Publication Only: Sickle cell disease
Sickle cell disease (SCD) is one of the most common hereditary hemoglobinopathy. Vitamin D deficiency has emerged as a public health focus in recent years and it is one of the most common nutritional conditions among persons with SCD and there are characteristics specific to SCD that may contribute to this phenomenon including decreased appetite, inability to absorb nutrients due to damage to the intestinal mucosa, as well as an increased basal metabolic rate and higher nutritional demands to sustain normal physiologic functioning.
Assess the level of vitamin D in pediatric SCD patients. Assess the effect of vitamin D supplementation on its clinical outcome including; frequency of vaso-occlusive crisis and hospital admission and rate of infections.
The current study is a follow-up prospective study that included 50 children and adolescents with SCD recruited from the regular attendants Hematology/Oncology Clinic in Pediatric Hospital, Ain Shams University. Patients with SCD as confirmed by qualitative and quantitative analysis of hemoglobin using high performance liquid chromatography (HPLC) at their steady state were recruited. Patients with any inflammatory condition within one month prior to sample collection and those on vitamin D and/or calcium supplementation in the last 6 months were excluded. All patients subjected to clinical assessment and laboratory assessment including complete blood count, liver and kidney function tests and serum 25-OH vitamin D level. Patients were divided into two groups according to their serum vitamin D level above and below 30 nmol and those with insufficient level were treated for three months and prospectively followed up for clinical outcome and had their vitamin D reassessed
There was a high prevalence of vitamin D deficiency (46%); 23 patients had vitamin D level less than 30nmol (Group 1) and 37 patients had vitamin D level more than 30 nmol (Group 2). Upon comparison between group 1 and group 2, there were no significant differences as regard clinic-demographic data and treatment modalities. Patients with SCD and vitamin D deficiency had statistically significant higher history of fracture than those of with normal vitamin D level. There were statistically significant lower frequencies of joint and bone pain, sickle crisis, hospital admission and infections in patients with normal level of vitamin D. There was statistically significant improvement in vitamin D level after supplementation. There was statistically significant improvement as regard number of crisis after 3 months of vitamin D therapy (P = 0.043), with no statistically significant difference other studied parameters. There was statistically significant negative correlation between vitamin D level and number of crisis after 3 months of vitamin D therapy
The high prevalence of vitamin D deficiency in Egyptian SCD patients highlights the importance of early diagnosis and management on the clinical outcome of those patients