Abstract
BACKGROUND—Patients with cystic fibrosis (CF) have many risk factors for reduced bone mineral density (BMD). The aim of this study was to determine the prevalence of osteoporosis and osteopenia in a large cross section of patients and to identify risk factors. METHODS—All patients attending the regional centre were invited to participate in the study. Bone mineral density was measured at the lumbar spine, femoral neck, and for total body with a Lunar DPX-L densitometer. Multiple indices of disease severity were investigated, and liver and thyroid function, blood calcium, phosphate, 25-OH vitamin D, follicle stimulating and luteinising hormone, oestradiol, and testosterone levels were measured. Patients completed a four day prospective dietary diary. Exercise was assessed by a seven day activity recall questionnaire. Sexual development and treatment histories were obtained. The relationship between all these variables and BMD measurements was analysed. RESULTS—Sixty six percent of 114 patients assessed had osteopenia or osteoporosis. The Shwachman-Kulczycki (SK) clinical score (higher score = less severe disease) correlated significantly with BMD at the lumbar spine and femoral neck, and with total body BMD (p<0.001). There was a predicted increase of 0.0032 g/cm2 in lumbar spine BMD for every unit increase in the SK score. Oral steroid use was significantly associated with reduced BMD at the lumbar spine (p = 0.017) and femoral neck (p = 0.027). CONCLUSIONS—Osteopenia and osteoporosis are common findings in a heterogeneous population of adults with CF. Patients at most risk are those with severe disease and those who have used corticosteroids.
Full Text
The Full Text of this article is available as a PDF (159.2 KB).
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Aris R. M., Renner J. B., Winders A. D., Buell H. E., Riggs D. B., Lester G. E., Ontjes D. A. Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis. Ann Intern Med. 1998 Feb 1;128(3):186–193. doi: 10.7326/0003-4819-128-3-199802010-00004. [DOI] [PubMed] [Google Scholar]
- Bachrach L. K., Loutit C. W., Moss R. B. Osteopenia in adults with cystic fibrosis. Am J Med. 1994 Jan;96(1):27–34. doi: 10.1016/0002-9343(94)90112-0. [DOI] [PubMed] [Google Scholar]
- Bailey D. A. The Saskatchewan Pediatric Bone Mineral Accrual Study: bone mineral acquisition during the growing years. Int J Sports Med. 1997 Jul;18 (Suppl 3):S191–S194. doi: 10.1055/s-2007-972713. [DOI] [PubMed] [Google Scholar]
- Baroncelli G. I., De Luca F., Magazzú G., Arrigo T., Sferlazzas C., Catena C., Bertelloni S., Saggese G. Bone demineralization in cystic fibrosis: evidence of imbalance between bone formation and degradation. Pediatr Res. 1997 Mar;41(3):397–403. doi: 10.1203/00006450-199703000-00016. [DOI] [PubMed] [Google Scholar]
- Bhudhikanok G. S., Lim J., Marcus R., Harkins A., Moss R. B., Bachrach L. K. Correlates of osteopenia in patients with cystic fibrosis. Pediatrics. 1996 Jan;97(1):103–111. [PubMed] [Google Scholar]
- Bhudhikanok G. S., Wang M. C., Marcus R., Harkins A., Moss R. B., Bachrach L. K. Bone acquisition and loss in children and adults with cystic fibrosis: a longitudinal study. J Pediatr. 1998 Jul;133(1):18–27. doi: 10.1016/s0022-3476(98)70172-6. [DOI] [PubMed] [Google Scholar]
- Boot A. M., de Ridder M. A., Pols H. A., Krenning E. P., de Muinck Keizer-Schrama S. M. Bone mineral density in children and adolescents: relation to puberty, calcium intake, and physical activity. J Clin Endocrinol Metab. 1997 Jan;82(1):57–62. doi: 10.1210/jcem.82.1.3665. [DOI] [PubMed] [Google Scholar]
- Cole T. J., Freeman J. V., Preece M. A. Body mass index reference curves for the UK, 1990. Arch Dis Child. 1995 Jul;73(1):25–29. doi: 10.1136/adc.73.1.25. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Conway S. P., Pond M. N., Bowler I., Smith D. L., Simmonds E. J., Joanes D. N., Hambleton G., Hiller E. J., Stableforth D. E., Weller P. The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores. Thorax. 1994 Sep;49(9):860–862. doi: 10.1136/thx.49.9.860. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Dodge J. A., Morison S., Lewis P. A., Coles E. C., Geddes D., Russell G., Littlewood J. M., Scott M. T. Incidence, population, and survival of cystic fibrosis in the UK, 1968-95. UK Cystic Fibrosis Survey Management Committee. Arch Dis Child. 1997 Dec;77(6):493–496. doi: 10.1136/adc.77.6.493. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Donovan D. S., Jr, Papadopoulos A., Staron R. B., Addesso V., Schulman L., McGregor C., Cosman F., Lindsay R. L., Shane E. Bone mass and vitamin D deficiency in adults with advanced cystic fibrosis lung disease. Am J Respir Crit Care Med. 1998 Jun;157(6 Pt 1):1892–1899. doi: 10.1164/ajrccm.157.6.9712089. [DOI] [PubMed] [Google Scholar]
- Ferrari S. L., Nicod L. P., Hamacher J., Spiliopoulos A., Slosman D. O., Rochat T., Bonjour J. P., Rizzoli R. Osteoporosis in patients undergoing lung transplantation. Eur Respir J. 1996 Nov;9(11):2378–2382. doi: 10.1183/09031936.96.09112378. [DOI] [PubMed] [Google Scholar]
- Frederiksen B., Lanng S., Koch C., Høiby N. Improved survival in the Danish center-treated cystic fibrosis patients: results of aggressive treatment. Pediatr Pulmonol. 1996 Mar;21(3):153–158. doi: 10.1002/(SICI)1099-0496(199603)21:3<153::AID-PPUL1>3.0.CO;2-R. [DOI] [PubMed] [Google Scholar]
- Freeman J. V., Cole T. J., Chinn S., Jones P. R., White E. M., Preece M. A. Cross sectional stature and weight reference curves for the UK, 1990. Arch Dis Child. 1995 Jul;73(1):17–24. doi: 10.1136/adc.73.1.17. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Gibbens D. T., Gilsanz V., Boechat M. I., Dufer D., Carlson M. E., Wang C. I. Osteoporosis in cystic fibrosis. J Pediatr. 1988 Aug;113(2):295–300. doi: 10.1016/s0022-3476(88)80268-3. [DOI] [PubMed] [Google Scholar]
- Glastre C., Braillon P., David L., Cochat P., Meunier P. J., Delmas P. D. Measurement of bone mineral content of the lumbar spine by dual energy x-ray absorptiometry in normal children: correlations with growth parameters. J Clin Endocrinol Metab. 1990 May;70(5):1330–1333. doi: 10.1210/jcem-70-5-1330. [DOI] [PubMed] [Google Scholar]
- Grey A. B., Ames R. W., Matthews R. D., Reid I. R. Bone mineral density and body composition in adult patients with cystic fibrosis. Thorax. 1993 Jun;48(6):589–593. doi: 10.1136/thx.48.6.589. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Hahn T. J., Squires A. E., Halstead L. R., Strominger D. B. Reduced serum 25-hydroxyvitamin D concentration and disordered mineral metabolism in patients with cystic fibrosis. J Pediatr. 1979 Jan;94(1):38–42. doi: 10.1016/s0022-3476(79)80346-7. [DOI] [PubMed] [Google Scholar]
- Haworth C. S., Selby P. L., Webb A. K., Dodd M. E., Musson H., McL Niven R., Economou G., Horrocks A. W., Freemont A. J., Mawer E. B. Low bone mineral density in adults with cystic fibrosis. Thorax. 1999 Nov;54(11):961–967. doi: 10.1136/thx.54.11.961. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Henderson R. C., Madsen C. D. Bone density in children and adolescents with cystic fibrosis. J Pediatr. 1996 Jan;128(1):28–34. doi: 10.1016/s0022-3476(96)70424-9. [DOI] [PubMed] [Google Scholar]
- Henderson R. C., Madsen C. D. Bone mineral content and body composition in children and young adults with cystic fibrosis. Pediatr Pulmonol. 1999 Feb;27(2):80–84. doi: 10.1002/(sici)1099-0496(199902)27:2<80::aid-ppul3>3.0.co;2-j. [DOI] [PubMed] [Google Scholar]
- Hui S. L., Slemenda C. W., Johnston C. C., Jr Age and bone mass as predictors of fracture in a prospective study. J Clin Invest. 1988 Jun;81(6):1804–1809. doi: 10.1172/JCI113523. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Lu P. W., Cowell C. T., LLoyd-Jones S. A., Briody J. N., Howman-Giles R. Volumetric bone mineral density in normal subjects, aged 5-27 years. J Clin Endocrinol Metab. 1996 Apr;81(4):1586–1590. doi: 10.1210/jcem.81.4.8636372. [DOI] [PubMed] [Google Scholar]
- Mischler E. H., Chesney P. J., Chesney R. W., Mazess R. B. Demineralization in cystic fibrosis detected by direct photon absorptiometry. Am J Dis Child. 1979 Jun;133(6):632–635. doi: 10.1001/archpedi.1979.02130060072016. [DOI] [PubMed] [Google Scholar]
- Prentice A., Parsons T. J., Cole T. J. Uncritical use of bone mineral density in absorptiometry may lead to size-related artifacts in the identification of bone mineral determinants. Am J Clin Nutr. 1994 Dec;60(6):837–842. doi: 10.1093/ajcn/60.6.837. [DOI] [PubMed] [Google Scholar]
- SHWACHMAN H., KULCZYCKI L. L. Long-term study of one hundred five patients with cystic fibrosis; studies made over a five- to fourteen-year period. AMA J Dis Child. 1958 Jul;96(1):6–15. doi: 10.1001/archpedi.1958.02060060008002. [DOI] [PubMed] [Google Scholar]
- Shane E., Silverberg S. J., Donovan D., Papadopoulos A., Staron R. B., Addesso V., Jorgesen B., McGregor C., Schulman L. Osteoporosis in lung transplantation candidates with end-stage pulmonary disease. Am J Med. 1996 Sep;101(3):262–269. doi: 10.1016/S0002-9343(96)00155-6. [DOI] [PubMed] [Google Scholar]
- Shaw N., Bedford C., Heaf D., Carty H., Dutton J. Osteopenia in adults with cystic fibrosis. Am J Med. 1995 Dec;99(6):690–692. doi: 10.1016/s0002-9343(99)80262-9. [DOI] [PubMed] [Google Scholar]
- Thomas M. K., Lloyd-Jones D. M., Thadhani R. I., Shaw A. C., Deraska D. J., Kitch B. T., Vamvakas E. C., Dick I. M., Prince R. L., Finkelstein J. S. Hypovitaminosis D in medical inpatients. N Engl J Med. 1998 Mar 19;338(12):777–783. doi: 10.1056/NEJM199803193381201. [DOI] [PubMed] [Google Scholar]
- Truscott J. G., Oldroyd B., Simpson M., Stewart S. P., Westmacott C. F., Milner R., Horsman A., Smith M. A. Variation in lumbar spine and femoral neck bone mineral measured by dual energy X-ray absorption: a study of 329 normal women. Br J Radiol. 1993 Jun;66(786):514–521. doi: 10.1259/0007-1285-66-786-514. [DOI] [PubMed] [Google Scholar]
- Wilson P. W., Paffenbarger R. S., Jr, Morris J. N., Havlik R. J. Assessment methods for physical activity and physical fitness in population studies: report of a NHLBI workshop. Am Heart J. 1986 Jun;111(6):1177–1192. doi: 10.1016/0002-8703(86)90022-0. [DOI] [PubMed] [Google Scholar]