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Vitamin D deficiency and its correction in children with sickle cell anaemia

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Abstract

Vitamin D deficiency is common in sickle cell anaemia (SCA, HbSS), although its significance and optimal means of correction are unknown. We conducted an audit to assess the clinical significance of 25-hydroxy vitamin D (25-OHD) deficiency in children with SCA and to evaluate two methods of vitamin D supplementation. We audited 25-OHD levels in 81 children with SCA and looked for statistical associations with biochemical, haematological and clinical parameters. In a separate group of regularly transfused children with SCA, we compared changes in 25-OHD blood concentrations following treatment with either high-dose intramuscular ergocalciferol (n = 15) or 4 days of high-dose oral cholecalciferol (n = 64). Ninety-one percent of children with SCA had 25-OHD levels <20 μg/L. The 25-OHD levels were negatively correlated with increasing age (P < 0.001) but showed no significant relationship to laboratory measurements, transcranial Doppler velocities or hospital attendance. Both intramuscular ergocalciferol and oral cholecalciferol supplementations resulted in increases of 25-OHD blood concentration to normal levels. The mean dose of ergocalciferol was greater than that of cholecalciferol (7,729 versus 5,234 international units (IU)/kg, P < 0.001), but the increment in 25-OHD levels was significantly greater in the oral cholecalciferol group (6.44 versus 2.82 (ng/L)/(IU/kg), P < 0.001). Both approaches resulted in vitamin D sufficiency for about 120 days. Increased 25-OHD concentration was significantly associated with increased serum calcium concentration. Vitamin D deficiency is very common in SCA and can be effectively corrected with high-dose intramuscular ergocalciferol or 4 days of high-dose oral cholecalciferol. Prospective, randomised studies are needed to assess the clinical value of vitamin D supplementation.

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References

  1. Brousse V, Makani J, Rees DC (2014) Management of sickle cell disease in the community. BMJ 348:g1765. doi:10.1136/bmj.g1765

    Article  PubMed  Google Scholar 

  2. Makariou S, Liberopoulos EN, Elisaf M, Challa A (2011) Novel roles of vitamin D in disease: what is new in 2011? Eur J Intern Med 22(4):355–362. doi:10.1016/j.ejim.2011.04.012

    Article  CAS  PubMed  Google Scholar 

  3. Holick MF, Chen TC (2008) Vitamin D deficiency: a worldwide problem with health consequences. Am J Clin Nutr 87(4):1080S–1086S

    CAS  PubMed  Google Scholar 

  4. Cianferotti L, Marcocci C (2012) Subclinical vitamin D deficiency. Best Pract Res Clin Endocrinol Metab 26(4):523–537. doi:10.1016/j.beem.2011.12.007

    Article  CAS  PubMed  Google Scholar 

  5. Jackson TC, Krauss MJ, Debaun MR, Strunk RC, Arbelaez AM (2012) Vitamin D deficiency and comorbidities in children with sickle cell anemia. Pediatr Hematol Oncol 29(3):261–266. doi:10.3109/08880018.2012.661034

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  6. Garrido C, Cela E, Belendez C, Mata C, Huerta J (2012) Status of vitamin D in children with sickle cell disease living in Madrid, Spain. Eur J Pediatr 171(12):1793–1798. doi:10.1007/s00431-012-1817-2

    Article  CAS  PubMed  Google Scholar 

  7. Chapelon E, Garabedian M, Brousse V, Souberbielle JC, Bresson JL, de Montalembert M (2009) Osteopenia and vitamin D deficiency in children with sickle cell disease. Eur J Haematol 83(6):572–578. doi:10.1111/j.1600-0609.2009.01333.x

    Article  CAS  PubMed  Google Scholar 

  8. Rees DC, Gibson JS (2012) Biomarkers in sickle cell disease. Br J Haematol 156(4):433–445. doi:10.1111/j.1365-2141.2011.08961.x

    Article  CAS  PubMed  Google Scholar 

  9. Powe CE, Evans MK, Wenger J, Zonderman AB, Berg AH, Nalls M, Tamez H, Zhang D, Bhan I, Karumanchi SA, Powe NR, Thadhani R (2013) Vitamin D-binding protein and vitamin D status of black Americans and white Americans. N Engl J Med 369(21):1991–2000. doi:10.1056/NEJMoa1306357

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  10. Diamond TH, Ho KW, Rohl PG, Meerkin M (2005) Annual intramuscular injection of a megadose of cholecalciferol for treatment of vitamin D deficiency: efficacy and safety data. Med J Aust 183(1):10–12

    PubMed  Google Scholar 

  11. Osunkwo I, Hodgman EI, Cherry K, Dampier C, Eckman J, Ziegler TR, Ofori-Acquah S, Tangpricha V (2011) Vitamin D deficiency and chronic pain in sickle cell disease. Br J Haematol 153(4):538–540. doi:10.1111/j.1365-2141.2010.08458.x

    Article  PubMed  Google Scholar 

  12. Osunkwo I, Ziegler TR, Alvarez J, McCracken C, Cherry K, Osunkwo CE, Ofori-Acquah SF, Ghosh S, Ogunbobode A, Rhodes J, Eckman JR, Dampier C, Tangpricha V (2012) High dose vitamin D therapy for chronic pain in children and adolescents with sickle cell disease: results of a randomized double blind pilot study. Br J Haematol 159(2):211–215. doi:10.1111/bjh.12019

    Article  CAS  PubMed  Google Scholar 

  13. Adams-Graves P, Daniels AB, Womack CR, Freire AX (2013) Bone mineral density patterns in vitamin D deficient African American men with sickle cell disease. Am J Med Sci. doi:10.1097/MAJ.0b013e3182893377

    Google Scholar 

  14. Sadat-Ali M, Al-Elq A, Al-Turki H, Sultan O, Al-Ali A, AlMulhim F (2011) Vitamin D level among patients with sickle cell anemia and its influence on bone mass. Am J Hematol 86(6):506–507. doi:10.1002/ajh.22010

    Article  PubMed  Google Scholar 

  15. Shaw NJ, Mughal MZ (2013) Vitamin D and child health part 1 (skeletal aspects). Arch Dis Child 98(5):363–367. doi:10.1136/archdischild-2011-301264

    Article  PubMed  Google Scholar 

  16. Shaw NJ, Mughal MZ (2013) Vitamin D and child health: part 2 (extraskeletal and other aspects). Arch Dis Child 98(5):368–372. doi:10.1136/archdischild-2012-302585

    Article  PubMed  Google Scholar 

  17. Manaseki-Holland S, Maroof Z, Bruce J, Mughal MZ, Masher MI, Bhutta ZA, Walraven G, Chandramohan D (2012) Effect on the incidence of pneumonia of vitamin D supplementation by quarterly bolus dose to infants in Kabul: a randomised controlled superiority trial. Lancet 379(9824):1419–1427. doi:10.1016/S0140-6736(11)61650-4

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  18. Tarcin O, Yavuz DG, Ozben B, Telli A, Ogunc AV, Yuksel M, Toprak A, Yazici D, Sancak S, Deyneli O, Akalin S (2009) Effect of vitamin D deficiency and replacement on endothelial function in asymptomatic subjects. J Clin Endocrinol Metab 94(10):4023–4030. doi:10.1210/jc.2008-1212

    Article  CAS  PubMed  Google Scholar 

  19. Gaston MH, Verter JI, Woods G, Pegelow C, Kelleher J, Presbury G, Zarkowsky H, Vichinsky E, Iyer R, Lobel JS et al (1986) Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. N Engl J Med 314(25):1593–1599. doi:10.1056/NEJM198606193142501

    Article  CAS  PubMed  Google Scholar 

  20. Romagnoli E, Mascia ML, Cipriani C, Fassino V, Mazzei F, D’Erasmo E, Carnevale V, Scillitani A, Minisola S (2008) Short and long-term variations in serum calciotropic hormones after a single very large dose of ergocalciferol (vitamin D2) or cholecalciferol (vitamin D3) in the elderly. J Clin Endocrinol Metab 93(8):3015–3020. doi:10.1210/jc.2008-0350

    Article  CAS  PubMed  Google Scholar 

  21. Tripkovic L, Lambert H, Hart K, Smith CP, Bucca G, Penson S, Chope G, Hypponen E, Berry J, Vieth R, Lanham-New S (2012) Comparison of vitamin D2 and vitamin D3 supplementation in raising serum 25-hydroxyvitamin D status: a systematic review and meta-analysis. Am J Clin Nutr 95(6):1357–1364. doi:10.3945/ajcn.111.031070

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  22. Cytlak UM, Hannemann A, Rees DC, Gibson JS (2013) Identification of the Ca entry pathway involved in deoxygenation-induced phosphatidylserine exposure in red blood cells from patients with sickle cell disease. Pflugers Arch. doi:10.1007/s00424-013-1308-y

    Google Scholar 

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Conflict of interest

The authors have no conflicts of interest to disclose.

Ethical standards

The study was an audit of clinical practice and was approved by institutional audit committee. Individual patient consent was not necessary as the audit involved the analysis of data which were all collected as part of routine clinical care. All procedures followed were in accordance with the Helsinki Declaration of 1975, as revised in 2008 (5).

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Authors and Affiliations

  1. Department of Paediatric Haematology, King’s College Hospital, King’s College London, Denmark Hill, London, SE5 9RS, UK

    Clare Wykes, Anita Arasaretnam & David C. Rees

  2. Department of Child Health, King’s College Hospital, Denmark Hill, London, SE5 9RS, UK

    Sandra O’Driscoll & Laura Farnham

  3. Department of Clinical Biochemistry, King’s College Hospital, Denmark Hill, London, SE5 9RS, UK

    Caje Moniz

Authors
  1. Clare Wykes
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  2. Anita Arasaretnam
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  3. Sandra O’Driscoll
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  4. Laura Farnham
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  5. Caje Moniz
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  6. David C. Rees
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Correspondence to David C. Rees.

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Wykes, C., Arasaretnam, A., O’Driscoll, S. et al. Vitamin D deficiency and its correction in children with sickle cell anaemia. Ann Hematol 93, 2051–2056 (2014). https://doi.org/10.1007/s00277-014-2144-7

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  • DOI: https://doi.org/10.1007/s00277-014-2144-7

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