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Sickle cell disease (SCD) is a disease of abnormal hemoglobin associated with severe clinical phenotype and recurrent complications. Hydroxyurea (HU) is one of the US-FDA approved and commonly used drug for the treatment of adult SCD patients with clinical -severity. However, its use in the pediatric groups remains atypical. Despite a high prevalence of the disease in the state Chhattisgarh, there is a lack of evidence supporting its use in pediatric patients. This study aimed to evaluate the pharmacological and clinical efficacy and safety of HU in a large pediatric cohort with SCD from Central India. The study cohort consisted of 164 SCD (138 Hb SS and 26 Hb S beta-thalassemia) children (≤14 years of age) on HU therapy, who were monitored for toxicity, hematological and clinical efficacy at baseline (Pre-HU) and after 24 months (Post-HU). The results highlight the beneficial effects of HU at a mean dose of 18.7 ± 7.0 mg/kg/day. A significant improvement was observed, not only in physical and clinical parameters but also in hematological parameters which include fetal hemoglobin (Hb F), total hemoglobin, hematocrit, mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) levels, when evaluated against the baseline. We did not observe any significant adverse effects during the treatment period. Similar results were obtained on independent analysis of Hb SS and Hb Sβ patients. These findings strengthen the beneficial effect of hydroxyurea in pediatric population also without any serious adverse effects and builds up ground for expanding its use under regular monitoring.

Background: Although clozapine is much researched in western literature, a review on Indian research on clozapine published in 2010 reported limited data and need for further research in this area., Aim: We aimed to conduct a systematic review of research on clozapine from India from 2010 to mid-2022 and also compare the same with research output before 2010., Methodology: A systematic various search engines, i.e., PUBMED, Medknow, Hinari and Google Scholar was done using the key words clozapine and India. Published articles with clozapine in the title and having an author from India, published during 2010 to July 2022 were included., Results: Initial Internet and hand searches yielded 280 articles, out of which 126 articles were excluded due to various reasons and 154 articles, were included for the review. This included 84 case reports, 49 original articles, 11 review articles and 10 letters to the editor as comments. We found an increase in the number of publications during the period of 2010-2022 compared to 1997-2009 in all types of publications. Over the years a significant proportion of the articles focused on various side effects of clozapine, factors associated with response and non-response to clozapine and evaluation of outcomes other than efficacy/effectiveness. However, all the studies were limited to a single centre with no multicentric studies on clozapine., Conclusion: Over the last 12 years or so, there is increase in the number of publications on clozapine. However, there is lack of multicentric studies., Competing Interests: Conflict of interest None., (Copyright © 2022 Elsevier B.V. All rights reserved.)