Bone health in children with cystic fibrosis

Česká verzia

Authors: M. Fajdelová; K. Kubejová; A. Feketeová; J. Ferenczová; V. Vargová
Authors place of work: Klinika detí a dorastu LF UPJŠ a DFN, Košice, Slovensko
Published in the journal: Čes-slov Pediat 2019; 74 (7): 425-429.
Category:

Summary

Low bone mineral density (BMD) in patients with cystic fibrosis (CF) was described for the first time by Mischler in 1979. Decrement in bone solidity in children with CF is mostly caused by pancreatic insufficiency, malnutrition, vitamin D and vitamin K deficiency, late onset of puberty, recurrent infections followed with increased pro-inflammatory activity and intensified bone resorption, as well as systemic corticosteroid treatment. Recent studies call attention to direct influence of CFTR (cystic fibrosis transmembrane conductance regulator) dysfunction in bone cells.

Objective: To assess the prevalence of cystic fibrosis related bone disease in our patients and to determine the correlation with studied parameters as well.

Methods: In our cross-sectional study we enrolled 38 children with cystic fibrosis (19 boys/19 girls, mean age at the time of densitometric scanning 12.71±4.08 years). We evaluated the influence of CF on bone health by 1) anthropometric measurements, 2) selected parameters of bone metabolism and hormonal profile and 3) DXA (dual-energy x-ray absorptiometry), while we adjusted BMD values in the region L1–L4 and TBLH (total body less head) to height (height Z-score). In all the patients we assessed pubertal development, inflammatory markers, pulmonary functions and treatment affecting growing skeleton.

Results: Mean hight Z-score was -0.25±1.21 SD, ranging from -2.37 to 2.23 SD. Growth impairment as characterised by decrement below -2 SD was present in 8 (21.05%) children with CF. Vitamin D deficiency defined as serum calcidiol concentration below 20 ng/ml was present despite concurrect supplementation in as much as 1/5 of patients. Mean value BMD Z-score in L1–L4 was -0.88±0.90 SD and we noticed cystic fibrosis induced low bone mineral density in 6 children (15.80%). None of the patients had clinically significant history of pathologic fractures. The most significant predictor of low BMD was proven to be nutritional impairment (p<0.05), which was present in 1/5 of patients with CF.

Conclusion: Cystic fibrosis is one of rare chronic disease, for which we have existing guidelines on diagnosis, prevention and treatment of secondary osteoporosis in pediatric patients. Maintaining optimal nutritional state and pulmonary functions, reducing oral corticosteroid therapy, adequate vitamin supplementation and frequent densitometric follow-up scanning are the basis for preventing occurance of cystic fibrosis induces bone disease.

Keywords:

Cystic fibrosis – DXA – Cystic Fibrosis Related Bone Disease – CFBD – Bone Mineral Density – BMD

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