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Guide to Bone Health and Disease in Cystic Fibrosis

Pulmonary Medicine and Cystic Fibrosis/Pulmonary Research and Treatment Center (R.M.A.), University of North Carolina, Chapel Hill, North Carolina 27599; Divisions of Rheumatology and Endocrinology (P.A.M., M.F.H.), Boston University, Boston, Massachusetts 02118; Pediatric Endocrinology (L.K.B.), Stanford University, Palo Alto, California 94304; Pediatric Gastroenterology (D.S.B.), State University of New York, Buffalo, New York 14214; Division of Pulmonary and Critical Care Medicine (M.P.B.) and Center for Human Nutrition (K.O.), Johns Hopkins University, Baltimore, Maryland 21205; Endocrinology Division (S.L.E.), Royal Brompton CF Unit, London SW3 6NP, United Kingdom; Division of Endocrinology (T.A.G.), University of Virginia, Charlottesville, Virginia 22908; Pediatric Endocrinology Division (D.S.H.), University of Texas, Dallas, Texas 75390; Manchester Adult CF Unit (C.S.H.), Manchester M13 9WL, United Kingdom; Divisions of Endocrinology and Pulmonary Medicine (P.M.J., N.B.W.), University of Cincinnati, Cincinnati, Ohio 45267; Division of Respirology (E.T.), University of Toronto, Toronto, Canada M5B 1W8; and Cystic Fibrosis Foundation (T.B.W.), Bethesda, Maryland 20814

Address all correspondence and requests for reprints to: Dr. Robert Aris, CB 7020, 4131 Bioinformatics, University of North Carolina, Chapel Hill, North Carolina 27599-7524. E-mail: aris{at}med.unc.edu.

Cystic fibrosis (CF) is the most common genetic disease within the Caucasian population and leads to premature respiratory failure. Approximately 60,000 individuals are currently living with CF in North America and Europe, 40% of whom are adults. The life span of these patients has increased from approximately 2 to 32 yr of age over the last three decades. Bone disease has emerged as a common complication in long-term survivors of CF. Some studies have observed that 50–75% of adults have low bone density and increased rates of fractures. Prevention and treatment of CF-related bone disease must address the myriad risk factors (decreased absorption of fat-soluble vitamins due to pancreatic insufficiency, altered sex hormone production, chronic lung infection with increased levels of bone-active cytokines, physical inactivity, and glucocorticoid therapy) for poor bone health. This review is a condensed and updated summary of the Guide to Bone Health and Disease in Cystic Fibrosis: A Consensus Conference, a statement that evolved from a meeting convened by the Cystic Fibrosis Foundation in May 2002 to address the pathogenesis, diagnosis, and treatment of bone disease in CF. The goal of this conference was to develop practice guidelines for optimizing bone health in patients with CF.

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