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Volume 10, Issue 3, Pages 134-142 (September 2009)


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Update on Cystic Fibrosis-Related Bone Disease: A Special Focus on Children

Isabelle Sermet-Gaudelus1, Mireille Castanet2, George Retsch-Bogart3, Robert M. Aris4Corresponding Author Informationemail address

Summary 

A high prevalence of low bone mineralization is documented in adult patients with cystic fibrosis (CF). Osteopenia is present in up to 85% of adult patients and osteoporosis in 10% to 34%. In children, study results are discordant probably because of comparisons to different control populations and corrections for bone size in growing children. Malnutrition, inflammation, vitamin D and vitamin K deficiency, altered sex hormone production, glucocorticoid therapy, and physical inactivity are well known risk factors for poor bone health. Puberty is a critical period for bone mineralization and requires a careful follow-up to achieve optimal bone peak mass. Strategies for optimizing bone health, such as monitoring bone mineral density (BMD) and providing preventive care are necessary from childhood through adolescence to minimize CF-related bone disease in adult CF patients.

1 Centre de Référence et de Compétence en Mucoviscidose, Hopital Necker-Enfants Malades, INSERM U 845, Université René Descartes, Paris, France

2 Service d’Endocrinologie Pédiatrique, Hopital Necker-Enfants Malades, INSERM U 845, Université René Descartes, Paris, France

3 Division of Pulmonary Medicine, Department of Pediatrics, University of North Carolina, Chapel Hill, NC, USA

4 Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of North Carolina, Chapel Hill, NC, USA

Corresponding Author InformationCorresponding author.

PII: S1526-0542(09)00040-2

doi:10.1016/j.prrv.2009.05.001


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