Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 2001 Jun;84(6):516–520. doi: 10.1136/adc.84.6.516

Bone status in cystic fibrosis

M Sood 1, G Hambleton 1, M Super 1, W Fraser 1, J Adams 1, M Mughal 1
PMCID: PMC1718803  PMID: 11369574

Abstract

AIM—To investigate bone mineral status of children with cystic fibrosis (CF).
METHODS—In 29 children with CF and 49 matched controls, bone mineral content (BMC), projected bone area (BA), and areal bone mineral density (BMD) of the whole body, total hip, and lumbar spine (L1-L4) were measured using dual energy x ray absorptiometry. The BMC values at each site were adjusted for BA, height, and weight. At the lumbar spine, the bone mineral apparent density (BMAD) was calculated by dividing the BMC by the estimated volume, derived from BA. Vertebral (T12-L3) trabecular bone mineral density (vTBMD) was measured using quantitative computed tomography in children with CF. Calcaneal broadband ultrasound attenuation (BUA) was measured in CF patients and controls using quantitative ultrasound. The disease severity of CF children was evaluated by the Shwachman-Kulczycki (SK) score.
RESULTS—The mean BUA, whole body and regional BA, adjusted BMC, and areal BMD of children with CF were not different from those of controls. The mean BMAD of the lumbar spine was reduced in CF patients compared with controls, whereas the mean vTBMD standard deviation scores were significantly higher in CF patients. The median SK score of the CF group was 81 (range 42-100), indicating that as a group our CF patient population had relatively mild disease.
CONCLUSION—The normal vertebral BMC, decreased BMAD, and higher vTBMD suggests that the vertebral cortical thickness or density might be reduced in CF subjects. The overall bone mineral status of CF children with relatively mild disease was not different from size matched controls.



Full Text

The Full Text of this article is available as a PDF (127.8 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. 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]
  2. 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]
  3. 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]
  4. 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]
  5. Carter D. R., Bouxsein M. L., Marcus R. New approaches for interpreting projected bone densitometry data. J Bone Miner Res. 1992 Feb;7(2):137–145. doi: 10.1002/jbmr.5650070204. [DOI] [PubMed] [Google Scholar]
  6. Cole T. J. Growth monitoring with the British 1990 growth reference. Arch Dis Child. 1997 Jan;76(1):47–49. doi: 10.1136/adc.76.1.47. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. 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]
  8. Dodge J. A., Morison S., Lewis P. A., Colest E. C., Geddes D., Russell G., Jackson A. D., Bentley B. Cystic fibrosis in the United Kingdom, 1968-1988: incidence, population and survival. Paediatr Perinat Epidemiol. 1993 Apr;7(2):157–166. doi: 10.1111/j.1365-3016.1993.tb00390.x. [DOI] [PubMed] [Google Scholar]
  9. Duke P. M., Litt I. F., Gross R. T. Adolescents' self-assessment of sexual maturation. Pediatrics. 1980 Dec;66(6):918–920. [PubMed] [Google Scholar]
  10. Faulkner K. G., Glüer C. C., Majumdar S., Lang P., Engelke K., Genant H. K. Noninvasive measurements of bone mass, structure, and strength: current methods and experimental techniques. AJR Am J Roentgenol. 1991 Dec;157(6):1229–1237. doi: 10.2214/ajr.157.6.1950872. [DOI] [PubMed] [Google Scholar]
  11. 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]
  12. Gilsanz V., Roe T. F., Mora S., Costin G., Goodman W. G. Changes in vertebral bone density in black girls and white girls during childhood and puberty. N Engl J Med. 1991 Dec 5;325(23):1597–1600. doi: 10.1056/NEJM199112053252302. [DOI] [PubMed] [Google Scholar]
  13. Glüer C. C., Wu C. Y., Genant H. K. Broadband ultrasound attenuation signals depend on trabecular orientation: an in vitro study. Osteoporos Int. 1993 Jul;3(4):185–191. doi: 10.1007/BF01623674. [DOI] [PubMed] [Google Scholar]
  14. Gärdsell P., Johnell O., Nilsson B. E. The predictive value of bone loss for fragility fractures in women: a longitudinal study over 15 years. Calcif Tissue Int. 1991 Aug;49(2):90–94. doi: 10.1007/BF02565127. [DOI] [PubMed] [Google Scholar]
  15. 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]
  16. Hans D., Dargent-Molina P., Schott A. M., Sebert J. L., Cormier C., Kotzki P. O., Delmas P. D., Pouilles J. M., Breart G., Meunier P. J. Ultrasonographic heel measurements to predict hip fracture in elderly women: the EPIDOS prospective study. Lancet. 1996 Aug 24;348(9026):511–514. doi: 10.1016/s0140-6736(95)11456-4. [DOI] [PubMed] [Google Scholar]
  17. Haworth C. S., Freemont A. J., Webb A. K., Dodd M. E., Selby P. L., Mawer E. B., Adams J. E. Hip fracture and bone histomorphometry in a young adult with cystic fibrosis. Eur Respir J. 1999 Aug;14(2):478–479. doi: 10.1034/j.1399-3003.1999.14b39.x. [DOI] [PubMed] [Google Scholar]
  18. 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]
  19. 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]
  20. Henderson R. C., Specter B. B. Kyphosis and fractures in children and young adults with cystic fibrosis. J Pediatr. 1994 Aug;125(2):208–212. doi: 10.1016/s0022-3476(94)70194-6. [DOI] [PubMed] [Google Scholar]
  21. Iqbal S. J. Vitamin D metabolism and the clinical aspects of measuring metabolites. Ann Clin Biochem. 1994 Mar;31(Pt 2):109–124. doi: 10.1177/000456329403100201. [DOI] [PubMed] [Google Scholar]
  22. Laursen E. M., Molgaard C., Michaelsen K. F., Koch C., Müller J. Bone mineral status in 134 patients with cystic fibrosis. Arch Dis Child. 1999 Sep;81(3):235–240. doi: 10.1136/adc.81.3.235. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Mughal M. Z., Langton C. M., Utretch G., Morrison J., Specker B. L. Comparison between broad-band ultrasound attenuation of the calcaneum and total body bone mineral density in children. Acta Paediatr. 1996 Jun;85(6):663–665. doi: 10.1111/j.1651-2227.1996.tb14119.x. [DOI] [PubMed] [Google Scholar]
  24. Mughal M. Z., Ward K., Qayyum N., Langton C. M. Assessment of bone status using the contact ultrasound bone analyser. Arch Dis Child. 1997 Jun;76(6):535–536. doi: 10.1136/adc.76.6.535. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Mølgaard C., Thomsen B. L., Prentice A., Cole T. J., Michaelsen K. F. Whole body bone mineral content in healthy children and adolescents. Arch Dis Child. 1997 Jan;76(1):9–15. doi: 10.1136/adc.76.1.9. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. 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]
  27. 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]
  28. Salamoni F., Roulet M., Gudinchet F., Pilet M., Thiébaud D., Burckhardt P. Bone mineral content in cystic fibrosis patients: correlation with fat-free mass. Arch Dis Child. 1996 Apr;74(4):314–318. doi: 10.1136/adc.74.4.314. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. 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]
  30. Slemenda C. W., Miller J. Z., Hui S. L., Reister T. K., Johnston C. C., Jr Role of physical activity in the development of skeletal mass in children. J Bone Miner Res. 1991 Nov;6(11):1227–1233. doi: 10.1002/jbmr.5650061113. [DOI] [PubMed] [Google Scholar]
  31. Solomons N. W., Wagonfeld J. B., Rieger C., Jacob R. A., Bolt M., Horst J. V., Rothberg R., Sandstead H. Some biochemical indices of nutrition in treated cystic fibrosis patients. Am J Clin Nutr. 1981 Apr;34(4):462–474. doi: 10.1093/ajcn/34.4.462. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES