Vitamin D Status in Children with Thalassemia Major

Saira Akram; Tariq Ghafoor; Saadia Awan; Aneeqa Akram; Raazia Nawaz; Asbah Rahman

doi:10.51253/pafmj.v75iSUPPL-2.6146

Authors

Saira Akram Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
Tariq Ghafoor Department of Paedes Oncology, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
Saadia Awan Department of Hematology, Pakistan Air Force, College Rawalpindi Pakistan
Aneeqa Akram Fatima Jinnah Medical University, Lahore Pakistan
Raazia Nawaz Department of Pediatrics, Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
Asbah Rahman Combined Military Hospital/National University of Medical Sciences (NUMS) Rawalpindi Pakistan

DOI:

https://doi.org/10.51253/pafmj.v75iSUPPL-2.6146

Keywords:

Children, Transfusion Dependent Thalassemia Major, Vitamin D Status

Abstract

Objective: To determine the vitamin D status in children with transfusion dependent beta thalassemia major attending outpatient department of Military hospital of Rawalpindi.

Study Design: Cross sectional study.

Place and Duration of Study: Outpatient Department, Pak Emirates Military Hospital, Rawalpindi Pakistan, from Jan to Oct 2020.

Methodology: All transfusion dependent beta-thalassemia major patients irrespective of age and sex were consecutively enrolled. Serum 25-hydroxy vitamin D3 (25(OH)D3) level was measured for the assessment of vitamin D status. Vitamin D sufficiency was defined as serum level of 25-OHD >30 ng/ml, vitamin D insufficiency was defined as serum 25-OHD 30-20 ng/ml, while vitamin D deficiency was defined as serum 25-OHD <20-10 ng/ml.

Results: Of 162 children, 79(48.8%) were males and 83(51.2%) were females. The mean age was 5.8±2.63 years. Vitamin D deficiency was observed in 43(26.5%) patients, vitamin D insufficiency in 85(52.5%), while vitamin D sufficiency in 34(21.0%) patients with (p-value <0.352). A statistically significant association of vitamin D status was observed with regular use of vitamin D supplementation (p-value <0.001), calcium supplementation (p-value <0.001) and bone pains (p-value of <0.001). Type of iron chelation and serum ferritin level (p-value <0.001) were also found to be statistically significant. Association of vitamin D level with regular blood transfusion and frequency of blood transfusion was not found to be statistically significant (p-value<0.231).

Conclusion: Children with beta thalassemia had a significant prevalence of vitamin D insufficiency, which contributes to bone damage.

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References

Bravo-Urquiola M, Arends A, Gomez G, Montilla S, Gerard N, Chacin M, et al. Molecular spectrum of beta-thalassemia mutations in the admixed Venezuelan population, and their linkage to beta-globin gene haplotypes. Hemoglobin 2012; 36(3): 209–218.

Weatherall DJ. The Evolving Spectrum of the Epidemiology of Thalassemia. Hematol Oncol Clin North Am 2018; 32(2): 165-175.

Kattamis A, Forni GL, Aydinok Y, Viprakasit V. Changing patterns in the epidemiology of β‐thalassemia. Eur J Haematol 2020; 105(6): 692-703.

Asif N, Hassan K. Management of thalassemia in Pakistan. J Islamabad Med Dental Coll 2016; 5(4): 152-153.

Li XY, Sun X, Chen J, Qin MQ, Luan Z, Zhu YP, et al. Hematopoietic stem cell transplantation for children with β-thalassemia major: multicenter experience in China. World J. Clin. Pediatr 2018; 14(1): 92-99.

Chuansumrit A, Pengpis P, Mahachoklertwattana P, Sirachainan N, Poomthavorn P, Sungkarat W, et al. Effect of Iron chelation therapy on glucose metabolism in non-transfusion-dependent Thalassaemia. Acta Haematol 2017; 137(1): 20–26.

Baldini M, Marcon A, Ulivieri FM, Seghezzi S, Cassin R, Messina C, et al. Bone quality in beta-thalassemia intermedia: relationships with bone quantity and endocrine and hematologic variables. Ann Hematol 2017; 96(6): 995–1003.

Allegra S, Cusato J, De Francia S, Longo F, Pirro E, Massano D, et al. The effect of vitamin D pathway genes and deferasirox pharmacogenetics on liver iron in thalassaemia major patients. Pharmacogenomics J 2019; 19(5): 417-427.

Ahmed S, Saleem M, Modell B, Petrou M. Screening extended families for genetic hemoglobin disorders in Pakistan. N Engl J Med. 2010; 347(15): 1162–1168.

PK M, Bhagwan B. Biochemical indices and radiological examination to evaluate bone health in children with β-thalassemia major. Int J Contemp Pediatr. 2019; 6(2): 549.

Fatima I, Yaqub N, Anwar T, Nisar YB, Khalid S, Gilani S. Prevalence of Endocrine Complications in Transfusion-Dependent Beta Thalassemic Pakistani Patients. Int J Pathol 2014; 12(2): 77-82.

Sultan S, Irfan SM, Ahmed SI. Biochemical Markers of Bone Turnover in Patients with β-Thalassemia Major: A Single Center Study from Southern Pakistan. Adv Hematol 2016; 5437609.

Ali GS, Najeeb MK, Jubrail AM, Selamoğlu Z. A Study of Vitamin D and Calcium Level in Beta Thalassemia Major in Duhok Province. Turkish J Agriculture-Food Sci Tech 2019; 7(11): 1924-1927.

Vogiatzi MG, Macklin EA, Trachtenberg FL, Fung EB, Cheung AM, Vichinsky E, et al. Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America. Br J Haematol 2009; 146(5): 546-556.

Soliman A, De Sanctis V, Yassin M. Vitamin d status in thalassemia major: an update. Mediterr J Hematol Infect Dis. 2013; 5(1): e2013057.

Holick MF. High prevalence of vitamin D inadequacy and implications for health. Mayo Clin Proc 2006; 81(3): 353-373.

Hamayun T. Assessment of vitamin D and calcium levels in multi transfused β-thalassemia syndrome patients of district Peshawar. Adv Basic Med Sci 2017; 1(2): 41-47.

Izzah AZ, Rofinda ZD, Arbi F. Vitamin D and Parathyroid Hormone Levels and Their Relation to Serum Ferritin Levels in Children with Thalassemia Major: One-Center Study in Western Indonesia. J Adv Med Pharmaceutical Sci 2017; 15(1): 1-5.

Fahim FM, Saad K, Askar EA, Eldin EN, Thabet AF. Growth parameters and vitamin D status in children with thalassemia major in upper Egypt. Int J Hematol Oncol Stem Cell Res 2013; 7(4): 10-14.

Saki F, Salehifar A, Kassaee SR, Omrani GR. Association of vitamin D and FGF23 with serum ferritin in hypoparathyroid thalassemia: a case control study. BMC Nephrol 2020; 21(1): 1-8.

Ansari SH, Lassi ZS, Khowaja SM, Adil SO, Shamsi TS. Hydroxyurea (hydroxycarbamide) for transfusion‐dependent β‐thalassaemia. Cochrane Database Syst Rev 2019; 3(3): CD012064.

Hall JE. Guyton and Hall: textbook of medical physiology. ed. 13th, Jordan: Elsevier; 2017.

Ozturk Z, Genc GE, Gumuslu S. Minerals in thalassaemia major patients: An overview. J Trace Elements Med Biol 2017; 41(1): 1-9.

El-Nashar M, Mortagy AK, El-Beblawy NM, El-Gohary E, Kamel IM, Rashad M, et al. Parathyroid hormone in pediatric patients with β-thalassemia major and its relation to bone mineral density; a case control study. Egyptian J Med Human Genetics 2017; 18(1): 75-78.

Aslam M, Khan F, Haq S, Metabolic Abnormalities in Patients with Beta Thalessemia Major. J Sharif Med Dental Coll 2018; 4(01): 3-7.