• Kátia Cardoso S. de Pediatria Médica, CH Lisboa Norte, Hospital de Santa Maria
  • Luía Pereira Centro Especializado de Fibrose Quística, CH Lisboa Norte, H Santa Maria; Clínica Universitária de Pediatria, Faculdade de Medicina, Universidade de Lisboa
  • Celeste Barreto Centro Especializado de Fibrose Quística, CH Lisboa Norte, H Santa Maria; Clínica Universitária de Pediatria, Faculdade de Medicina, Universidade de Lisboa



Children, cystic fibrosis related bone disease


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.


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How to Cite

Cardoso K, Pereira L, Barreto C. CYSTIC FIBROSIS -RELATED BONE DISEASE. REVNEC [Internet]. 2016Sep.5 [cited 2022Dec.5];22(4):227-33. Available from:



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