Diagnosis and prevention of osteoporosis in children with cystic fibrosis

Author:

Zhekaite E. K.1ORCID,Odinaeva N. D.2ORCID,Voronkova N. Yu.1ORCID,Maksimycheva T. Yu.1ORCID,Sorokin A. S.3ORCID

Affiliation:

1. State Budgetary Healthcare Institution of the Moscow region “Research Clinical Institute of Childhood”, Healthcare Ministry of Moscow Region; Federal State Budgetary Scientific Institution “Research Centre for Medical Genetics”, Ministry of Science and Higher Education of the Russian Federation

2. State Budgetary Healthcare Institution of the Moscow region “Research Clinical Institute of Childhood”, Healthcare Ministry of Moscow Region

3. Federal State Budgetary Educational Institution of Higher Education “Russian University of Economics named after G.V.Plekhanov”, Ministry of Science and Higher Education of the Russian Federation

Abstract

Osteoporosis is a metabolic disease of the skeleton characterized by a decrease in bone mass, a disruption of the microarchitecture of bone tissue and, as a result, fractures with minimal trauma. The urgency of the problem of osteoporosis in children with cystic fibrosis arises from the high risk of a decrease in bone mineral density (BMD) in this category of patients.The aim is to investigate the patterns and mechanisms of bone mineral density reduction in children with cystic fibrosis.Methods. 100 patients with cystic fibrosis were examined. The control group included 61 healthy children aged 6 to 17 years. Alimentary and biochemical risk factors for the decrease in BMD were analyzed. X-ray densitometry was performed in children over 6 years of age using a DEXXUM device (South Korea). The concentration of calcidiol 25(OH)D and other biochemical parameters in blood were determined using the enzyme immunoassay method.Results. 62% of patients with CF and 82% of healthy children had normal BMD. The leading factors affecting BMD in patients with cystic fibrosis are: history of meconium ileus, chronic infection with Pseudomonas aeruginosa, lower indicators of nutritional status, and lower lung function (FEV1, %pred). The biochemical markers of osteoremodeling that determine the risk group for osteoporosis in patients with CF are osteocalcin and acid phosphatase. Healthy children who consumed more dietary calcium had better BMD. The effect of dietary calcium intake and vitamin D dose on BMD in patients with CF was not observed.Conclusion. The features of inflammatory and catabolic processes in cystic fibrosis influence the remodeling processes by reducing osteosynthesis and activating osteoresorption. In both groups of children studied, low nutritional status had a negative effect on BMD.

Publisher

Scientific and Practical Reviewed Journal Pulmonology

Reference10 articles.

1. Galindo-Zavala R., Bou-Torrent R., Magallares-López B. et al. Expert panel consensus recommendations for diagnosis and treatment of secondary osteoporosis in children. Pediatr. Rheumatol. Online J. 2020; 18 (1): 20. DOI: 10.1186/s12969-020-0411-9.

2. [Register of patients with cystic fibrosis in the Russian Federation. 2011]. Pul’monologiya. 2014 (Suppl.). Available at: https://mukoviscidoz.org/doc/registr/Registr_end_2011.pdf [Accessed: June 22, 2023] (in Russian).

3. Kondratyeva E.I., Krasovskiy S.A., Starinova M.A. et al., eds. [Register of patients with cystic fibrosis in the Russian Federation. 2020]. Moscow: Medpraktika-M; 2022. Available at: https://api.med-gen.ru/site/assets/files/51107/site_registre_2020.pdf (in Russian).

4. 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.

5. Sinaasappel M., Stern M., Littlewood J. Nutrition in patients with cystic fibrosis: a European Consensus. J. Cyst. Fibros. 2002; 1 (2): 51–75. DOI: 10.1016/s1569-1993(02)00032-2.

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