Abstract

CARDOSO, Kátia; PEREIRA, Luísa  and  BARRETO, Celeste. Cystic fibrosis: related bone disease. Nascer e Crescer [online]. 2013, vol.22, n.4, pp.227-233. ISSN 0872-0754.

Introduction: More than seven decades have passed since the first description of cystic fibrosis as a deadly childhood disease. Since then, progress has been made to extend the lives of these patients. As a consequence, children with cystic fibrosis are living into adulthood, which was once an unexpected outcome. Nevertheless, this increased survival brought new secondary complications as well as problems caused by the long-lasting medication. Cystic fibrosis-related bone disease is a recent but common complication in long-term survivors. Since its first description in 1979, a large number of papers have been published concerning the various areas of research around this secondary complication of cystic fibrosis. Aims: This article attempts to review the literature related to several research areas around the cystic fibrosis-related bone disease summarizing the most relevant aspects of this problem. Development: Throughout the article the authors emphasize knowledge about the epidemiology, pathophysiology and clinical aspects, and describe the most current recommendations for the diagnosis, prevention, treatment and monitoring of cystic fibrosis-related bone disease. Conclusions: Multifactorial aetiology and interdependence and complexity of underlying pathological mechanisms stress the need and importance of monitoring patients with cystic fibrosis-related bone disease in specialized centers that allow a multi- and interdisciplinary integrated and differentiated follow-up.

Keywords : Children; cystic fibrosis related bone disease.

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