Hydroxyurea in sickle cell disease—A study of clinico-pharmacological efficacy in the Indian haplotype

Italia, Khushnooma; Jain, Dipty; Gattani, Sushma; Jijina, Farah; Nadkarni, Anita; Sawant, Pratibha; Nair, Sona; Mohanty, Dipika; Ghosh, Kanjaksha; Colah, Roshan

doi:10.1016/j.bcmd.2008.08.003

Cited by 66 publications

(63 citation statements)

References 34 publications

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“…Therefore studies have assessed HU response in different subsets of patients with SCD as part of a continuing search to identify factors that could explain this variability. Published reports have compared HU effects between groups of patients associated with different beta-globin gene haplotypes, interactions with other hemoglobin variants (such as HbC and HbOArab), various ethnic groups and genetic modifiers of HbF synthesis [10,[20][21][22][23][24].…”

Section: Discussionmentioning

confidence: 99%

The Effect of Hydroxyurea Therapy in Bahraini Sickle Cell Disease Patients

Shome

Ajmi

Radhi

et al. 2015

Indian J Hematol Blood Transfus

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Hydroxyurea (HU) is used as a disease-modifying agent in sickle cell disease (SCD). Its beneficial effects have been ascribed to inhibition of the sickling process through increase of fetal hemoglobin (HbF) levels and influence on multiple factors affecting adhesion of erythrocytes to vascular endothelium. The present study investigates the effect of HU in SCD patients who were grouped on the basis of association with α- and β-thalassemia using routine laboratory methods. A retrospective cross-sectional chart-review was done of 51 adult Bahraini SCD patients attending Salmaniya Medical Complex, Bahrain. Four sub-groups of cases were identified: (i) homozygous sickle cell anemia, 24 cases; (ii) SCD with microcytosis, 16 cases; (iii) sickle α-thalassemia, seven cases; and (iv) sickle β thalassemia, four cases. Documented laboratory and clinical data included hemoglobin level (Hb), hematocrit (Hct), red cell indices, hemoglobin fractions, hospital admissions (frequency), number of inpatient-days, pain episodes (frequency) and red cell transfusion requirement (number of units). Pre- and post-treatment data were compared. Hydroxyurea treatment led to highly significant reduction of HbS % and pain crisis episodes in all patient groups. Other changes such as increases of total hemoglobin, Hct and HbF and reduction of hospital admissions, inpatient days and red cell units transfused also occurred but with less consistent levels of significance within patient sub-groups. Treatment with HU is beneficial for all subgroups of Bahraini SCD patients, without or with α- and β-thalassemia interactions.

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Section: Discussionmentioning

confidence: 99%

The Effect of Hydroxyurea Therapy in Bahraini Sickle Cell Disease Patients

Shome

Ajmi

Radhi

et al. 2015

Indian J Hematol Blood Transfus

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“…36 Published genetic predictors of treatment HbF are even more limited; the lack of CAR haplotype was beneficial in MSH, 35 whereas the presence of the XmnI ␥-globin promoter polymorphism was not required for a robust effect among Indian patients. 37 In the current study, we focused more on baseline clinical laboratory parameters, plus first-dose PK parameters, as potential predictors of either the %HbF response or the MTD dose itself. Although a variety of parameters had significant association in univariate analysis, subsequent multiple variable analysis identified only 5 specific parameters with significant associations with MTD HbF and 5 for MTD dose (Table 2).…”

Section: Discussionmentioning

confidence: 99%

Pharmacokinetics, pharmacodynamics, and pharmacogenetics of hydroxyurea treatment for children with sickle cell anemia

Ware

Despotovic

Mortier

et al. 2011

Blood

151

231

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“…Two of the articles were multi-year observational studies that followed patients of a previous study and focused primarily on the morbidity and mortality of patients using hydroxyurea, which fell out of the scope of the clinical question. 1,2 Seven were observational studies, and only "Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH): effect of hydroxyurea on the frequency of painful crises in sickle cell anemia" was a double-blinded randomized controlled trial [3][4][5][6][7][8][9] . The remaining article was a systematic review article written by Lanzkron et al 10 A decision was made to clinically appraise the MSH trial because it directly addressed the clinical question at hand, and it is a highimpact study that is still often cited today for the quality of evidence and clinical relevance it produced.…”

Section: Related Literaturementioning

confidence: 99%

Hydroxyurea lowers the frequency of sickle cell vaso-occlusive crises

Macrander¹

2017

Clin. Res. Prac.

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Hydroxyurea in sickle cell disease—A study of clinico-pharmacological efficacy in the Indian haplotype

Cited by 66 publications

References 34 publications

The Effect of Hydroxyurea Therapy in Bahraini Sickle Cell Disease Patients

The Effect of Hydroxyurea Therapy in Bahraini Sickle Cell Disease Patients

Pharmacokinetics, pharmacodynamics, and pharmacogenetics of hydroxyurea treatment for children with sickle cell anemia

Hydroxyurea lowers the frequency of sickle cell vaso-occlusive crises

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