[Controversies in cystic fibrosis--from pediatrician to specialist].
OBJECTIVE
Cystic fibrosis has become, in the last 70 years, the most important potentially fatal inherited disease that affects white individuals around the world. Although it is considered a genetic disorder, which strikes cells of different organs, not all patients present similar clinical response. Many clinical manifestations, mainly pulmonary and gastrointestinal, may develop in cystic fibrosis patients. The aim of this article is to offer pediatricians an updated review of the controversies and recent advances in the treatment of cystic fibrosis.


METHODS
Systematic review in the Medline database.


RESULTS
Seventy-nine articles about cystic fibrosis published in international journals were reviewed. This article presents an updated and critical review of the main events related to the incidence, pathophysiology, diagnosis and treatment of cystic fibrosis.


CONCLUSIONS
Even though no treatment is available for this disease, new findings about its etiology and pathophysiology have been discovered in the last two decades, improving treatment and survival of cystic fibrosis patients.
Ribeiro
Jose Dirceu
JD
Departamento de Pediatria, Universidade Estadual de Campinas, Campinas, SP. dirceu@head.fcm.unicamp.br
Ribeiro
Maria Angela G de O
MA
Ribeiro
Antonio Fernando
AF