A Roadmap to the Brittle Bones of Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive disorder which despite advances in medical care continues to be a life-limiting and often fatal disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. Unlike the osteoporosis seen in postmenopa...

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Main Authors: Ashwini P. Gore, Soon Ho Kwon, Antine E. Stenbit
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Journal of Osteoporosis
Online Access:http://dx.doi.org/10.4061/2011/926045
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author Ashwini P. Gore
Soon Ho Kwon
Antine E. Stenbit
author_facet Ashwini P. Gore
Soon Ho Kwon
Antine E. Stenbit
author_sort Ashwini P. Gore
collection DOAJ
description Cystic fibrosis (CF) is an autosomal recessive disorder which despite advances in medical care continues to be a life-limiting and often fatal disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. Unlike the osteoporosis seen in postmenopausal population, bone disease in CF begins at a young age and is associated with significant morbidity due to fractures, kyphosis, increased pain, and decreased lung function. The maintenance of bone health is essential for the CF population during their lives to prevent pain and fractures but also as they approach lung transplantation since severe bone disease can lead to exclusion from lung transplantation. Early recognition, prevention, and treatment are key to maintaining optimal bone health in CF patients and often require a multidisciplinary approach. This article will review the pathophysiology, current clinical practice guidelines, and potential future therapies for treating CF-related bone disease.
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series Journal of Osteoporosis
spelling doaj-art-0a52abac1daa4a4cb1d8770055588afc2025-02-03T01:12:04ZengWileyJournal of Osteoporosis2042-00642011-01-01201110.4061/2011/926045926045A Roadmap to the Brittle Bones of Cystic FibrosisAshwini P. Gore0Soon Ho Kwon1Antine E. Stenbit2Division of Endocrinology, Diabetes & Medical Genetics, Department of Medicine, Medical University of South Carolina, Charleston, SC 29425-6300, USADivision of Endocrinology, Diabetes & Medical Genetics, Department of Medicine, Medical University of South Carolina, Charleston, SC 29425-6300, USADivision of Pulmonary, Critical Care, Allergy & Sleep Medicine, Department of Medicine, Medical University of South Carolina, Charleston, SC 29425-6300, USACystic fibrosis (CF) is an autosomal recessive disorder which despite advances in medical care continues to be a life-limiting and often fatal disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. Unlike the osteoporosis seen in postmenopausal population, bone disease in CF begins at a young age and is associated with significant morbidity due to fractures, kyphosis, increased pain, and decreased lung function. The maintenance of bone health is essential for the CF population during their lives to prevent pain and fractures but also as they approach lung transplantation since severe bone disease can lead to exclusion from lung transplantation. Early recognition, prevention, and treatment are key to maintaining optimal bone health in CF patients and often require a multidisciplinary approach. This article will review the pathophysiology, current clinical practice guidelines, and potential future therapies for treating CF-related bone disease.http://dx.doi.org/10.4061/2011/926045
spellingShingle Ashwini P. Gore
Soon Ho Kwon
Antine E. Stenbit
A Roadmap to the Brittle Bones of Cystic Fibrosis
Journal of Osteoporosis
title A Roadmap to the Brittle Bones of Cystic Fibrosis
title_full A Roadmap to the Brittle Bones of Cystic Fibrosis
title_fullStr A Roadmap to the Brittle Bones of Cystic Fibrosis
title_full_unstemmed A Roadmap to the Brittle Bones of Cystic Fibrosis
title_short A Roadmap to the Brittle Bones of Cystic Fibrosis
title_sort roadmap to the brittle bones of cystic fibrosis
url http://dx.doi.org/10.4061/2011/926045
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