Cystic fibrosis-related bone disease.
OBJECTIVE
This review highlights recently published data on the pathophysiology, guidelines and treatment of cystic fibrosis (CF)-related bone disease.


RESULTS
The exact role of the cystic fibrosis transmembrane conductance regulator (CFTR), specifically the ΔF508 allele, has been investigated in F508del-CFTR homozygous mice and the F508del-CFTR mutation may contribute to CF-related bone disease by slowing new bone formation. The European Cystic Fibrosis Society has issued guidelines for bone mineral density assessment, management of low-trauma fractures and bisphosphonate therapy. A systematic review based on meta-analyses reports that oral and intravenous bisphosphonates both improve bone mineral density in CF patients, but no data are available concerning the reduction of low-trauma fractures.


CONCLUSIONS
European Cystic Fibrosis Society guidelines may help physicians to improve the management of CF-related bone disease.
aDepartment of Rheumatology bINSERM U1088 'Pathophysiological mechanisms and consequences of cardiovascular calcification: role of cardiovascular and bone remodelling', Avenue René Laennec, University hospital of Amiens, Amiens cPMOI EA 4490, IFR 114, F-59000 Lille dDépartement Universitaire de Rhumatologie, Hôpital Roger-Salengro, CHU Lille, 59037 Lille cedex, France.
Julien
J
Paccou
Patrice
P
Fardellone
Bernard
B
Cortet
D000818:Animals; D015519:Bone Density / Q000187:drug effects* / Q000235:genetics; D050071:Bone Density Conservation Agents / Q000627:therapeutic use*; D001847:Bone Diseases / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology* / Q000517:prevention & control*; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004164:Diphosphonates / Q000627:therapeutic use*; D005260:Female; D050723:Fractures, Bone / Q000503:physiopathology / Q000517:prevention & control; D006801:Humans; D008297:Male; D051379:Mice; D018181:Mice, Inbred CFTR; D009154:Mutation / Q000235:genetics; D017410:Practice Guidelines as Topic; D012307:Risk Factors; D014807:Vitamin D / Q000627:therapeutic use*
D050071:Bone Density Conservation Agents; C505032:CFTR protein, human; D004164:Diphosphonates; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D014807:Vitamin D