source: American Society of Pediatric Hematology/Oncology

year: 2017

authors: Pascale Grégoire-Pelchat, Nathalie Alos, Yves D. Pastore, Virginie Ribault, Nancy Robitaille, Geneviève Mailhot

Background: Sickle cell disease (SCD) can lead to multiple complications, including bone complications and pain crisis. Vitamin D has been reported to have multiple skeletal and extra-skeletal effects, which might help prevent those complications.

Objectives: The objectives of our study were to 1) evaluate the clinical practice at the Sainte-Justine University Hospital Center (UHC) regarding the assessment of vitamin D status in children with SCD, and 2) document the prevalence of vitamin D deficiency, vitamin D intakes and compliance to vitamin D supplementation in our cohort. A secondary objective of our study was to determine if vitamin D deficiency is associated with SCD complications.

Results: Our study population included 116 SCD patients, 53% girls; median age at the time of the evaluation of 11.2 years (range 1.3 to 18.4); 49% were Haitians, 49% Africans and 2% of other ethnicities, genotype was 65% HbSS, 27% HbSC and 8% other genotypes. Characteristics of the study population were representative of the entire cohort followed up at our center. Vitamin D status was then assessed in 38% of SCD children followed up in our clinic. Amongst those, 67% of children were vitamin D insufficient while 33% were deficient. Vitamin D intakes were also insufficient (352.59 +/- 234 IU) and were correlated to serum 25-OH-vitamin D levels (p = 0.005). Moreover, 17% of patients took vitamin D supplements, but only 50% were adherent to their supplementation. Finally, 28.6% of insufficient patients had renal complications, compared to 8.3% for non-insufficient patients (p=0.025) while 18.9% of deficient patients tended to have gall-bladder complications compared to 7.2% for non-deficient patients (p= 0.071).

Conclusion: Given the high prevalence of vitamin D deficiency and its potential association with SCD complications, vitamin D supplementation and effective compliance strategies should be considered in Canadian children with SCD. These results highlight the need for randomized double blind placebo controlled trials to test the impact of optimal vitamin D supplementation on clinically important outcomes in children with SCD.

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