Cystic fibrosis-related bone disease: insights into a growing problem.
Curr Opin Endocrinol Diabetes Obes
; 20(6): 547-52, 2013 Dec.
Article
en En
| MEDLINE
| ID: mdl-24468756
ABSTRACT
PURPOSE OF REVIEW This review will describe the clinical significance, pathogenesis and treatment of cystic fibrosis related bone disease (CFBD). RECENT FINDINGS:
CFBD continues to increase as the life expectancy of individuals with cystic fibrosis increases. According to clinical guidelines, individuals with cystic fibrosis should be initially screened at the age of 18 years via dual-energy x-ray absorptiometry, if not done so previously. The underlying pathogenesis of CFBD appears to be multifactorial, but increasing data imply a direct impact by the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR deficiency and/or dysfunction impair osteoblast activity and differentiation, and indirectly promote osteoclast formation. Unfortunately, once diagnosed with CFBD, few cystic fibrosis tested medical therapies exist.SUMMARY:
CFBD is an increasingly recognized complication that has a significant impact on the overall health of the individual. Recommendations to identify patients with cystic fibrosis who are at risk for fracture using dual-energy x-ray absorptiometry have been established. Therapeutic agents directly studied in patients with cystic fibrosis are limited to bisphosphonates, although other potential treatment agents exist. Finally, an improved understanding of the pathologic mechanisms will aid in the study and development of therapies.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Deficiencia de Vitamina D
/
Enfermedades Óseas
/
Tamizaje Masivo
/
Regulador de Conductancia de Transmembrana de Fibrosis Quística
/
Fibrosis Quística
/
Fracturas Óseas
Tipo de estudio:
Guideline
/
Prognostic_studies
/
Screening_studies
Aspecto:
Patient_preference
Límite:
Female
/
Humans
/
Male
Idioma:
En
Revista:
Curr Opin Endocrinol Diabetes Obes
Asunto de la revista:
ENDOCRINOLOGIA
/
METABOLISMO
Año:
2013
Tipo del documento:
Article
País de afiliación:
Estados Unidos