Background: Growth impairment is a known complication of sickle cell disease (SCD). Effects of hydroxyurea (HU) on growth in older children with SCD have never been reported.
Objective and hypotheses: This study was done to explore the potential effects of HU on growth parameters of older children with SCD and correlate these changes with clinical improvement.
Method: A prospective study was conducted on 97 SCD patients started on HU at Sultan Qaboos University Hospital (Q16UH), Oman during the period between January 2013 and October 2015. Weight, height, and BMI were collected at baseline, 6 and 18 months after start of HU. Anthropometric data were converted to Z scores and compared with World Health Organization (WHO) standards. Z scores were also compared between SCD patients who showed significant clinical improvement in annual vaso-occlusive crisis (VOC) rate and those who did not.
Results: The initial Z scores of included SCD patients were lower than WHO norms for their age and sex. The follow up Z-scores of the whole studied patients at 6 and 18 months from starting HU did not change significantly for both weight and height parameters, however, BMI Z-scores improved significantly at both 6 and 18 months follow up after HU (P value 0.044 and 0.028 respectively). Similarly no significant changes were observed in weight or height Z in those who showed clinical improvement and those who did not during the period of follow up. BMI Z score improved significantly at 18 months follow up for the clinical improvement group (P=0.014). Patients with minimal or no clinical improvement in annual VOCs showed non-significant changes in BMI Z scores after HU therapy.
Conclusion: Hydroxyurea therapy did not adversely affect nor improve the weight and height in older children with SCD even in those with significant clinical improvement. BMI Z scores improved at 18 months of follow-up for the whole studied patients and in those with clinical improvement however, a longer follow up is recommended.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology