'Old' bones in young bodies: the tale of cystic fibrosis.
OBJECTIVE
Cystic fibrosis (CF) is the most common genetic disease within the white population and leads to premature respiratory failure. Approximately, 60 000 individuals are currently living with CF in North America and Europe, almost half of whom are adults.


RESULTS
Dozens of studies across the globe indicate that CF adults have low bone density and increased rates of fractures. This genesis of the problem appears to be in late childhood to adolescence.


CONCLUSIONS
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 serum levels of proinflammatory, bone-active cytokines, malnutrition and low body weight, physical inactivity and glucocorticoid therapy) for poor bone health. This review will address the pathogenesis, diagnosis and treatment of bone disease in CF. It will also discuss best practice guidelines for optimizing bone health in patients with CF.
Sparks
Amy A
AA
Division of Pulmonary and Critical Care Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA.
McGee
Sarah J
SJ
Boone
Cheryl E
CE
Neuringer
Isabel P
IP
Jones
Sarah K
SK
Aris
Robert M
RM
D000328:Adult; D015519:Bone Density; D001849:Bone Diseases, Endocrine / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D003550:Cystic Fibrosis / Q000150:complications* / Q000503:physiopathology; D050723:Fractures, Bone / Q000209:etiology; D006801:Humans; D017410:Practice Guidelines as Topic; D055815:Young Adult