Authors

1 Pediatric department, child growth and developmental research center, research institute for primordial prevention of non-communicable disease, Isfahan University of medical sciences, Isfahan, Iran.

2 Islamic Azad University of Najafabad

3 Islamic Azad University of Najafabad.

4 Hormozgan University of Medical Sciences

5 child growth and developmental research center, research institute for primordial prevention of non-communicable disease, Isfahan University of medical sciences, Isfahan, Iran.

6 Pediatrics Department, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran

Abstract

Background: Cystic fibrosis is a multisystemic disorder. It is the most common autosomal recessive disorder in whites that causes complications such as changes in bone density. Therefore, evaluating bone densitometry and serum levels of vitamin D in children with cystic fibrosis is the aim of our study.
Methods: This study was performed on 54 children with cystic fibrosis. After recording demographic information, bone densitometry and serum levels of vitamin D were recorded. Chi-square and Pearson correlation tests were used to compare data.
Results: Based on our study 53.7% of patients with CF had vitamin D less than 20 nmol / l. It was also found that based on lumbar BMD, 20.3% had a BMD less than 2.5 - Based on femoral BMD, 18.5% had a BMD less than -2.5. And our results demonstrated that there is no relation between BMD with vitamin D, calcium and phosphorus but it is significantly correlated with age, height, weight, and BMI.
Conclusion: Decreased BMD is high in patients with CF; on the other hand there is a deficiency of vitamin D in more than 50% of these patients to whom vitamin D supplements should be prescribed along with the standard treatments. Further studies are also required to investigate the effect of other factors on BMD.

Keywords

  1. Abdul Wahab A, Hammoudeh M, Allangawi M, Al-Khalaf F, Chandra P. Bone mineral density in cystic fibrosis patients with the CFTR I1234V mutation in a large kindred family is associated with pancreatic sufficiency. International journal of rheumatology. 2014; 2014.
  2. 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. Guide to bone health and disease in cystic fibrosis. The Journal of Clinical Endocrinology & Metabolism. 2005; 90(3):1888-96.
  3. Brookes DS, Briody JN, Munns CF, Davies PS, Hill RJ. Cystic fibrosis-related bone disease in children: Examination of peripheral quantitative computed tomography (pQCT) data. Journal of Cystic Fibrosis. 2015; 14(5):668-77.
  4. Reisi M, Behnam M, Sayedi SJ, Salimi F, Kargar P, Salehi M, Saneian H, Kashani I, Kelishadi R. Prevalence of Cystic Fibrosis Trans-membrane Conductance Regulator Gene common mutations in children with cystic fibrosis in Isfahan, Iran. International Journal of Pediatrics. 2019; 7(4):9333-9.
  5. Donadio MVF, Souza GCd, Tiecher G, Heinzmann-Filho JP, Paim TF, Hommerding PX, Marostica PJC. Bone mineral density, pulmonary function, chronological age, and age at diagnosis in children and adolescents with cystic fibrosis. Jornal de pediatria. 2013; 89(2):151-7.
  6. Donovan Jr DS, Papadopoulos A, Staron RB, Addesso V, Schulman L, McGregor C, F Cosman F, Lindsay RL, Shane E. Bone mass and vitamin D deficiency in adults with advanced cystic fibrosis lung disease. American journal of respiratory and critical care medicine. 1998; 157(6):1892-9.
  7. Reisi M, Modaresi MR, Aghaii Z, Mirlohi SH, Rafiemanesh H, Azizi G, Sayedi SJ. Efficacy and safety of oral sildenafil in cystic fibrosis children with mild to moderate lung disease. Pediatric Pulmonology. 2020; 55(1):156-60.
  8. Putman MS, Haagensen A, Neuringer I, Sicilian L. Celiac disease in patients with cystic fibrosis-related bone disease. Case Reports in Endocrinology. 2017; 2017.
  9. Putman MS, Baker JF, Uluer A, Herlyn K, Lapey A, Sicilian L, Tillotson AP, Gordon CM, Merkel PA, Finkelstein JS. Trends in bone mineral density in young adults with cystic fibrosis over a 15 year period. Journal of Cystic Fibrosis. 2015; 14(4):526-32.
  10. Modaresi MR, Faghinia J, Reisi M, Keivanfar M, Navaie S, Seyyedi J, Baharzade F. Cystic fibrosis prevalence among a group of high-risk children in the main referral children hospital in Iran. Journal of education and health promotion. 2017; 6.
  11. Daley T, Hughan K, Rayas M, Kelly A, Tangpricha V. Vitamin D deficiency and its treatment in cystic fibrosis. Journal of Cystic Fibrosis. 2019; 18:S66-S73.
  12. Wolfenden LL, Judd SE, Shah R, Sanyal R, Ziegler TR, Tangpricha V. Vitamin D and bone health in adults with cystic fibrosis. Clinical endocrinology. 2008; 69(3):374-81.
  13. Ongaratto R, Rosa KMd, Eloi JC, Epifanio M, Marostica P, Pinto LA. Association between hypovitaminosis D and frequency of pulmonary exacerbations in children and adolescents with cystic fibrosis. Einstein (Sao Paulo). 2018; 16.
  14. Aghamohammadi A, Keivanfar M, Navaei S, Shirzadi R, Masiha F, Allameh Z, Heydari A, Eslami S, Azizi G, Reisi M, Modaresi MR. First cystic fibrosis patient registry annual data report-Cystic fibrosis foundation of Iran. Acta Medica Iranica. 2019; 57(1):33-41.
  15. Wani WA, Nazir M, Bhat JI, Ahmad QI, Charoo BA, Ali SW. Vitamin D status correlates with the markers of cystic fibrosis-related pulmonary disease. Pediatrics & Neonatology. 2019; 60(2):210-5.
  16. Chesdachai S, Tangpricha V. Treatment of vitamin D deficiency in cystic fibrosis. The Journal of steroid biochemistry and molecular biology. 2016; 164:36-9.
  17. Rovner AJ, Stallings VA, Schall JI, Leonard MB, Zemel BS. Vitamin D insufficiency in children, adolescents, and young adults with cystic fibrosis despite routine oral supplementation. The American journal of clinical nutrition. 2007; 86(6):1694-9.
  18. Keivanfar M, Daris S, Reisi M, Mehrkesh M. The fractional excretion of sodium in patients with cystic fibrosis treated with oral sodium chloride. American Journal of Clinical and Experimental Urology. 2020; 8(6):185.
  19. Timmers NK, Stellato RK, Van Der Ent CK, Houwen RH, Woestenenk JW. Vitamin D intake, serum 25-hydroxy vitamin D and pulmonary function in pediatric patients with cystic fibrosis: a longitudinal approach. British Journal of Nutrition. 2019; 121(2):195-201.
  20. Brodlie M, Orchard WA, Reeks GA, Pattman S, McCabe H, O'Brien CJ, Thomas MF, Spencer DA. Vitamin D in children with cystic fibrosis. Archives of disease in childhood. 2012; 97(11):982-4.
  21. Lansing AH, McDonald C, Patel RA, Meihls S, Crowell K, Chatfield B, Pohl JF. Vitamin D deficiency in pediatric patients with cystic fibrosis: associated risk factors in the northern United States. Southern medical journal. 2015; 108(3):164-9.
  22. Gupta S, Mukherjee A, Khadgawat R, Kabra M, Lodha R, Kabra SK. Bone mineral density of Indian children and adolescents with cystic fibrosis. Indian Pediatrics. 2017; 54(7):545-9.
  23. Sharma S, Jaksic M, Fenwick S, Byrnes C, Cundy T. Accrual of bone mass in children and adolescents with cystic fibrosis. The Journal of Clinical Endocrinology & Metabolism. 2017; 102(5):1734-9.
  24. Ghaderian M, Keivanfar M, Barani Beiranvand S, Reisi M. Cardiac Involvement in Mild Cystic Fibrosis Lung Involvement Assessed by Tissue‐Doppler Echocardiography. International Journal of Pediatrics. 2019; 7(10):10205-14.