A randomized clinical trial comparing 3 different replacement regimens of vitamin D in clinically asymptomatic pediatrics and adolescents with vitamin D insufficiency.

BACKGROUND: Pediatric and Adolescent populations both have special needs for vitamin D especially for growing bone. Inadequate vitamin D is defined as 25 (OH) D(25hydroxy vitamin D) < 30 ng/ml. METHODS: We conducted a randomized, controlled clinical trial from July 2014 over 1 year, aiming to assess the changes in 25 (OH) D and biochemical outcome on calcium and PTH(parathyroid hormone) using 3 different regimens of vitamin D replacement. Initial and 4 month 25 (OH) D, calcium, PTH and 12 month 25 (OH) D levels were assayed. Participants divided into 3 groups: 1) given 400 IU daily, 2) given 45000 IU weekly for 2 months then 400 IU daily, 3) given 2000 IU daily for 3 months then 1000 IU daily. RESULTS: The results showed significant difference between the 3 groups as regards 25 (OH) D at 4 and 12 months (P < 0.001). Regimens used in group 2 and 3 caused increase in 25 (OH) D after 4 month (median increase is 225% and 200% respectively). 25 (OH) D dropped in group 1 and 2 (median decrease is 42 and 53% respectively) but continued to increase in group 3 (median change is 6%). In group 2 serum calcium median change was 1.2% with few cases of hypercalcuria. 94.9, 76.1 and 7.7 are the percent of vitamin D deficient participants in groups 1, 2 and 3 respectively after 12 months follow up. CONCLUSION: We advise as a replacement for vitamin D insufficiency, low loading dose with high maintaince dose rather than the opposite to achieve steady increase in serum 25 (OH) D with no hypercalcemic side effects.

First report of co-morbidity of pantothenate kinase-associated neurodegeneration and three types of chronic hemolytic anemias.

BACKGROUND: Pantothenate kinase-associated neurodegeneration (PKAN), sickle cell anemia, and thalassemia are autosomal recessive disorders that can cause iron deposition in tissues during childhood. PKAN is characterized by accumulation of iron in the basal ganglia causing progressive extrapyramidal manifestations. Thalassemia and sickle cell disease can cause iron overload and deposition in tissues, including central nervous system. PRESENTATION OF CASE: we herein report the first report of comorbidity of PKAN, ß-thalassemia-major, sickle cell and glucose-6-phosphate dehydrogenase deficiency (G6PD) anemias in a 9 years old Saudi female patient who presented with gait disturbance, speech difficulty, and progressive movement disorders of the neck, upper and lower limbs. CONCLUSION: Although extremely rare, ß-thalassemia-major, sickle cell and G6PD anemias can be associated with PKAN. It is unknown whether this association is random or due to an unknown factor that may have caused several mutations.