Evaluation of pharmacological efficacy and safety of hydroxyurea in sickle cell disease: Study of a pediatric cohort from Chhattisgarh, India

References

• Hockham C, Bhatt S, Colah R, et al. The spatial epidemiology of sickle-cell anaemia in India. Sci Rep. 2018;8(1):17685. doi:10.1038/s41598-018-36077-w.
   PubMedGoogle Scholar
• Patra PK, Chauhan VS, Khodiar PK, Dalla AR, Serjeant GR. Screening for the sickle cell gene in Chhattisgarh state, India: an approach to a major public health problem. J Community Genet. 2011;2(3):147–151. doi:10.1007/s12687-011-0050-4.
   PubMedGoogle Scholar
• Agrawal RK, Patel RK, Shah V, Nainiwal L, Trivedi B. Hydroxyurea in sickle cell disease: drug review. Indian J Hematol Blood Transfus. 2014;30(2):91–96. doi:10.1007/s12288-013-0261-4.
   PubMed Web of Science ®Google Scholar
• Platt OS. Hydroxyurea for the treatment of sickle cell anemia. N Engl J Med. 2008;358(13):1362–1369. doi:10.1056/NEJMct0708272.
   PubMed Web of Science ®Google Scholar
• Stettler N, McKiernan CM, Melin CQ, Adejoro OO, Walczak NB. Proportion of adults with sickle cell anemia and pain crises receiving hydroxyurea. JAMA. 2015;313(16):1671–1672. doi:10.1001/jama.2015.3075.
   PubMed Web of Science ®Google Scholar
• Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2010;115(12):2354–2363. doi:10.1182/blood-2009-05-221333.
   PubMed Web of Science ®Google Scholar
• Wang WC, Ware RE, Miller ST, et al. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet Lond Engl. 2011;377(9778):1663–1672. doi:10.1016/S0140-6736(11)60355-3.
   PubMed Web of Science ®Google Scholar
• Wong TE, Brandow AM, Lim W, Lottenberg R. Update on the use of hydroxyurea therapy in sickle cell disease. Blood. 2014;124(26):3850–3857; quiz 4004. doi:10.1182/blood-2014-08-435768.
   PubMed Web of Science ®Google Scholar
• Bookchin RM, Lew VL. Sickle red cell dehydration: mechanisms and interventions. Curr Opin Hematol. 2002;9(2):107–110. doi:10.1097/00062752-200203000-00004.
   PubMed Web of Science ®Google Scholar
• Covas DT, de Lucena Angulo I, Vianna Bonini Palma P, Zago MA. Effects of hydroxyurea on the membrane of erythrocytes and platelets in sickle cell anemia. Haematologica. 2004;89(3):273–280.
   PubMed Web of Science ®Google Scholar
• Hankins JS, Ware RE, Rogers ZR, et al. Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood. 2005;106(7):2269–2275. doi:10.1182/blood-2004-12-4973.
   PubMed Web of Science ®Google Scholar
• Tshilolo L, Tomlinson G, Williams TN, et al. Hydroxyurea for Children with Sickle Cell Anemia in Sub-Saharan Africa. N Engl J Med. 2019;380(2):121–131. doi:10.1056/NEJMoa1813598.
   PubMed Web of Science ®Google Scholar
• Tripathi A, Jerrell JM, Stallworth JR. Clinical complications in severe pediatric sickle cell disease and the impact of hydroxyurea. Pediatr Blood Cancer. 2011;56(1):90–94. doi:10.1002/pbc.22822.
   PubMed Web of Science ®Google Scholar
• Steinberg MH, Barton F, Castro O, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003;289(13):1645–1651. doi:10.1001/jama.289.13.1645.
   PubMed Web of Science ®Google Scholar
• Varawalla NY, Fitches AC, Old JM. Analysis of β-globin gene haplotypes in Asian Indians: origin and spread of β-thalassaemia on the Indian subcontinent. Hum Genet. 1992;90(4):443–449. doi:10.1007/BF00220475.
   PubMed Web of Science ®Google Scholar
• Dehury S, Meher S, Patel S, et al. Compound heterozygote of Hb S (HBB: c.20A > T)/Hb Westdale (HBB: c.380_396delTGCAGGCTGCCTATCAG): Report of four cases from Odisha State, India. Hemoglobin. 2019;43(2):132–136. doi:10.1080/03630269.2019.1602052.
   PubMed Web of Science ®Google Scholar
• Jain DL, Apte M, Colah R, et al. Efficacy of fixed low dose hydroxyurea in Indian children with sickle cell anemia: a single centre experience. Indian Pediatr. 2013;50(10):929–933. doi:10.1007/s13312-013-0264-0.
   PubMed Web of Science ®Google Scholar
• Habara A, Steinberg MH. Minireview: Genetic basis of heterogeneity and severity in sickle cell disease. Exp Biol Med (Maywood). 2016;241(7):689–696. doi:10.1177/1535370216636726.
   PubMed Web of Science ®Google Scholar
• Jain D, Mohanty D. Clinical manifestations of sickle cell disease in India: misconceptions and reality. Curr Opin Hematol. 2018;25(3):171–176. doi:10.1097/MOH.0000000000000422.
   PubMed Web of Science ®Google Scholar
• Steinberg MH, Chui DHK, Dover GJ, Sebastiani P, Alsultan A. Fetal hemoglobin in sickle cell anemia: a glass half full? Blood. 2014;123(4):481–485. doi:10.1182/blood-2013-09-528067.
   PubMed Web of Science ®Google Scholar
• Rees DC, Gibson JS. Biomarkers in sickle cell disease. Br J Haematol. 2012;156(4):433–445. doi:10.1111/j.1365-2141.2011.08961.x.
   PubMed Web of Science ®Google Scholar
• Steinberg MH. Genetic etiologies for phenotypic diversity in sickle cell anemia. ScientificWorldJ. 2009;9:46–67. doi:10.1100/tsw.2009.10.
   PubMed Web of Science ®Google Scholar
• Ferster A, Tahriri P, Vermylen C, et al. Five years of experience with hydroxyurea in children and young adults with sickle cell disease. Blood. 2001;97(11):3628–3632. doi:10.1182/blood.V97.11.3628.
   PubMed Web of Science ®Google Scholar
• Patel DK, Mashon RS, Patel S, Das BS, Purohit P, Bishwal SC. Low dose hydroxyurea is effective in reducing the incidence of painful crisis and frequency of blood transfusion in sickle cell anemia patients from Eastern India. Hemoglobin. 2012;36(5):409–420. doi:10.3109/03630269.2012.709897.
   PubMed Web of Science ®Google Scholar
• Ghasemi A, Keikhaei B, Ghodsi R. Side effects of hydroxyurea in patients with Thalassemia major and thalassemia intermedia and sickle cell anemia. Iran J Pediatr Hematol Oncol. 2014;4(3):114–117.
   PubMedGoogle Scholar
• Hall R, Gardner K, Rees DC, Chakravorty S. High body mass index in children with sickle cell disease: a retrospective single-centre audit. BMJ Paediatr Open. 2018;2(1):e000302. doi:10.1136/bmjpo-2018-000302.
   PubMedGoogle Scholar
• Yadav R, Lazarus M, Ghanghoria P, et al. Sickle cell disease in Madhya Pradesh, Central India: A comparison of clinical profile of sickle cell homozygote vs. sickle-beta thalassaemia individuals. Hematology. 2016;21(9):558–563. doi:10.1080/10245332.2016.1148893.
   PubMed Web of Science ®Google Scholar
• Ngo D, Bae H, Steinberg MH, et al. Fetal hemoglobin in sickle cell anemia: genetic studies of the Arab-Indian haplotype. Blood Cells Mol Dis. 2013;51(1):22–26. doi:10.1016/j.bcmd.2012.12.005.
   PubMed Web of Science ®Google Scholar
• Segal JB, Strouse JJ, Beach MC, et al. Hydroxyurea for the treatment of sickle cell disease. Evid Rep Technol Assess. 2008 Mar;(165):1–95.
   PubMedGoogle Scholar
• Neves F, Menezes Neto OA, Polis LB, et al. Hematological differences between patients with different subtypes of sickle cell disease on hydroxyurea treatment. Rev Bras Hematol Hemoter. 2012;34(6):426–429. doi:10.5581/1516-8484.20120107.
   PubMedGoogle Scholar
• Silva-Pinto AC, Angulo IL, Brunetta DM, et al. Clinical and hematological effects of hydroxyurea therapy in sickle cell patients: a single-center experience in Brazil. Sao Paulo Med J. 2013;131(4):238–243. doi:10.1590/1516-3180.2013.1314467.
   PubMed Web of Science ®Google Scholar
• Curtis SA, Danda N, Etzion Z, Cohen HW, Billett HH. Elevated steady state wbc and platelet counts are associated with frequent emergency room use in adults with sickle cell anemia. PLoS ONE. 2015;10(8):e0133116. doi:10.1371/journal.pone.0133116.
   PubMed Web of Science ®Google Scholar
• Ofakunrin AOD, Oguche S, Adekola K, et al. Effectiveness and safety of hydroxyurea in the treatment of sickle cell anaemia children in Jos, North Central Nigeria. J Trop Pediatr. 2020;66(3):290–298. doi:10.1093/tropej/fmz070.
   PubMed Web of Science ®Google Scholar
• Shah R, Taborda C, Chawla S. Acute and chronic hepatobiliary manifestations of sickle cell disease: A review. World J Gastrointest Pathophysiol. 2017;8(3):108–116. doi:10.4291/wjgp.v8.i3.108.
   PubMedGoogle Scholar
• Ahn H, Li CS, Wang W. Sickle cell hepatopathy: clinical presentation, treatment, and outcome in pediatric and adult patients. Pediatr Blood Cancer. 2005;45(2):184–190. doi:10.1002/pbc.20317.
   PubMed Web of Science ®Google Scholar
• Koskinas J, Manesis EK, Zacharakis GH, Galiatsatos N, Sevastos N, Archimandritis AJ. Liver involvement in acute vaso-occlusive crisis of sickle cell disease: prevalence and predisposing factors. Scand J Gastroenterol. 2007;42(4):499–507. doi:10.1080/00365520600988212.
   PubMed Web of Science ®Google Scholar
• Traina F, Jorge SG, Yamanaka A, de Meirelles LR, Costa FF, Saad STO. Chronic liver abnormalities in sickle cell disease: a clinicopathological study in 70 living patients. Acta Haematol. 2007;118(3):129–135. doi:10.1159/000107744.
   PubMed Web of Science ®Google Scholar
• Green NS, Barral S. Genetic modifiers of HbF and response to hydroxyurea in sickle cell disease. Pediatr Blood Cancer. 2011;56(2):177–181. doi:10.1002/pbc.22754.
   PubMed Web of Science ®Google Scholar
• Patel S, Purohit P, Mashon RS, et al. The effect of hydroxyurea on compound heterozygotes for sickle cell-hemoglobin D-Punjab-A single centre experience in eastern India: The Effect of Hydroxyurea on HbSD-Punjab. Pediatr Blood Cancer. 2014;61(8):1341–1346. doi:10.1002/pbc.25004.
   PubMed Web of Science ®Google Scholar
• Cokic VP, Smith RD, Beleslin-Cokic BB, et al. Hydroxyurea induces fetal hemoglobin by the nitric oxide-dependent activation of soluble guanylyl cyclase. J Clin Invest. 2003;111(2):231–239. doi:10.1172/JCI16672.
   PubMed Web of Science ®Google Scholar
• Lebensburger JD, Pestina TI, Ware RE, Boyd KL, Persons DA. Hydroxyurea therapy requires HbF induction for clinical benefit in a sickle cell mouse model. Haematologica. 2010;95(9):1599–1603. doi:10.3324/haematol.2010.023325.
   PubMed Web of Science ®Google Scholar