Page last updated: 2024-10-28

hydroxyurea and Hemoglobinopathies

hydroxyurea has been researched along with Hemoglobinopathies in 32 studies

Hemoglobinopathies: A group of inherited disorders characterized by structural alterations within the hemoglobin molecule.

Research Excerpts

ExcerptRelevanceReference
"The klf10 gene could indirectly modify γ-globin chain production and hence the level of fetal hemoglobin (HbF) ameliorating the phenotype of β-hemoglobinopathies and the response to hydroxycarbamide (hydroxyurea [HU]) therapy."7.85Klf10 Gene, a Secondary Modifier and a Pharmacogenomic Biomarker of Hydroxyurea Treatment Among Patients With Hemoglobinopathies. ( Aly, NH; El Sherif, NH; Elalfy, MS; Kamal, TM, 2017)
"Hydroxyurea is known to reduce ineffective erythropoiesis and thereby hemolysis leading to a reduction in bilirubin levels in patients with hemoglobinopathies."7.76The effect of UGT1A1 promoter polymorphism on bilirubin response to hydroxyurea therapy in hemoglobinopathies. ( Colah, RB; Ghosh, K; Italia, KY; Jain, D; Jijina, FF; Merchant, R; Mukherjee, M; Nadkarni, AH, 2010)
" Hydroxyurea, EPO preparations, sodium phenylbutyrate, arginine butyrate, and 5-azacytidine/decitabine have shown efficacy in approximately 40% to 70% of sickle cell and beta-thalassemia patients."4.84Fetal globin stimulant therapies in the beta-hemoglobinopathies: principles and current potential. ( Perrine, SP, 2008)
" Single nucleotide polymorphisms (SNPs) in genes located within as well as outside the human beta-globin cluster have recently been shown to be significantly associated with Hb F increase in relation to hydroxyurea (HU) treatment in hemoglobinopathies patients."4.84Pharmacogenomics and therapeutics of hemoglobinopathies. ( Grosveld, FG; Patrinos, GP, 2008)
"Hydroxyurea is an S-phase-specific drug that reversibly inhibits ribonucleoside diphosphate reductase enzyme which catalyses an essential step in the DNA biosynthesis."2.72A comprehensive review of hydroxyurea for β-haemoglobinopathies: the role revisited during COVID-19 pandemic. ( Mettananda, S; Premawardhena, A; Yasara, N, 2021)
" However, application of these drugs at large is not yet justified because a series of questions concerning their long-term efficacy, the correct dosage and timing, their tolerance and toxicity, and the potential long-term dangers, including mutagenicity are still unresolved."2.40New therapies for the haemoglobinopathies. ( Loukopoulos, D, 1997)
"Both diagnosis and treatment of hemoglobinopathies have been associated with an increased risk of fertility impairment."1.91Desire for biological parenthood and patient counseling on the risk of infertility among adolescents and adults with hemoglobinopathies. ( Balcerek, M; Borgmann-Staudt, A; Diepold, M; Diesch-Furlanetto, T; Drexler, B; Dülberg, J; Eisenreich, B; Friedrich, LA; Hegemann, I; Infanti, L; Kager, L; Kroiss, S; Mandic, L; Merki, R; Njue, LM; Radauer-Plank, AC; Roll, S; Rovó, A; Salow, V; Scheinemann, K; Schilling, R; Schneider, M; Sommerhäuser, G, 2023)
"Moreover, genotype analysis of β-thalassemia major and intermedia patients and an independent cohort of β-thalassemia/SCD compound heterozygous patients that do or do not respond to HU treatment showed that the homozygous mutant state of a tagSNP in the KLF10 3'UTR is not present in β-thalassemia intermedia patients and is underrepresented in β-thalassemia/SCD compound heterozygous patients that respond well to HU treatment."1.38KLF10 gene expression is associated with high fetal hemoglobin levels and with response to hydroxyurea treatment in β-hemoglobinopathy patients. ( Bartsakoulia, M; Borg, J; Christou, S; Felice, AE; Georgitsi, M; Grosveld, FG; Hou, J; Karkabouna, S; Kleanthous, M; Kourakli, A; Lappa-Manakou, C; Lederer, C; Ozgur, Z; Papachatzopoulou, A; Patrinos, GP; Philipsen, S; Phylactides, M; Stavrou, EF; Tafrali, C; van Ijcken, W; von Lindern, M, 2012)
"Based on the molecular analysis of beta-thalassemia intermedia, beta-thalassemia homozygotes or compound heterozygotes combined with alpha-thalassemia, as well as the conjunctive abnormalities of beta-thalassemia heterozygote with triplicated haplotype of alpha-globin genes, were the most common cause of thalassemia intermedia in China."1.31The studies of hemoglobinopathies and thalassemia in China--the experiences in Shanghai Institute of Medical Genetics. ( Huang, S; Zeng, Y, 2001)

Research

Studies (32)

TimeframeStudies, this research(%)All Research%
pre-19903 (9.38)18.7374
1990's9 (28.13)18.2507
2000's7 (21.88)29.6817
2010's8 (25.00)24.3611
2020's5 (15.63)2.80

Authors

AuthorsStudies
Patrinos, GP3
Chui, DHK1
Hardison, RC1
Steinberg, MH1
Starlard-Davenport, A1
Gu, Q1
Pace, BS1
Radauer-Plank, AC1
Diesch-Furlanetto, T1
Schneider, M1
Sommerhäuser, G1
Friedrich, LA1
Salow, V1
Dülberg, J1
Diepold, M1
Rovó, A1
Njue, LM1
Drexler, B1
Infanti, L1
Kroiss, S1
Merki, R1
Scheinemann, K1
Eisenreich, B1
Hegemann, I1
Mandic, L1
Kager, L1
Borgmann-Staudt, A1
Schilling, R1
Roll, S1
Balcerek, M1
Tauseef, U1
Anjum, M1
Ibrahim, M1
Baqai, HS1
Tauseef, A1
Tauseef, M1
Asghar, MS1
Zafar, M1
Rasheed, U1
Shaikh, N1
Yasara, N1
Premawardhena, A1
Mettananda, S1
Hankins, JS1
Estepp, JH1
Hodges, JR1
Villavicencio, MA1
Robison, LL1
Weiss, MJ1
Kang, G1
Schreiber, JE1
Porter, JS1
Kaste, SC1
Saving, KL1
Bryant, PC1
Deyo, JE1
Nottage, KA1
King, AA1
Brandow, AM1
Lebensburger, JD1
Adesina, O1
Chou, ST1
Zemel, BS1
Smeltzer, MP1
Wang, WC1
Gurney, JG1
Boucher, AA1
Gurunathan, A1
Taylor, JM1
Ricci, KW1
Vadivelu, S1
Quinn, CT1
Russo, G2
De Franceschi, L1
Colombatti, R1
Rigano, P2
Perrotta, S1
Voi, V1
Palazzi, G1
Fidone, C1
Quota, A1
Graziadei, G1
Pietrangelo, A1
Pinto, V1
Ruffo, GB1
Sorrentino, F1
Venturelli, D1
Casale, M1
Ferrara, F1
Sainati, L1
Cappellini, MD1
Piga, A1
Maggio, A2
Forni, GL1
Allali, S1
Taylor, M1
Albinni, S1
Amiranoff, D1
de Montalembert, M1
Oberoi, S1
Das, R1
Trehan, A1
Ahluwalia, J1
Bansal, D1
Malhotra, P1
Marwaha, RK1
Elalfy, MS1
El Sherif, NH1
Kamal, TM1
Aly, NH1
Perrine, SP1
Mabaera, R1
West, RJ1
Conine, SJ1
Macari, ER1
Boyd, CD1
Engman, CA1
Lowrey, CH1
Italia, KY1
Jijina, FF1
Jain, D1
Merchant, R1
Nadkarni, AH1
Mukherjee, M1
Ghosh, K1
Colah, RB1
Borg, J1
Phylactides, M1
Bartsakoulia, M1
Tafrali, C1
Lederer, C1
Felice, AE1
Papachatzopoulou, A1
Kourakli, A1
Stavrou, EF1
Christou, S1
Hou, J1
Karkabouna, S1
Lappa-Manakou, C1
Ozgur, Z1
van Ijcken, W1
von Lindern, M1
Grosveld, FG2
Georgitsi, M1
Kleanthous, M1
Philipsen, S1
La Spina, M1
Atweh, GF1
DeSimone, J1
Saunthararajah, Y1
Fathallah, H1
Weinberg, RS1
Nagel, RL1
Fabry, ME1
Adams, RJ1
D'Amico, G1
Mascolino, A1
Bachir, D1
Galacteros, F2
Rodgers, GP1
Rachmilewitz, EA1
Rose, C1
Bauters, F1
Olivieri, NF1
Loukopoulos, D1
Hattori, Y1
Zeng, Y1
Huang, S1
Stamatoyannopoulos, JA1
Nienhuis, AW1
Marwick, C1
Nathan, DG3
Dean, A1
Wu, YJ1
Ley, T1
Fordis, CM1
Schechter, AN1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Crizanlizumab Improves Tissue Oxygen Supply Demand Matching in Patients With Sickle Cell Anemia[NCT05469828]Phase 1/Phase 230 participants (Anticipated)Interventional2024-07-01Not yet recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

18 reviews available for hydroxyurea and Hemoglobinopathies

ArticleYear
Targeting Genetic Modifiers of HBG Gene Expression in Sickle Cell Disease: The miRNA Option.
    Molecular diagnosis & therapy, 2022, Volume: 26, Issue:5

    Topics: Anemia, Sickle Cell; Fetal Hemoglobin; Gene Expression; Hemoglobinopathies; Humans; Hydroxyurea; Mic

2022
A comprehensive review of hydroxyurea for β-haemoglobinopathies: the role revisited during COVID-19 pandemic.
    Orphanet journal of rare diseases, 2021, 03-01, Volume: 16, Issue:1

    Topics: Bloodless Medical and Surgical Procedures; COVID-19 Drug Treatment; Enzyme Inhibitors; Hemoglobinopa

2021
Current challenges in the management of patients with sickle cell disease - A report of the Italian experience.
    Orphanet journal of rare diseases, 2019, 05-30, Volume: 14, Issue:1

    Topics: Anemia, Sickle Cell; Disease Management; Hematologic Diseases; Hemoglobinopathies; Humans; Hydroxyur

2019
Transfusing children with hemoglobinopathies.
    Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine, 2019, Volume: 26, Issue:3

    Topics: Allografts; Anemia, Sickle Cell; Child; Emergencies; Erythrocyte Transfusion; Exchange Transfusion,

2019
Fetal globin stimulant therapies in the beta-hemoglobinopathies: principles and current potential.
    Pediatric annals, 2008, Volume: 37, Issue:5

    Topics: Anemia, Sickle Cell; Antisickling Agents; beta-Thalassemia; Erythropoiesis; Erythropoietin; Fatty Ac

2008
A cell stress signaling model of fetal hemoglobin induction: what doesn't kill red blood cells may make them stronger.
    Experimental hematology, 2008, Volume: 36, Issue:9

    Topics: Adolescent; Animals; Butyrates; Clinical Trials as Topic; DNA (Cytosine-5-)-Methyltransferases; Drug

2008
New drugs for childhood anemia.
    Minerva pediatrica, 2003, Volume: 55, Issue:5

    Topics: Adult; Age Factors; Anemia; Anemia, Hemolytic, Autoimmune; Anemia, Sickle Cell; Antibodies, Monoclon

2003
Hemoglobinopathies.
    Hematology. American Society of Hematology. Education Program, 2003

    Topics: Animals; Azacitidine; Butyrates; Cerebral Hemorrhage; Decitabine; Disease Models, Animal; DNA Methyl

2003
Pharmacogenomics and therapeutics of hemoglobinopathies.
    Hemoglobin, 2008, Volume: 32, Issue:1-2

    Topics: Azacitidine; Butyrates; Decitabine; Fetal Hemoglobin; Genome, Human; Hemoglobinopathies; Humans; Hyd

2008
[Potential alternatives to erythrocyte transfusion in hemoglobinopathies: hydroxyurea (HU), erythropoietin (EPO), butyrate derivatives, blood substitutes].
    Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine, 1994, Volume: 1, Issue:1

    Topics: Adolescent; Adult; Anemia, Sickle Cell; Blood Substitutes; Butyrates; Child; Erythrocyte Transfusion

1994
Novel treatment options in the severe beta-globin disorders.
    British journal of haematology, 1995, Volume: 91, Issue:2

    Topics: Erythropoietin; Fatty Acids, Volatile; Genetic Therapy; Globins; Hemoglobinopathies; Humans; Hydroxy

1995
Fetal erythropoiesis and the diagnosis and treatment of hemoglobin disorders in the fetus and child.
    Seminars in perinatology, 1997, Volume: 21, Issue:1

    Topics: alpha-Thalassemia; Antisickling Agents; Erythropoiesis; Fetal Diseases; Fetus; Globins; Hemoglobinop

1997
New therapies for the haemoglobinopathies.
    Journal of internal medicine. Supplement, 1997, Volume: 740

    Topics: Anemia, Sickle Cell; Antisickling Agents; Butyrates; Butyric Acid; Drug Therapy, Combination; Erythr

1997
[Diagnosis and therapy of hemoglobinopathies].
    Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine, 1999, Jun-10, Volume: 88, Issue:6

    Topics: Antisickling Agents; Bone Marrow Transplantation; Globins; Hemoglobinopathies; Humans; Hydroxyurea;

1999
Therapeutic approaches to hemoglobin switching in treatment of hemoglobinopathies.
    Annual review of medicine, 1992, Volume: 43

    Topics: Anemia, Sickle Cell; Animals; Azacitidine; Erythropoietin; Fetal Hemoglobin; Gene Expression Regulat

1992
Pharmacologic manipulation of fetal hemoglobin in the hemoglobinopathies.
    Annals of the New York Academy of Sciences, 1990, Volume: 612

    Topics: Animals; Erythrocytes; Fetal Hemoglobin; Gene Expression; Hemoglobin A; Hemoglobinopathies; Humans;

1990
Augmentation of hemoglobin synthesis by S-phase specific drugs in the K562 cell line.
    Progress in clinical and biological research, 1985, Volume: 191

    Topics: Adult; Azacitidine; Cell Line; Cytarabine; Deoxyribonuclease HpaII; DNA Restriction Enzymes; Fetal H

1985
Regulation of fetal hemoglobin synthesis by cell cycle specific drugs.
    Progress in clinical and biological research, 1985, Volume: 191

    Topics: Adolescent; Adult; Anemia, Sickle Cell; Animals; Cell Cycle; Colony-Forming Units Assay; DNA; DNA (C

1985

Other Studies

14 other studies available for hydroxyurea and Hemoglobinopathies

ArticleYear
Strategies to improve pharmacogenomic-guided treatment options for patients with β-hemoglobinopathies.
    Expert review of hematology, 2021, Volume: 14, Issue:10

    Topics: Anemia, Sickle Cell; beta-Thalassemia; Hemoglobinopathies; Humans; Hydroxyurea; Pharmacogenetics

2021
Desire for biological parenthood and patient counseling on the risk of infertility among adolescents and adults with hemoglobinopathies.
    Pediatric blood & cancer, 2023, Volume: 70, Issue:7

    Topics: Adolescent; Adult; Anemia, Sickle Cell; Child; Counseling; Cross-Sectional Studies; Female; Fertilit

2023
OCCURRENCE OF UNUSUAL HAEMOGLOBINOPATHIES IN BALOCHISTAN: HB SD AND HB SE - PRESENTATION WITH OSTEOMYELITIS.
    Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo, 2021, Volume: 39

    Topics: Administration, Intravenous; Administration, Oral; Anemia, Sickle Cell; Anti-Bacterial Agents; Antis

2021
Sickle Cell Clinical Research and Intervention Program (SCCRIP): A lifespan cohort study for sickle cell disease progression from the pediatric stage into adulthood.
    Pediatric blood & cancer, 2018, Volume: 65, Issue:9

    Topics: Adolescent; Adult; Anemia, Sickle Cell; Biological Specimen Banks; Blood Transfusion; Body Fluids; C

2018
Hemoglobin Southampton complicated by cerebral ischemia, moyamoya, and hydroxyurea-induced methemoglobinemia.
    American journal of hematology, 2019, Volume: 94, Issue:8

    Topics: Aspirin; Brain Ischemia; Child; Female; Hemoglobinopathies; Hemoglobins, Abnormal; Humans; Hydroxyur

2019
HbSD-Punjab: clinical and hematological profile of a rare hemoglobinopathy.
    Journal of pediatric hematology/oncology, 2014, Volume: 36, Issue:3

    Topics: Acute Chest Syndrome; Anemia; Antisickling Agents; Child; Child, Preschool; Chromatography, High Pre

2014
Klf10 Gene, a Secondary Modifier and a Pharmacogenomic Biomarker of Hydroxyurea Treatment Among Patients With Hemoglobinopathies.
    Journal of pediatric hematology/oncology, 2017, Volume: 39, Issue:3

    Topics: Child; Cross-Sectional Studies; Early Growth Response Transcription Factors; Fetal Hemoglobin; Genes

2017
The effect of UGT1A1 promoter polymorphism on bilirubin response to hydroxyurea therapy in hemoglobinopathies.
    Clinical biochemistry, 2010, Volume: 43, Issue:16-17

    Topics: Adolescent; Adult; Bilirubin; Child; Gene Deletion; Glucuronosyltransferase; Hemoglobinopathies; Hum

2010
KLF10 gene expression is associated with high fetal hemoglobin levels and with response to hydroxyurea treatment in β-hemoglobinopathy patients.
    Pharmacogenomics, 2012, Volume: 13, Issue:13

    Topics: 3' Untranslated Regions; Adult; Anemia, Sickle Cell; Antisickling Agents; beta-Thalassemia; Early Gr

2012
Treatment with hydroxyurea and iron chelation therapy in patients with hemoglobinopathies.
    European journal of haematology, 2005, Volume: 75, Issue:3

    Topics: Ferritins; Hemoglobinopathies; Humans; Hydroxyurea; Iron Chelating Agents

2005
Hydroxyurea therapy in highly unstable hemoglobin carriers.
    Blood, 1996, Oct-01, Volume: 88, Issue:7

    Topics: Adult; Anemia, Hemolytic; Combined Modality Therapy; Female; Fetal Hemoglobin; Gene Expression Regul

1996
The studies of hemoglobinopathies and thalassemia in China--the experiences in Shanghai Institute of Medical Genetics.
    Clinica chimica acta; international journal of clinical chemistry, 2001, Volume: 313, Issue:1-2

    Topics: beta-Thalassemia; China; Globins; Hemoglobinopathies; Heterozygote; Homozygote; Humans; Hydroxyurea;

2001
Sickle cell problems continue to challenge medical science, but some progress is noted.
    JAMA, 1990, Jan-26, Volume: 263, Issue:4

    Topics: Anemia, Sickle Cell; Female; Fetal Hemoglobin; Hemoglobinopathies; Humans; Hydroxyurea; Sickle Cell

1990
Regulation of fetal hemoglobin synthesis in the hemoglobinopathies.
    Annals of the New York Academy of Sciences, 1985, Volume: 445

    Topics: Anemia, Sickle Cell; Animals; Cells, Cultured; Erythropoiesis; Fetal Hemoglobin; Haplorhini; Hematop

1985