title: [Insights into cystic fibrosis-related bone disease].
abstract: With the increasing life expectancy of patients with cystic fibrosis (CF), prevalence of late complications such as CF-related bone disease (CFBD) has increased. It was initially described in 24% of the adult population with CF and has also been reported in the pediatric population. CFBD is multifactorial and progresses in different steps. Both decreased bone formation and increased bone resorption (in different amounts) are observed. CFBD is likely primitive (directly related to the CFTR defect itself), but is also worsened by acquired secondary factors such as lung infections, chronic inflammation, denutrition, vitamin deficiency, and decreased physical activity. CFBD may be clinically apparent (i.e., mainly vertebral and costal fractures), or clinically asymptomatic (therefore corresponding to abnormalities in bone density and architecture). CFBD management mainly aims to prevent the occurrence of fractures. Prevention and regular monitoring of bone disease as early as 8 years of age is of the utmost importance, as is the control of possible secondary deleterious CFBD factors. New radiological tools, such as high-resolution peripheral quantitative computed tomography, allow an accurate evaluation of cortical and trabecular bone micro-architecture in addition to compartmental density; as such, they will likely improve the assessment of the bone fracture threat in CF patients in the near future.
authors.affiliation: Université Claude-Bernard Lyon 1, 43, boulevard du 11-Novembre-1918, 69100 Villeurbanne, France.
authors.forename: C
authors.initials: C
authors.lastname: Braun
authors.affiliation: Université Claude-Bernard Lyon 1, 43, boulevard du 11-Novembre-1918, 69100 Villeurbanne, France; Centre de référence des maladies rénales rares, hôpital femme mère enfant, 69500 Bron, France; Inserm 1033 LYOS, prévention des maladies osseuses, 69008 Lyon, France.
authors.forename: J
authors.initials: J
authors.lastname: Bacchetta
authors.affiliation: Université Claude-Bernard Lyon 1, 43, boulevard du 11-Novembre-1918, 69100 Villeurbanne, France; Centre de ressources et de compétences de la mucoviscidose, hôpital femme mère enfant, 69500 Bron, France; UMR 5558, équipe EMET, 43, boulevard du 11-Novembre-1918, 69100 Villeurbanne, France. Electronic address: philippe.reix@chu-lyon.fr.
authors.forename: P
authors.initials: P
authors.lastname: Reix
mesh_terms: D015502:Absorptiometry, Photon; D015519:Bone Density; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D001847:Bone Diseases / Q000188:drug therapy / Q000209:etiology*; D016723:Bone Remodeling / Q000502:physiology; D002136:Calcium, Dietary / Q000008:administration & dosage; D003550:Cystic Fibrosis / Q000150:complications*; D004164:Diphosphonates / Q000627:therapeutic use; D015444:Exercise; D050723:Fractures, Bone / Q000517:prevention & control*; D006801:Humans; D010281:Parathyroid Hormone / Q000502:physiology; D053245:RANK Ligand / Q000502:physiology; D019379:Teriparatide / Q000627:therapeutic use; D014807:Vitamin D / Q000502:physiology / Q000627:therapeutic use
chemical_list: D050071:Bone Density Conservation Agents; D002136:Calcium, Dietary; D004164:Diphosphonates; D010281:Parathyroid Hormone; D053245:RANK Ligand; D019379:Teriparatide; D014807:Vitamin D