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Paediatr Respir Rev. 2009 Sep;10(3):134-42. doi: 10.1016/j.prrv.2009.05.001. Epub 2009 Jul 17.

Update on cystic fibrosis-related bone disease: a special focus on children.

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


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.

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