Osteoporoza w mukowiscydozie/ Osteoporosis in cystic fibrosis

• Autorzy: Magdalena Kołodziej , Agnieszka Wiśniowska
• Języki publikacji: EN

Abstrakty

EN

Mukowiscydoza jest chorobą uwarunkowaną genetycznie spowodowaną nieprawidłowym działaniem transbłonowego regulatora mukowiscydozy. Choroba ta manifestuje się przede wszystkim w układzie oddechowym oraz pokarmowym. W przebiegu mukowiscydozy obserwuje się także zmiany w układzie kostno- stawowym, takie jak występowanie obniżonej gęstości kości, a w konsekwencji osteopenii i osteoporozy. Najczęściej wykorzystywanym badaniem diagnostycznym osteoporozy jest densytometria wykonywana średnio co 2 do 5 lat. Inne badania to oznaczenie poziomu witamin D i K, wapnia oraz markerow obrotu kostnego. W leczeniu osteoporozy wykorzystuje się farmakoterapię i fizjoterapię. Leczenie farmakologiczne to przede wszystkim suplementacja witaminami D i K oraz wapniem jak rownież stosowanie bifosfonianow, parathormonu lub terapia hormonem wzrostu. Farmakoterapia osteoporozy jest trudna ze względu na wzajemną interakcję lekow oraz niekorzystny wpływ niektorych lekow stosowanych w leczeniu mukowiscydozy np. steroidow na gęstość kości. Stosując leczenie farmakologiczne nie możemy zaniedbywać istotnego terapeutycznego działania jakim jest fizjoterapia. Terapia drog oddechowych, ćwiczenia ogolnousprawniające i zwiększona aktywność fizyczna w codziennym życiu są zasadniczymi elementami kompleksowego postępowania leczniczego. Wpływają na długotrwałą poprawę wynikow leczenia oraz na wydłużenie i jakość życia pacjentow. Konsekwencje mukowiscydozy, takie jak bole mięśniowo-szkieletowe, wady postawy czy cukrzyca wymagają wdrożenia odpowiedniej strategii postępowania fizjoterapeutycznego.

EN

Mucoviscidosis is a genetic disease caused by the malfunction of Cystic Fibrosis Transmembrane Regulator. This disease is manifested primarily in the respiratory and gastrointestinal systems. Cystic fibrosis affects skeletal system, leads to low bone mineral density and causes osteopenia and osteoporosis. Bone density changes in cystic fibrosis depends on disorders of the respiratory and gastrointestinal systems or are associated with genetic alterations. The most frequently used bone density test is densitometry, which is performed every two to five years. Determining the level of vitamins D, K and calcium and bone turnover markers. Treatment of cystic fibrosis is consisting of pharmacotherapy and physiotherapy. Pharmacological treatment is primarily supplementation vitamins D, K and calcium as well as bisphosphonates, parathyroid hormone and growth hormone therapy. Unfortunately pharmacotherapy of mucoviscidosis is difficult due to mutual interaction of drugs and negative effect of certain drugs e.g. steroids on bone density. Pharmacotherapy treatment should be used together with physiotherapy. Essential rehabilitation elements are respiratory therapy, whole body exercises and physical activity in everyday life. These activities affect the long-therm improvement in treatment effects and leads to increasing of lifetime and quality of life of the patients. Consequences of cystic fibrosis e.g musculoskeletal pain, posture defects and diabetes are more frequently occurs and require using appropriate strategy of physiotherapy.

Słowa kluczowe

EN

gęstość mineralna kości; mukowiscydoza; fizjoterapia; bone mineral density; cystic fibrosis; physiotherapy

• Czasopismo: Advances in Rehabilitation
• Rocznik: 2015
• Tom: 29
• Numer: 1
• Strony: 39-44

Daty

wydano

2015-03-01

online

2015-07-16

Twórcy

autor

Magdalena Kołodziej

• Instytut Pielęgniarstwa i Nauk o Zdrowiu,

autor

Agnieszka Wiśniowska

• Instytut Fizjoterapii

Bibliografia

• 1. Buzzetti R, Salvatore D, Baldo E, et al. An overview of international literature from cystic fibrosis registries: 1. Mortality and survival studies in cystic fibrosis. J Cyst Fibros 2009; 8: 229-237.
• 2. Boucher RC. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med 2007; 58: 157-170.
• 3. European Association for the Study of the Liver EASL Clinical Practice Guidelines: Management of cholestatic liver diseases. J Hepatol 2009; 51: 237-267.
• 4. Aris RM, Merkel PA, Bachrach LK, Borowitz DS, Boyle MP, Elkin SL. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab Mar 2005; 90(3): 1888-1896.
• 5. Mischler EH, Chesney J, Chesney RW, et al. Demineralisation in cystic fibrosis. Am J Dis Child 1979; 133: 632-635.
• 6. Paccou J, Zeboulon N, Combescure C, Gosec L, Cortet B. The prevalence of osteoporosis, osteopenia and fractures among adults with cystic fibrosis. A systematic literature review with meta- analysis. Calcif Tissue Int 2010; 86: 1-7.
• 7. Haworth CS, Selby PL, Webb AK, Martin L, Elborn JS, Sharples LD. Inflammatory related changes in bone mineral content in adults with cystic fibrosis. Thorax 2004; 59: 613-617.
• 8. Shead EF, Haworth CS, Barker H, Bilton D, Compston JE. Osteoclast function, bone turnover and inflammatory cytokines during infective exacerbations of cystic fibrosis. J Cyst Fibros 2010; 9: 93-98.
• 9. Haworth CS. Impact of cystic fibrosis on bone health. Curr Opin Pulm Med 2010; 16: 616-622.
• 10. Legroux- Gerot I, Leroy S, Prudhomme C, Perez T, Flipo RM, Wallaert B. Cortet B. Bone loss in adults with cystic fibrosis: Prevalence, associated factors and usefulness of biological markers. Joint Bone Spine 2012; 79: 73-77.
• 11. Shead EF, Haworth CS, Gunn E, Bilton D, Scott MA, Compston J. Osteoclastogenesis during Infective Exacerbations in Patients with Cystic Fibrosis. Am J Respir Crit Care Med 2006; 174: 306-311.
• 12. Sermet- Gaudelus I, Souberbielle JC, et al. Low Bone Mineral Density in Young Children with Cystic Fibrosis. Am J Respir Crit Care Med 2007; 175: 951-957.
• 13. Neri AS, Lori I, et al. Bone mineral density in cystic fibrosis patients under the age of 18 years. Minerva Pediatr 2008; 60: 147-154.
• 14. Aris RM, Guise TA. Cystic fibrosis and bone disease: are we missing a genetic link? Eur Respir J 2005; 25: 9-11.
• 15. King SJ, Topliss DJ, Kotsimbos T. Reduced bone density in cystic fibrosis: deltaF508 mutation is an independent risk factor. Eur Respir J 2005; 25: 54-61.
• 16. LeHeron L, Guillaume C, et al. Cystic fibrosis tranmembrane conductance regulator (CFTR) regulates the production of osteoprotegerin (OPG) and prostaglandin (PG) E2 in human bone. J Cyst Fibros 2010; 9:69-72.
• 17. Matuszewska A. Markery obrotu kostnego. Przegl Reumat 2006; 3: 5- 7.
• 18. Minarowska A, Minarowski Ł i wsp. Hand Radiograms as an alternative for bone mass screening in cystic fibrosis. Prog Health Sci 2013; 3: 7-12.
• 19. Nast D, Paniagua C, Anderson P. Cystic fibrosis: A clinician’s tool for management of care advancing into the adult population. J Am Acad Nurse Pract 2012; 24: 625-632.
• 20. Ambroszkiewicz J, Sands D, Gajewska J, Chelchowska M, Laskowsk- Klita T. Bone turnover markers, osteoprotegerin and RANKL cytokines in children with cystic fibrosis Adv Med Scienc 2013; 58: 338-343.
• 21. Zienab S, Guilbault C, et al. Fenretinide prevents the development of osteoporosis in CFTR-KO mice. J Cyst Fibros 2008; 7: 222-230.
• 22. Papaioannou A, Freitag A, et al. Alendronate Once Weekly for the Prevention and Treatment of Bone Loss in Canadian Adult Cystic Fibrosis Patients (CFOS Trial). Chest 2008; 134: 794-800.
• 23. Conway SP, Oldroyd B, Morton A, Truscott JG, Peckham DG. Effect of oral bisphosphonates on bone mineral density and body composition in adult patients with cystic fibrosis: a pilot study. Thorax 2004; 59: 699-703.
• 24. Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. CDSR 2012; 4: CD002010.
• 25. Wilkes DL, Schneiderman JE, Nguyen T, et al. Exercise and physical activity in children with cystic fibrosis. Paediatr Respir Rev 2009; 10:105-￢109.
• 26. Mortensen LA, Chan GM, Alder SC, et al. Bone mineral status in prepubertal children with cystic fibrosis. J Pediatr 2000; 136:648-652.
• 27. Hardin DS, Arumugam R, et al. Normal bone mineral density in cystic fibrosis. Arch Dis Child 2001;84:363-368.
• 28. Sood M, Hambleton G, et al. Bone status in cystic fibrosis. Arch Dis Child 2001;84:516-520.
• 29. Festini F, Ballarin S, et al. Prevalence of pain in adults with cystic fibrosis. J Cyst Fibros 2004;3:51-57.
• 30. Joint pain and arthritis in cystic fibrosis. Leeds Regional Adult and Paediatric Cystic Fibrosis Units, St James’s University Hospital, Leeds, UK. July, 2008.
• 31. Rossini M, Del Marco A, et al. Prevalence and correlates of vertebral fractures in adults with cystic fibrosis. Bone 2004; 35:771-776.
• 32. Aris RM, Renner JB, et al. Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis. Ann Int Med 1998;128:186-193.
• 33. Fauroux B, Isabey D, et al. Nonchemical influence of inspiratory pressure support on inspiratory activity in humans. J Appl Physiol 1998;85:2169-2175.
• 34. Barry SC, Gallagher CG. Corticosteroids and skeletal muscle function in cystic fibrosis. J Appl Physiol 2003;95:1379-1384.
• 35. Prasad SA, Tannenbaum EL, Mikelsons C., Physiotherapy in cystic fibrosis, J R Soc Med 2000; 38: 27-37.
• 36. Voldby C, Green K, et al. Day-Time Variability And Short Term Effect Of Chest Physiotherapy On Multiple Breath Nitrogen Washout In Children With Cystic Fibrosis. ATS 2014; 21: 8-25.
• 37. Urquhart D, Sell Z, et al. Effects of a supervised, outpatient exercise and physiotherapy programme in children with cystic fibrosis. Pediatr Pulmonol 2012;47:1235-￢1241.
• 38. Marciniak J. Biomateriały w chirurgii kostnej. Wyd Polit Śląskiej, Gliwice 1992.
• 39. Dodd M, Prasad SA. Physiotherapy management of cystic fibrosis. Chronic Respiratory Disease 2005;2:139-149.
• 40. Heinonen A, Sievanen H, et al. High-impact exercise and bones of growing girls: a 9-month controlled trial. Osteoporos Int 2000;11:1010-7.
• 41. McVean R, Langman H, et al. Musculoskeletal problems in an adult CF unit. J Cyst Fibros 2002;1:S179.
• 42. McQueen KL, Button BM, Heathcote CH. Massage and musculo-skeletal physiotherapy service for adults: effects on chest mobility, posture, muscle tension, pain, ease of breathing and well-being. Pediatr Pulmonol 2003; 25:240.
• 43. Massery M. Musculoskeletal and neuromuscular interventions: a physical approach to cystic fibrosis. J R Soc Med 2005;98;Suppl 45:55-66.
• 44. Pitukcheewanont P, Safani D. Extremely low-level, short- -term mechanical stimulation increases cancellous and cortical bone density and muscle mass of children with low bone density: a pilot study. The Endocrinologist 2006; 16(3): 128.
• 45. http://www.thoracic.org.au/professional-information/position-papers-guidelines/cystic-fibrosis/ Thoracic Society of Australia and New Zealand. Physiotherapy for Cystic Fibrosis in Australia: A Consensus Statement. 2012.
• 46. Burrows M. Exercise and Bone Mineral Accrual in Children and Adolescents J Sports Sci Med 2007; 6(3): 305-312.
• 47. Vicente-Rodriguez G, Ara I, Perez-Gomez J, Dorado C, Calbet JA. Muscular development and physical activity as major determinants of femoral bone mass acquisition during growth. Br J Sports Med 2005;39:611-616.
• 48. Nixon PA, Orenstein DM, Kelsey SF. Habitual physical activity in children and adolescents with cystic fibrosis. Med Sci Sports Exerc 2001;33:30-35.

Identyfikatory

DOI

10.1515/rehab-2015-0018