic fibrosis in adults: diagnostic and therapeutic aspects. J Bras Pneumol. 2008;34(2):107-17.
Dalcin, P. d. e. . T., & Abreu E Silva, F. A. (2008). Cystic fibrosis in adults: diagnostic and therapeutic aspects. Jornal Brasileiro De Pneumologia : Publicacao Oficial Da Sociedade Brasileira De Pneumologia E Tisilogia, 34(2), pp. 107-17.
Dalcin Pde T, Abreu E Silva FA. Cystic Fibrosis in Adults: Diagnostic and Therapeutic Aspects. J Bras Pneumol. 2008;34(2):107-17. PubMed PMID: 18345455.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cystic fibrosis in adults: diagnostic and therapeutic aspects. AU - Dalcin,Paulo de Tarso Roth, AU - Abreu E Silva,Fernando Antônio de, PY - 2007/07/29/received PY - 2007/08/28/accepted PY - 2008/3/18/pubmed PY - 2008/9/23/medline PY - 2008/3/18/entrez SP - 107 EP - 17 JF - Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia JO - J Bras Pneumol VL - 34 IS - 2 N2 - Once considered a childhood disease, cystic fibrosis is now also a disease of adults. Increased longevity has resulted in the aging of the cystic fibrosis population. The consequent age-related medical problems among adults with cystic fibrosis have increased medical care needs. These needs are being met by a growing number of non-pediatric pulmonologists and other non-pediatric specialists. The objective of this review was to summarize the current knowledge about diagnosis and treatment in adult cystic fibrosis. In most cases, the diagnosis is suggested by manifestations of chronic sinopulmonary disease and exocrine pancreatic insufficiency. The diagnosis is confirmed by a positive sweat test result. Adult patients may, however, present pancreatic sufficiency and atypical clinical features, sometimes in combination with normal or borderline sweat test results. In such cases, identifying cystic fibrosis mutations and measuring nasal potential difference can have diagnostic utility. The standard therapeutic approach to pulmonary disease includes the use of antibiotics, airway clearance, exercise, mucolytics, bronchodilators, oxygen therapy, anti-inflammatory agents and nutritional support. Appropriate application of these therapies results in most cystic fibrosis patients surviving into adulthood with an acceptable quality of life. SN - 1806-3756 UR - https://www.unboundmedicine.com/medline/citation/18345455/Cystic_fibrosis_in_adults:_diagnostic_and_therapeutic_aspects_ L2 - http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132008000200008&lng=en&nrm=iso&tlng=en DB - PRIME DP - Unbound Medicine ER -