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Bone health and disease in cystic fibrosis.
Paediatr Respir Rev 2016; 20 Suppl:2-5PR

Abstract

Low bone mineral density is common in children and adults with CF. It has a multifactorial aetiology that includes direct effects of CFTR dysfunction on bone cell activity, as well as the secondary effects of CFTR dysfunction including pancreatic insufficiency (leading to malnutrition/malabsorption of fat soluble vitamins) and pulmonary infection (leading to systemic inflammation and increased bone resorption). Strategies to improve bone health in CF include optimising general CF nutritional and pulmonary care and the judicious use of bisphosphonates in selected patients. CFTR correctors/potentiators may have positive impact on bone metabolism in people with CF.

Authors+Show Affiliations

Adult Cystic Fibrosis Centre, Papworth Hospital, Cambridge, UK.Adult Cystic Fibrosis Centre, Papworth Hospital, Cambridge, UK. Electronic address: charles.haworth@papworth.nhs.uk.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

27461283

Citation

Marquette, Malcolm, and Charles S. Haworth. "Bone Health and Disease in Cystic Fibrosis." Paediatric Respiratory Reviews, vol. 20 Suppl, 2016, pp. 2-5.
Marquette M, Haworth CS. Bone health and disease in cystic fibrosis. Paediatr Respir Rev. 2016;20 Suppl:2-5.
Marquette, M., & Haworth, C. S. (2016). Bone health and disease in cystic fibrosis. Paediatric Respiratory Reviews, 20 Suppl, pp. 2-5. doi:10.1016/j.prrv.2016.06.003.
Marquette M, Haworth CS. Bone Health and Disease in Cystic Fibrosis. Paediatr Respir Rev. 2016;20 Suppl:2-5. PubMed PMID: 27461283.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Bone health and disease in cystic fibrosis. AU - Marquette,Malcolm, AU - Haworth,Charles S, Y1 - 2016/06/21/ PY - 2016/7/28/entrez PY - 2016/7/28/pubmed PY - 2017/3/24/medline KW - Bone KW - Cystic fibrosis KW - Osteomalacia KW - Osteoporosis SP - 2 EP - 5 JF - Paediatric respiratory reviews JO - Paediatr Respir Rev VL - 20 Suppl N2 - Low bone mineral density is common in children and adults with CF. It has a multifactorial aetiology that includes direct effects of CFTR dysfunction on bone cell activity, as well as the secondary effects of CFTR dysfunction including pancreatic insufficiency (leading to malnutrition/malabsorption of fat soluble vitamins) and pulmonary infection (leading to systemic inflammation and increased bone resorption). Strategies to improve bone health in CF include optimising general CF nutritional and pulmonary care and the judicious use of bisphosphonates in selected patients. CFTR correctors/potentiators may have positive impact on bone metabolism in people with CF. SN - 1526-0550 UR - https://www.unboundmedicine.com/medline/citation/27461283/Bone_health_and_disease_in_cystic_fibrosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1526-0542(16)30040-9 DB - PRIME DP - Unbound Medicine ER -