Details
Category: Volume 07 Issue 07 July 2019
Hits: 2013

Title: Study of Vitamin D Levels in Thalessemia Major Patients in Children

Authors: Shruti Dhale, Subash Valinjkar, Jyothi Janardhanan, Sagar Walhekar

 DOI: https://dx.doi.org/10.18535/jmscr/v7i7.49

Abstract

Introduction: Thalassemia is an inherited disorder of hemoglobin synthesis. Regular blood transfusions and chelation therapy have noticeably prolonged survival in thalassemic patients.[1] Despite a significant increase in the lifespan of these patients, they suffer from multiple abnormalities probably due to iron overload, including endocrinal abnormalities such as hypogonadism, diabetes mellitus, hypothyroidism and hypoparathyroidism.[2]  The pattern of levels of vitamin D levels among the patients with thalassemia major undergoing repeated blood transfusions remain unexplored. Very few studies have been undertaken among Indian population.

Methodology: It is a prospective study and was conducted at St George hospital, Mumbai,on diagnosed patients of thalassemia major admitted to the pediatric wards. Duration of the study was 3 months and 36 patients were included.

Inclusion Criteria

  1. All admitted patients of thalassemia major aged 12 years and below.

Exclusion Criteria

  1. Chronic hemolyticanemia apart from thalassemia major
  2. Those that was very sick
  3. Those suffering from malnutrition
  4. Those who were on supplementation of calcium, phosphorus and vitamin d .

Patients who were willing to participate and sign the inform consent were enrolled in the study. Serum 25 hydroxyvit D levels were done using CLIA.

Results: The mean age of the studied thalassemia patients was 8.76 ± 3.7 years with no gender preponderance. Mean (SD) Vitamin D levels was 16.26 ±8.75 and was found to be statistically significant (p<0.001).

Conclusion: It is evident from the present study that the levels of vitamin D are deficient among β-thalassemia major patients on repeated blood transfusion. The deficiencies may be due to iron overload or due to nutritional deficiency. Frequent monitoring and supplementation in deficient states is recommended.

Keywords: Beta Thalassemia major, Vitamin D levels.

References

  1. Basha KP, ShettyB, Shenoy UV. Prevalence of Hypoparathyroidism (HPT) in Beta Thalassemia Major. Journal of Clinical and Diagnostic Research. 2014 Feb; 8(2):24-26.
  2. NR Thiagarajan, Cg Delhi Kumar, Jayaprakash Sahoo And Sriram Krishnamurthy. Effect of Vitamin D and Calcium Supplementation on Bone Mineral Content in Children with Thalassemia.Indian Pediatrics VOLUME 56: APRIL 15, 2019
  3. Merchant R, Udani A, Puri V, D’cruz V, Patkar D, Karkera A. Evaluation of osteopathy in thalassemia by bone mineral densitometry and biochemical indices. Indian J Pediatr. 2010;77:987-91.
  4. Tzoulis P, Ang AL, Shah FT, Berovic M, Prescott E,Jones R, et al. Prevalence of low bone mass and vitamin D deficiency in β-thalassemia major. Hemoglobin. 2014;38: 173-8.
  5. Leung TF, Hung ECW, Lam CWK, Li CK, Chu Y, Chik KW, et al. Bone mineral density in children with thalassaemia major: Determining factors and effects of bone marrow transplantation. Bone Marrow Transplant. 2005;36:331-6
  6. Saffari F, Mahyar A, Jalilolgadr S. Endocrine and metabolic disorders in β-thalassemia major patients. Casp J Intern Med. 2012;3:466-72.
  7. Cappellini MD, Cohen A, Porter J, Taher A, Viprakasit V, editors. Guidelines for the Management of Transfusion Dependent Thalassaemia (TDT). 3rd ed. Nicosia (CY): Thalassaemia International Federation; 2014.
  8. Voskaridou E, Terpos E. New insights into the pathophysiology and management of osteoporosis in patients with beta thalassaemia. Br J Haematol. 2004;127:127-39
  9. Soliman A, De Sanctis V, Yassin M. Vitamin D status in thalassemia major-an update. Mediterr J Hematol Infect Dis. 2013; 5(1): e2013057.
  10. Chahkandi T, Norouziasl S, Farzad M, Ghanad F. Endocrine disorders in beta thalassemia major patients. Inter J Pediatr. 2017;5(8):5531-8.
  11. Vogiatzi MG, Macklin EA, Trachtenberg FL. et al. Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassemia syndromes in North America. Br J Haematol. 2009 Sep; 146(5):546-56.
  12. Pirinççioğlu AG, Akpolat V, Köksal O, Haspolat K, Söker M. Bone mineral density in children with beta-thalassemia major in Diyarbakir. Bone. 2011; Oct; 49(4):819-23.
  13. Napoli N., Carmena E., Bucchieri S., et al. Low serum levels of 25-hydroxy vitamin D in adults affected by thalassemia major or intermedia. Bone 2006; 38: 888–892.
  14. Claster S, Wood JC, Noetzli L, et al. Nutritional deficiencies in iron overloaded patients with hemoglobinopathies. Am J Hematol. 2009; 84: 344–8.
  15. KHADILKAR, et al. Prevention and Treatment of Vitamin D and Calcium Deficiency in Children and Adolescents: Indian Academy of Pediatrics (IAP) Guidelines. VOLUME 54__JULY 15, 2017
  16. Munns CF, Shaw N, Kiely M, Specker BL, Thacher TD, Ozono K, et al. Global consensus recommendations on prevention and management of nutritional rickets. J ClinEndocrinolMetab. 2016;101:394-415.

Corresponding Author

Jyothi Janardhanan

M.D. Pediatrics, Department of Pediatrics, Grant Govt Medical College, Mumbai, India

Postal Address: Kallelam House, Kozhummal, Kannur, Kerala, Pin: 670521