Clin Osteol 2006; 11(4): 104-110

Bone disease in cystic fibrosisReview articles

E. Očenášková, H. Vaníček

With prolonged survival of cystic fibrosis patients, new problems emerged, such as their bone health. Osteoporosis and its complications may increase their morbidity and mortality and negatively influence their life quality. There is a complex of negative contributing factors, especially unfavourable nutritional status, vitamin deficiencies, inflammation, low physical activity, cystic fibrosis related diabetes mellitus, liver disease, hypogonadism and systemic corticosteroids in late stages. Puberty and adolescence are specific risk periods from this point of view. Assessing bone mineral density in CF, body parameters and maturation should be taken into account. Although the frequency of low bone density in CF might be overestimated, preventive measures are necessary already in childhood.

Keywords: Cystic fibrosis, DXA, bone mineral density, osteoporosis, vitamin D, pQCT

Published: December 11, 2006  Show citation

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Očenášková E, Vaníček H. Bone disease in cystic fibrosis. Osteologický bulletin. 2006;11(4):104-110.
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References

  1. Vávrová V et al. Cystická fibróza. Praha: GRADA Publishing, 2006.
  2. Quinton PM. Chlorid impermeability in cystic fibrosis. Nature 1983;301 Go to original source...
  3. Kerem B, Rommens JM, Buchanan JA, Markiewicz D, Cox TK, Chakravarti A, Buchwald M, Tsui LC. Identification of the cystic fibrosis gene: genetic ana Science 1989;245:1073-1080. Go to original source...
  4. Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL. Identification of the cystic fibrosis gene: clo and characterization of complementary DNA Science 1989;245:1066-1073. Go to original source...
  5. Rommens JM, lannuzzi MC, Kerem B, Drumm ML, Melmer G, Dean M, Roz­ mahel R, Cole JL, Kennedy D, Hidaka N, et al. Identification of the cystic fibro­ sis gene: chromosome walking and jumping. Science 1989;245:1059-65. Go to original source...
  6. Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration 2000;67:117 Go to original source...
  7. Thiagarajah JR, Verkman AS. CFTR pharmacology and its role in intestinal fluid secretion. Curr Opin Pharmacol 2003;3:594-9. Go to original source...
  8. Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus state­ ment. Cystic Fibrosis Foundation Consensus Panel. J Pediatr 1998;132:589-95. Go to original source...
  9. Ahmed N, Corey M, Forstner G, Zielenski J, Tsui LC, Ellis L, Tullis E, Durie P. Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas. Gut 2003;52:1159-1164. Go to original source...
  10. Hahn TJ, Squires AE, Halstead LR, Strominger DB. Reduced serum 25-hydroxyvitamin D concentration and disordered mineral metabolism in patients with cys­ tic fibrosis. J Pediatr 1979;94:38^2. Go to original source...
  11. Mischler EH, Chesney PJ, Chesney RW, Mazess RB. Demineralization in cystic fibrosis detected by direct photon absorptiometry. Am J Dis Child 1979; 133: 632-5. Go to original source...
  12. Buntain HM, Greer RM, Schluter PJ, Wong JC, Batch JA, Potter JM, Lewindon PJ, Powell E, Wainwright CE, Bell SC. Bone mineral density in Australian child­ ren, adolescents and adults with cystic fibrosis: a controlled cross sectional stu Thorax 2004;59:149-55. Go to original source...
  13. Fok J, Brown NE, Zuberbuhler P, Tabak J, Tom M. Low bone mineral density in cystic fibrosis patients. Can J Diet Pract Res 2002;63:192-7. Go to original source...
  14. Greer RM, Buntain HM, Potter JM, Wainwright CE, Wong JC, O'Rourke PK, Francis PW, Bell SC, Batch JA Abnormalities of the PTH-vitamin D axis and bone turnover markers in children, adolescents and adults with cystic fibrosis: comparison with healthy controls. Osteoporos Int 2003;14:404-11. Go to original source...
  15. Ujhelyi R, Treszl A, Vasarhelyi B, Holics K, Toth M, Arato A, Tulassay T, Tulassay Z, Szathmari M. Bone mineral density and bone acquisition in children and young adults with cystic fibrosis: a follow-up study. J Pediatr Gastroenterol Nutr 2004;38:401-6. Go to original source...
  16. Aris RM, Ontjes DA, Buell HE, Blackwood AD, Lark RK, Caminiti M, Brown SA, Renner JB, Chalermskulrat W, Lester GE. Abnormal bone turnover in cystic fibrosis adults. Osteoporos Int 2002;13:151-7. Go to original source...
  17. Aris RM, Merkel PA, Bachrach LK, Borowitz DS, Boyle MP, Elkin SL, Guise TA, Hardin DS, Haworth CS, Holick MF, Joseph PM, O'Brien K, Tullis E, Watts NB, White TB. Consensus Conference Report: Guide to Bone Health and Disease in Cystic Fibrosis. J Clin Endocrinol Metab 2005;90:1888-96. Go to original source...
  18. Baroncelli GI, De Luca F, Magazzu G, Arrigo T, Sferlazzas C, Catena C, Bertelloni S, Saggese G. Bone demineralization in cystic fibrosis: evidence of imbalan­ ce between bone formation and degradation. Pediatr Res 1997;41:397-403. Go to original source...
  19. Elkin SL, Fairney A, Burnett S, Kemp M, Kyd P, Burgess J, Compston JE, Hod­ son ME Vertebral deformities and low bone mineral density in adults with cystic fibrosis: a cross-sectional study. Osteoporos Int 2001;12:366-372. Go to original source...
  20. Haworth CS, Selby PL, Webb AK, Dodd ME, Musson H, McL Niven R, Economou G, Horrocks AW, Freemont AJ, Mawer EB, Adams JE. Low bone mineral density in adults with cystic fibrosis. Thorax 1999;54:961-967. Go to original source...
  21. Rossini M, Del Marco A, Dal Santo F, Gatti D, Braggion C, James G, Adami S. Prevalence and correlates of vertebral fractures in adults with cystic fibrosis 2004;35:771-6.
  22. Schulze KJ, O'Brien KO, Germain-Lee EL, Booth SL, Leonard A, Rosenstein BJ. Calcium kinetics are altered in clinically stable girls with cystic fibrosis. J C Endocrinol Metab 2004;89:3385-91. Go to original source...
  23. Schulze KJ, Cutchins C, Rosenstein BJ, Germain-Lee EL, O'Brien KO. Calcium acquisition rates do not support age-appropriate gains in total body bone mineral content in prepuberty and late puberty in girls with cystic fibrosis. Osteop 2006;17:73140. Go to original source...
  24. Dif F, Marty C, Baudoin C, de Vernejoul MC, Levi G. Severe osteopenia in CFTR-null mice. Bone 2004;35:595-603. Go to original source...
  25. King SJ, Topliss DJ, Kotsimbos T, Nyulasi IB, Bailey M, Ebeling PR, Wilso Reduced bone density in cystic fibrosis: DeltaF508 mutation is an independent risk factor. Eur Respir J 2005;25:54-61. Go to original source...
  26. Aris RM, Guise TA. Cystic fi link? Eur Respir J. 2005;25:9-11 Go to original source...
  27. Vaníček H, Brátová M, Pozler O, Kohout P. Intestinal Permeability in Cystic Fib Patients. Book of Abstracts, 22. European CF Conference, Berlin 1998; PS2-20:85.
  28. Castellani C, Malerba G, Sangalli A, Delmarco A, Petrelli E, Rossini M, Assael BM, Mottes M. The genetic background of osteoporosis in cystic fibrosis: Asso­ ciation analysis with polymorphic markers in four candidate genes. J Cyst Fibros. Publikace na internetu před tištěnou, 16. 5. 2006. Go to original source...
  29. Gibbens DT, Gilsanz V, Boechat MI, Dufer D, Carlson ME, Wang CI.Osteoporosis in cystic fibrosis. J Pediatr 1988;113:295-300. Go to original source...
  30. Marin VB, Velandia S, Hunter B, Gattas V, Fielbaum O, Herrera O, Diaz E. Ener­ gy expenditure, nutrition status, and body composition in children with cystic fi­ brosis. Nutrition 2004;20:181-6. Go to original source...
  31. Ninkovic M, Love SA, Tom B, Alexander GJ, Compston JE. High prevalence of osteoporosis in patients with chronic liver disease prior to liver transplantation. Calcif Tissue Int 2001;69:321-6. Go to original source...
  32. Alex G, Catto-Smith AG, Ditchfield M, Roseby R, Robinson PJ, Cameron FJ, Oli­ ver MR. Is significant cystic fibrosis-related liver disease a risk factor in the de lopment ofbone mineralization abnormalities? Pediatr Pulmonol.2006;41:338-44. Go to original source...
  33. Conway SP, Wolfe SP, Brownlee KG, White H, Oldroyd B, Truscott JG, Harvey JM, Shearer MJ. Vitamin K status among children with cystic fibrosis and its relationship to bone mineral density and bone turnover. Pediatrics 2005; 115: 1325-31. Go to original source...
  34. Rashid M, Durie P, Andrew M, Kalnins D, Shin J, Corey M, Tullis E, Pencharz PB. Prevalence of vitamin K deficiency in cystic fibrosis. Am J Clin Nutr 1999;70:378-82. Go to original source...
  35. Nicolaidou P, Stavrinadis I, Loukou I, Ppadopoulou A, Georgouli H, Douros K, Priftis KN, Gourgiotis D, Matsinos YG, Doudounakis S. The effect of vitamin K supplementation on biochemical markers of bone formation in children and adolescents with cystic fibrosis. Eur J Pediatr 2006;165:540-5. Go to original source...
  36. Chavasse RJ, Francis J, Balfour-Lynn I, Rosenthal M, Bush A. Serum vitamin D levels in children with cystic fibrosis. Pediatr Pulmonol 2004;38:119-22. Go to original source...
  37. Boyle MP, Noschese ML, Watts SL, Davis ME, Stenner SE, Lechtzin N. Failure of high-dose ergocalciferol to correct vitamin D deficiency in adults with cystic fibrosis. Am J Respir Crit Care Med 2005;172:212-7. Go to original source...
  38. Boyle MP, Noschese ML, Watts SL, Davis ME, Lechtzin N. Prevalence of 25-hydroxyvitamin D deficiency in adults with CF and effect of high dose ergocalcife­ rol supplementation. Ped Pulmonol 2003;25:S350.
  39. Back EI, Frindt C, Nohr D, Frank J, Ziebach R, Stern M, Ranke M, Biesalski HK. Antioxidant deficiency in cystic fibrosis: when is the right time to take action? Am J Clin Nutr 2004;80:374-84. Go to original source...
  40. Madarasi A, Lugassi A, Greiner E, Holics K, Biro L, Mozsary E. Antioxidant sta­ tus in patients with cystic fibrosis. Ann Nutr Metab 2000;44:207-11. Go to original source...
  41. Wood LG, Fitzgerald DA, Gibson PG, Cooper, DM, Collins CE, Garg ML. Oxidative stress in cystic fibrosis: dietary and metabolic factors. J Am Coll Nutr 2001;20(2 Suppl):157-65. Go to original source...
  42. Frost HM. Bone's mechanostat: a 2003 update. Anat Rec 2003;275A(2):1081-101. Go to original source...
  43. Frost HM, Schonau E. The "muscle-bone unit" in children and adolescents: a 2000 overview. J Pediatr Endocrinol Metab 2000;13:571-90. Go to original source...
  44. Neu CM, Rauch F, Manz F, Schoenau E. Modeling of cross-sectional bone size, mass and geometry at the proximal radius: a study of normal bone development using peripheral quantitative computed tomography. Osteoporos Int. 2001; 12: 538-47. Go to original source...
  45. Ionescu AA, Evans WD, Pettit RJ, Nixon LS, Stone MD, Shale DJ. Hidden deple­ tion of fat-free mass and bone mineral density in adults with cystic fibrosis. Chest 2003;124:2220-8. Go to original source...
  46. Shead EF, Haworth CS, Gunn E, Bilton D, Scott MA, Compston JE. Osteoclastogenesis during Infective Exacerbations in Ptients with Cystic Fibrosis. Am J Respir Crit Care Med 2006;174:306-11. Go to original source...
  47. Aris RM, Stephens AR, Ontjes DA, Denene Blackwood A, Lark RK, Hensler MB, Neuringer IP, Lester GE. Adverse alterations in bone metabolism are associated with lung infection in adults with cystic fibrosis. Am J Respir Crit Care Med 2000;162:1674-1678. Go to original source...
  48. Haworth CS, Selby PL, Webb AK, Martin L, Elborn JS, Sharples LD, Adams, JE. Inflammatory related changes in bone mineral content in adults with cystic fi sis. Thorax 2004;59:613-7. Go to original source...
  49. Gordon CM, Binello E, Leboff MS, Wohl ME, Rosen CJ, Colin AA Relationship between insulin-like growth factor I, dehydroepiandrosterone sulfate and proresorptive cytokines and bone density in cystic fibrosis. Osteoporos Int 2006;17:783 Go to original source...
  50. Bianchi ML, Romano G, Saraifoger S, Costantini D, Limonta C, Colombo C. BMD and body composition in children and young patients affected by cystic fi­ brosis. J Bone Miner Res 2006;21:388-96. Go to original source...
  51. Moshang T, Holsclaw Child. I980;I34:1139 42.
  52. Landon C, Rosenfeld RG. Short stature and pubertal delay in male adolesce with cystic fibrosis. Androgen treatment. Am J Dis Child 1984;138:388-91. Go to original source...
  53. Stead RJ, Hodson ME, Batten JC, Adams J, Jacobs HS. Amenorrhoea in cystic fibrosis. Clin Endocrinol (Oxf) 1987;26:187-95. Go to original source...
  54. Johannesson M, Landgren BM, Csemiczky G, Hjelte L, Gottlieb C. Female pa­ tients with cystic fibrosis suffer from reproductive endocrinological disorde pite good clinical status. Hum Reprod 1998;13:2092-7. Go to original source...
  55. Leifke E, Friemert M, Heilmann M, Puvogel N, Smaczny C, von zur Muhlen A, Brabant G. Sex steroids and body composition in men with cystic fibrosis. Eur J Endocrinol 2003;148:551-7. Go to original source...
  56. Conway SP, Morton AM, Oldroyd B, Truscott JG, White H, Smith AH, Haigh I. Osteoporosis and osteopenia in adults and adolescents lence and associated factors. Thorax 2000;55:798-804. Go to original source...
  57. Shane E, Silverberg SJ, Donovan D, Ihpadopoulos A, Staron RB, Addesso V, gesen B, McGregor C, Schulman L. Osteoporosis in lung transplantation candi­ dates with end-stage pulmonary disease. Am J Med 1996;101:262-9. Go to original source...
  58. Shane E, Papadopoulos A, Staron RB, Addesso V, Donovan D, McGregor C, Schulman LL. Bone loss and fracture after lung transplantation. Transplantat 1999;68:220-7. Go to original source...
  59. Elkin SL, Vedi S, Bord S, Garrahan NJ, Hodson ME, Compston JE. Histomorphometric analysis ofbone biopsies from the iliac crest of adults with cystic sis. Am J Respir Crit Care Med 2002;166:1470^. Go to original source...
  60. Haworth CS, Webb AK, Egan JJ, Selby PL, Hasleton PS, Bishop PW, Freemont TJ. Bone histomorphometry in adult patients with cystic fibrosis. CHEST 2000; 118:434 439. Go to original source...
  61. Bayer M, Kutílek Š, Feber J, Gut J. Metabolická onemocnění skeletu u dět ha: GRADA Publishing, 2002.
  62. Aris RM, Renner JB, Winders AD, Buell HE, Riggs DB, Lester GE, Ontjes DA Increased rate of fractures and severe kyphosis: sequelae of living into adulth with cystic fibrosis. Ann Intern Med 1998;128:186-193. Go to original source...
  63. Rossini M, Del Marco A, Dal Santo F, Gatti D, Braggion C, James G, Adami S. Prevalence and correlates of vertebral fractures in adults with cystic fibrosis. 2004;35:771-6.
  64. Kerem E, Conway S, Elborn S, Heijerman H. Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 2005;4:7-26. Go to original source...
  65. Flohr F, Lutz A, App EM, Matthys H, Reincke M. Bone mineral density and quan­ titative ultrasound in adults with cystic fibrosi
  66. - navštíveno 7. 7. 2006. http://www.iscd.org/Visitors/pdfs/ISCD_OP2005.pdf
  67. Bachrach LK, Loutit CW, Moss RB. Osteopenia in adults with cystic fibros J Med 1994;96:27-34. Go to original source...
  68. Bhudhikanok GS, Lim J, Marcus R, Harkins A, Moss RB, Bachrach LK. Corre­ lates of osteopenia in patients with cystic fibrosis. Pediatrics 1996;97:1
  69. Haworth CS, Selby PL, Horrock, AW, Mawer EB, Adams JE, Webb AK. A pro­ spective study of change in bone mineral density over one year in adults with c tic fibrosis. Thorax 2002;57:719-23. Go to original source...
  70. Hardin DS, Arumugam R, Seilheimer DK, LeBlanc A, Ellis KJ. Normal bone mi­ neral density in cystic fibrosis. Arch Dis Child 2001;84:363-368. Go to original source...
  71. Sood M, Hambleton G, Super M, Fraser WD, Adams JE, Mughal MZ. Bone sta­ tus in cystic fibrosis. Arch Dis Child 2001;84:516-20. Go to original source...
  72. Gronowitz E, Garemo M, Lindblad A, Mellstrom D, Strandvik B. Decreased bone mineral density in normal-growing patients with cystic fibrosis. Acta Ihediatr 2003;92:688-93. Go to original source...
  73. Giron RM, Sanchez Molini P, Garcia Vadillo A, Quintana ML, Rodriguez Salvanes F, Jimenez I, Mancha A, Cisneros C, Ancochea J. Efectividad en la aplicación de tres protocolos de prevención y tratamiento de la osteoporosis en pacientes adultos con fibrosis quística. [Protocol for prevention and treatment of osteo sis in patients with cystic fibrosis]Med Clin (Barc) 2005;125:325-8. Go to original source...
  74. Buntain HM, Greer RM, Wong JC, Schluter PJ, Batch J, Lewindon P, Bell SC, Wainwright CE. Pubertal development and its influences on bone mineral density in Australian children and adolescents with cystic fibrosis. J Paediatr Child Hea 2005;41:317-22. Go to original source...
  75. Haslam RH, Borovnicar DJ, Stroud DB, Strauss BJ, Bines JE. Correlates of pre­ pubertal bone mineral density in cystic fibrosis. Arch Dis Child 2001;85:166-71. Go to original source...
  76. Mortensen LA, Chan GM, Alder SC, Marshall BC. Bone mineral status in prepu­ bertal children with cystic fibrosis. J Pediatr 2000;136:648-52. Go to original source...
  77. Botton E, Saraux A, Laselve H, Jousse S, Le Goff P. Musculoskeletal manif tions in cystic fibrosis.Joint Bone Spine 2003;70:327-35. Go to original source...
  78. Buntain HM, Schluter PJ, Bell SC, Greer RM, Wong JC, Batch J, Lewindon P, Wainwright CE. Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis. Thorax 2006;6:146-54. Go to original source...
  79. Cawood TJ, McKenna MJ, Gallagher CG, Smith D, Wen YC, Gibney J, Dodd JD, O'Shea D. Oral bisphosphonates improve Bone Mineral Density in adults with cystic fibrosis. Ir Med J 2005;98:270-3.
  80. Gronowitz E, Mellstrom D, Strandvik B. Normal annual increase of bone mineral density during two years in patients with cystic fibrosis. Pediatrics 2004;114:435 Go to original source...
  81. Hardin DS, Rice J, Ahn C, Ferkol T, Howenstine M, Spears S, Prestidge C, Seil­ heimer DK, Shepherd R. Growth hormone treatment enhances nutrition and growth in children with cystic fibrosis receiving enteral nutrition. J Ibdiatr 2005;146:324-8. Go to original source...
  82. Eubanks V, Koppersmith N, Wooldridge N, Clancy JP, Lyrene R, Arani RB, Lee J, Moldawer L, Atchison J, Sorscher EJ, Makris CM. Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis. J Pediatr 2002;140:439^4. Go to original source...
  83. McKone EF, Tonelli MR, Aitken ML. Adrenal insufficiency and testicular failure secondary to megestrol acetate therapy in a patient with cystic fibrosis. Ibdiatr Pulmonol 2002;34:381-3. Go to original source...
  84. Deal C. The use of intermittent human parathyroid hormone as a treatment for teoporosis. Curr Rheumatol Rep 2004;6:49-58. Go to original source...
  85. Ringuier B, Leboucher B, Leblanc M, Troussier F, Duveau E, Audran M, Ginies JL. [Effect of oral biphosphonates in patients with cystic fibrosis and low bone mineral density] Arch Ibdiatr 2004;11:1445-9. - Anglický abstrakt článku ve francouzštině.
  86. Aris RM, Lester GE, Caminiti M, Blackwood AD, Hensler M, Lark RK, Hecker TM, Renner JB, Guillen U, Brown SA, Neuringer IP, Chalermskulrat W, Ontjes DA Efficacy of alendronate in adults with cystic fibrosis with low bone density. Am J Respir Crit Care Med 2004;169:77-82. Go to original source...
  87. Hanly JG, McKenna MJ, Quigley C, Freaney R, Muldowney FP, FitzGerald MX. Hypovitaminosis D and response to supplementation in older patients with cystic fibrosis. Q J Med 1985;56:377-85.




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