Background: Low bone mineral density (BMD) is prevalent in adults with cystic fibrosis. To identify appropriate therapeutic strategies and the optimal time for intervention, it is necessary to document the natural history of cystic fibrosis related low BMD. Methods: 114 adults with cystic fibrosis underwent bone densitometry a median (25-75% interquartile range) of 12 (12-13) months after initial assessment of bone density. BMD was measured in the lumbar spine, femoral neck, total hip, and distal forearm on recruitment to the trial and at follow up. Results: In patients less than or equal to24 years of age (n=55, mean (SD) age 19.5 (2.6) years) in whom an annual increase in BMD would normally be expected, BMD increased by a mean (95% Cl) 2.9% (1.6 to 4.2) per year in the distal forearm (p<0.001), but decreased by 2.5% (95% Cl-3.8 to -1.2) per year in the femoral neck (p<0.001) and by 2.2% (95% Cl-3.3 to -1.0) per year in the total hip (p<0.001). In patients greater than or equal to25 years of age (n=59, mean (SD) age 30.3 (5.4) years) in whom no annual change in BMD would normally be expected, BMD decreased by 1.9% (95% Cl-2.9 to -0.8) per year in the femoral neck (p<0.001), by 1.5% (95% Cl-2.4 to -0.6) per year in the total hip (p=0.001), and by 0.8% (95% Cl-1.5 to -0.1) per year in the distal forearm (p=0.026). There was no significant annual change in lumbar spine BMD in either patient cohort. Conclusions: Reduced rates of bone accretion and accelerated rates of bone loss explain the high prevalence of low BMD in adults with cystic fibrosis.
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Massachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Boston Childrens Hosp, Div Endocrinol, Boston, MA USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Putman, Melissa S.
Baker, Joshua F.
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Univ Penn, Div Rheumatol, Philadelphia, PA 19104 USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Baker, Joshua F.
Uluer, Ahmet
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Boston Childrens Hosp, Div Resp Dis, Boston, MA USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Uluer, Ahmet
Herlyn, Karen
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Univ Hosp Schleswig Holstein, Poliklin Rheumatol, Campus Luebeck, GermanyMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Herlyn, Karen
Lapey, Allen
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Massachusetts Gen Hosp, Dept Pediat, Div Pulm, Boston, MA 02114 USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Lapey, Allen
Sicilian, Leonard
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Massachusetts Gen Hosp, Dept Med, Div Pulm, Boston, MA 02114 USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Sicilian, Leonard
Tillotson, Angela Pizzo
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Mercy Hosp, Mattina R Proctor Diabet Ctr, Portland, ME USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Tillotson, Angela Pizzo
Gordon, Catherine M.
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Boston Childrens Hosp, Div Endocrinol, Boston, MA USA
Hasbro Childrens Hosp, Div Adolescent Med & Endocrinol, Providence, RI USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Gordon, Catherine M.
Merkel, Peter A.
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Univ Penn, Div Rheumatol, Philadelphia, PA 19104 USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
Merkel, Peter A.
Finkelstein, Joel S.
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Massachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USAMassachusetts Gen Hosp, Endocrine Unit, Dept Med, Boston, MA 02114 USA
机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Haworth, CS
Selby, PL
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Selby, PL
Webb, AK
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Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, EnglandWythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Webb, AK
Dodd, ME
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Dodd, ME
Musson, H
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Musson, H
Niven, RM
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Niven, RM
Economou, G
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Economou, G
Horrocks, AW
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Horrocks, AW
Freemont, AJ
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Freemont, AJ
Mawer, EB
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Mawer, EB
Adams, JE
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机构:Wythenshawe Hosp, Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
机构:Wythenshawe Hosp, S Manchester Univ Hosp NHS Trust, Manchester Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Haworth, CS
Selby, PL
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机构:Wythenshawe Hosp, S Manchester Univ Hosp NHS Trust, Manchester Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Selby, PL
Adams, JE
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机构:Wythenshawe Hosp, S Manchester Univ Hosp NHS Trust, Manchester Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Adams, JE
Mawer, EB
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机构:Wythenshawe Hosp, S Manchester Univ Hosp NHS Trust, Manchester Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Mawer, EB
Horrocks, AW
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机构:Wythenshawe Hosp, S Manchester Univ Hosp NHS Trust, Manchester Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England
Horrocks, AW
Webb, AK
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Wythenshawe Hosp, S Manchester Univ Hosp NHS Trust, Manchester Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, EnglandWythenshawe Hosp, S Manchester Univ Hosp NHS Trust, Manchester Adult Cyst Fibrosis Unit, Manchester M23 9LT, Lancs, England