Osteoporosis in cystic fibrosis: terminology, diagnosis and clinical signs
https://doi.org/10.18093/0869-0189-2017-27-2-291-297
Abstract
Continuous growth of proportion of adult patients in the total number of patients with cystic fibrosis (CF) is accompanied by the increasing rate of pulmonary and extrapulmonary complications, mainly secondary osteoporosis. This is a multifactorial disease predominantly associated with chronic microbial and inflammatory processes, vitamin D deficiency, low body mass index, delayed puberty and hypogonadism, and long-term (≥3 months) therapy with oral steroids. Controversal issues of terminology and current diagnostic approach to osteoporosis in CF patients have been discussed in the article. Diagnosis of osteoporosis in CF patients should not be based solely on osteodensitometry findings but also on thorough analysis of clinical signs and symptoms, other image diagnostic investigations and laboratory data. Detection of the risk factors could help to control this disease.
About the Authors
I. A. BaranovaRussian Federation
Irina A. Baranova, Doctor of Medicine, Professor at Department of Hospital Therapy, Pediatric Faculty
ul. Ostrovityanova 1, Moscow, 117997
Competing Interests:
Баранова Ирина Александровна – доктор медицинских наук, профессор кафедры госпитальной терапии педиатрического факультета
117997, Москва, ул. Островитянова, 1;
E. I. Kondrat'eva
Russian Federation
Elena I. Kondrat'eva, Doctor of Medicine, Professor, Principal Researcher, Research and Clinical Division of Cystic Fibrosis
ul. Moskvorech'e 1, Moscow, 1115478
A. A. Krasovskiy
Russian Federation
Stanislav A. Krasovskiy, Candidate of Medicine, Senior Researcher at Laboratory of Cystic Fibrosis, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; Senior Researcher at Research and Clinical Division of Cystic Fibrosis, Federal Medical Genetic Academic Center, Russian Academy of Medical Science; pneumologist at the 2nd Pulmonology Department, D.D.Pletnev City Clinical Hospital, Moscow Healthcare Department
References
1. Lesnyak O.M., Benevolenskaya L.I., eds. Clinical Guidelines. Osteoporosis: Diagnosis, Prevention, and Treatment. Moscow: GEOTAR-Media; 2010 (in Russian).
2. Aris R.M., Merkel P.A., Bachrach L.K. et al. Guide to bone health and disease in cystic fibrosis. J. Clin. Endocrinol. Metab. 2005; 90 (3): 1888–1896. DOI: 10.1210/jc.20041629.
3. Krasovskiy S.A., Baranova I.A., Demin N.V., Amelina E.L. Bone mineral density and prevalence of vertebral deformities and peripheral fractures in adult cystic fibrosis patients. Pul'monologiya. 2011; (5): 71–78 (in Russian).
4. Kapranov N.I., Kapustina T.Yu. Bone mineral density in cystic fibrosis patients. Pediatriya. 2008; (5): 36–41 (in Russian).
5. Jacquot J., Delion M., Gangloff S. et al. Bone disease in cystic fibrosis: new pathogenic insights opening novel therapies. Osteoporos. Int. 2016; 27 (4): 1401–1412. DOI: 10.1007/s00198-015-3343-3.
6. Le Henaff C., Mansouri R., Modrowski D. et al. Increased NF-κB activity and decreased Wnt/β-catenin signaling mediate reduced osteoblast differentiation and function in ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) Mice. J. Biol. Chem. 2015; 290 (29): 18009–18017. DOI: 10.1074/jbc.M115.646208.
7. Delion M., Braux J., Jourdain M.L. et al. Overexpression of RANKL in osteoblasts: a possible mechanism of susceptibility to bone disease in cystic fibrosis. J. Pathol. 2016; 240 (1): 50–60. DOI: 10.1002/path.4753.
8. Gore A.P., Kwon S.H., Stenbi A.E. A Roadmap to the brittle bones of cystic fibrosis. J. Osteoporos. 2010; 2011: 926045. DOI: 10.4061/2011/926045.
9. King S.L., Topliss D.J., Kotsimbos T. et al. Reduced bone density in cystic fibrosis: DeltaF508 mutation is an independent risk factor. Eur. Respir. J. 2005; 25 (1): 54–61. DOI: 10.1183/09031936.04.00050204.
10. Ward L.M., Konji V.N., Ma J. The management of osteoporosis in children. Osteoporos. Int. 2016; 27 (7): 2147– 2179. DOI: 10.1007/s00198-016-3515-9.
11. Gordon C.M., Leonard M.B., Zemel B.S. 2013 Pediatric Position Development Conference: Executive summary and reflections. J. Clin. Densitom. 2014; 17 (2): 219–224. DOI: 10.1016/j.jocd.2014.01.007.
12. Skripnikova I.A., Shchelyagina L.A., Novikov V.E. et al. Bone densitometry in clinical practice. Guidelines. Moscow; 2015. Available at: https://www.gnicpm.ru/UserFiles/Metod.rekomendacii-densitometria.pdf (in Russian).
13. Sermet-Gaudelus I., Bianchi M.L., Garabedian M. et al. European cystic fibrosis bone mineralisation guidelines. J. Cyst. Fibros. 2011; 10 (Suppl. 2): S16–S23. DOI: 10.1016/S1569-1993(11)60004-0.
14. Lesnyak O.M., ed. Osteoporosis. Practical Handbook. Moscow: GEOTAR-Media; 2016 (in Russian). 15. ISCD. 2015 ISCD Official Positions – Adult. Available at: http://www.iscd.org/official-positions/2015-iscd-official-positions-adult/
15. Lesnyak O.M., ed. Clinical Guidelines on Prevention and Management of Osteoporosis. Yaroslavl': Litera; 2016 (in Russian).
16. Lee J., Vasikaran S. Current recommendations for laboratory testing and use of bone turnover markersin management of osteoporosis. Ann. Lab. Med. 2012; 32 (2): 105–112. DOI: 10.3343/alm.2012.32.2.105.
17. Tangpricha V., Kelly A., Stephenson A. et al. An update on the screening, diagnosis, management, and treatment of vitamin d deficiency in individuals with cystic fibrosis: evidence-based recommendations from the cystic fibrosis foundation from the Cystic Fibrosis Foundation. J. Clin. Endocrinol. Metab. 2012; 97 (4): 1082–1093. DOI: 10.1210/jc.2011-3050.
Review
For citations:
Baranova I.A., Kondrat'eva E.I., Krasovskiy A.A. Osteoporosis in cystic fibrosis: terminology, diagnosis and clinical signs. PULMONOLOGIYA. 2017;27(2):291-297. (In Russ.) https://doi.org/10.18093/0869-0189-2017-27-2-291-297