A clinically meaningful fetal hemoglobin threshold for children with sickle cell anemia during hydroxyurea therapy

Estepp, Jeremie H.; Smeltzer, Matthew P.; Kang, Guolian; Li, Chen; Wang, Winfred C.; Abrams, Christina; Aygun, Banu; Ware, Russell E.; Nottage, Kerri; Hankins, Jane S.

doi:10.1002/ajh.24906

Cited by 88Hemoglobinopathies and Related DisordersBlood groups and transfusionHemophilia Treatment and Research

A clinically meaningful fetal hemoglobin threshold for children with sickle cell anemia during hydroxyurea therapy

Open access

Estepp, Jeremie H., Smeltzer, Matthew P., Kang, Guolian, Li, Chen, Wang, Winfred C., Abrams, Christina, Aygun, Banu, Ware, Russell E. +2 more·2017·American Journal of Hematology

DOI 10.1002/ajh.24906PMC PMC5675769

Open this paper in CoChat — free account Open PDF

Abstract

Hydroxyurea has proven clinical benefits and is recommended to be offered to all children with sickle cell anemia (SCA), but the optimal dosing regimen remains controversial. Induction of red blood cell fetal hemoglobin (HbF) by hydroxyurea appears to be dose‐dependent. However, it is unknown whether maximizing HbF% improves clinical outcomes. HUSTLE (NCT00305175) is a prospective observational study with a primary goal of describing the long‐term clinical effects of hydroxyurea escalated to maximal tolerated dose (MTD) in children with SCA. In 230 children, providing 610 patient‐years of follow up, the mean attained HbF% at MTD was >20% for up to 4 years of follow‐up. When HbF% values were ≤20%, children had twice the odds of hospitalization for any reason (P < .0001), including vaso‐occlusive pain (P < .01) and acute chest syndrome (ACS) (P < .01), and more than four times the odds of admission for fever (P < .001). Thirty day readmission rates were not affected by HbF%. Neutropenia (ANC <1000 × 10 6/L) was rare (2.3% of all laboratory monitoring), transient, and benign. Therefore, attaining HbF >20% was associated with fewer hospitalizations without significant toxicity. These data support the use of hydroxyurea in children, and suggest that the preferred dosing strategy is one that targets a HbF endpoint >20%.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Integrity signals

Registry

Crossref · 10.1002/ajh.24906

Cites

46 of 46 references verified0 citation errors · 0 not checkable

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Of 230 children treated with hydroxyurea with 510 patient-years of follow-up, patients with HbF less than 20% had twice the risk of hospitalization for pain and acute chest syndrome and 2 times the risk of fever as patients with greater than 20% HbF.
“Of 230 children treated with hydroxyurea with 510 patient-years of follow-up, patients with HbF less than 20% had twice the risk of hospitalization for pain and acute chest syndrome and 2 times the risk of fever as patients with .20%HbF.65”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [65]
Split into 2 atoms — 0 confirmed, 2 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measured

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Mortality in sickle cell disease. Life expectancy and risk factors for early death
Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P. (1994) · New England Journal of Medicine · doi:10.1056/nejm199406093302303 · Cited by 3,320 · see citing works
Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia
Charache, S., Terrin, M. L., Moore, R. D., Dover, G. J., Barton, F. B., Eckert, S. V., McMahon, R. P., & Bonds, D. R. (1995) · New England Journal of Medicine · doi:10.1056/nejm199505183322001 · Cited by 2,340 · cited 1× · see citing works
“days, with a median (IQR, range) duration of 30 (28-30, 1-60) days.”
Background
Management of Sickle Cell Disease: Summary of the 2014 Evidence-Based Report by Expert Panel Members
Yawn, B. P., Buchanan, G. R., Afenyi-Annan, A. N., Ballas, S. K., Hassell, K. L., James, A. H., Jordan, L., Lanzkron, S. M., Lottenberg, R., Savage, W. J., et al. (2014) · JAMA · doi:10.1001/jama.2014.10517 · Cited by 1,599 · see citing works
Self-citation
Pain in Sickle Cell Disease: Rates and Risk Factors
Platt, O. S., Thorington, B. D., Brambilla, D. J., Milner, P. F., Rosse, W. F., Vichinsky, E., & Kinney, T. R. (1991) · New England Journal of Medicine · doi:10.1056/nejm199107043250103 · Cited by 1,546 · see citing works
Methods for evaluation of medication adherence and persistence using automated databases
Andrade, S. E., Kahler, K. H., Frech, F. H., & Chan, K. A. (2006) · Pharmacoepidemiology and Drug Safety · doi:10.1002/pds.1230 · Cited by 1,077 · see citing works
Effect of Hydroxyurea on Mortality and Morbidity in Adult Sickle Cell Anemia
Steinberg, M. H., Barton, F. B., Castro, O. L., Pegelow, C. H., Ballas, S. K., Kutlar, A., Orringer, E. P., Bellevue, R., Olivieri, N. F., Eckman, J. R., et al. (2003) · JAMA · doi:10.1001/jama.289.13.1645 · Cited by 928 · see citing works
Crizanlizumab for the prevention of pain crises in sickle cell disease.
Ataga, K. I., Kutlar, A., Kanter, J., Liles, D., Cancado, R., Friedrisch, J., Guthrie, T. H., Knight-Madden, J., Alvarez, O. A., Gordeuk, V. R., et al. (2017) · New England Journal of Medicine · doi:10.1056/nejmoa1611770 · Cited by 914 · see citing works
Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG)
Wang, W. C., Ware, R. E., Miller, S. T., Iyer, R. V., Casella, J. F., Minniti, C. P., Rana, S., Thornburg, C. D., Rogers, Z. R., Kalpatthi, R. V., et al. (2011) · The Lancet · doi:10.1016/s0140-6736(11)60355-3 · Cited by 803 · see citing works
Self-citation
Acute Care Utilization and Rehospitalizations for Sickle Cell Disease
Brousseau, D. C. (2010) · JAMA · doi:10.1001/jama.2010.378 · Cited by 636 · see citing works
Fetal hemoglobin in sickle cell anemia
Akinsheye, I., Alsultan, A., Solovieff, N., Ngo, D., Baldwin, C. T., Sebastiani, P., Chui, D. H. K., & Steinberg, M. H. (2011) · Blood · doi:10.1182/blood-2011-03-325258 · Cited by 553 · see citing works
Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial
Ware, R. E., Davis, B. R., Schultz, W. H., Brown, R. C., Aygun, B., Sarnaik, S., Odame, I., Fuh, B., George, A., Owen, W., et al. (2016) · The Lancet · doi:10.1016/s0140-6736(15)01041-7 · Cited by 515 · see citing works
Self-citation
Definitions of the phenotypic manifestations of sickle cell disease
Ballas, S. K., Lieff, S., Benjamin, L. J., Dampier, C., Heeney, M. M., Hoppe, C. C., Johnson, C. S., Rogers, Z. R., Smith‐Whitley, K., Wang, W. C., et al. (2009) · American Journal of Hematology · doi:10.1002/ajh.21550 · Cited by 454 · see citing works
Self-citation
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)
Voskaridou, E., Christoulas, D., Bilalis, A., Plata, E., Varvagiannis, K., Stamatopoulos, G., Sinopoulou, K., Balassopoulou, A., Loukopoulos, D., & Terpos, E. (2010) · Blood · doi:10.1182/blood-2009-05-221333 · Cited by 430 · see citing works
Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group.
Kinney, T. R., Helms, R. W., O’Branski, E. E., Ohene‐Frempong, K., Wang, W. C., Daeschner, C., Vichinsky, E. P., Redding‐Lallinger, R., Gee, B. E., Platt, O. S., et al. (1999) · PubMed · Cited by 417 · see citing works
Self-citation
Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea
Steinberg, M. H., Lu, Z.-H., Barton, F. B., Terrin, M. L., Charache, S., Dover, G. J., & Hydroxyurea, T. M. S. O. (1997) · Blood · doi:10.1182/blood.v89.3.1078 · Cited by 407 · see citing works
How I use hydroxyurea to treat young patients with sickle cell anemia
Ware, R. E. (2010) · Blood · doi:10.1182/blood-2009-04-146852 · Cited by 395 · see citing works
Self-citation
Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease.
LEIKIN, S. L., Gallagher, D. M., Kinney, T. R., Sloane, D., Klug, P., & RIDA, W. N. (1989) · PubMed · Cited by 361 · see citing works
Mortality in Children and Adolescents With Sickle Cell Disease
LEIKIN, S. L., Gallagher, D. M., Kinney, T. R., Sloane, D., Klug, P. P., & RIDA, W. N. (1989) · PEDIATRICS · doi:10.1542/peds.84.3.500 · Cited by 319 · see citing works
Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia?
Powars, D., Weiss, J., Chan, L., & Schroeder, W. (1984) · Blood · doi:10.1182/blood.v63.4.921.921 · Cited by 314 · see citing works
Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease
Zimmerman, S. A., Schultz, W. H., Davis, J. S., Pickens, C. V., Mortier, N. A., Howard, T. A., & Ware, R. E. (2003) · Blood · doi:10.1182/blood-2003-07-2475 · Cited by 312 · see citing works
Self-citation
Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study
Hankins, J. S., Ware, R. E., Rogers, Z. R., Wynn, L. W., Lane, P. A., Scott, J. P., & Wang, W. C. (2005) · Blood · doi:10.1182/blood-2004-12-4973 · Cited by 283 · see citing works
Self-citation
Fetal hemoglobin in sickle cell anemia: a glass half full?
Steinberg, M. H., Chui, D. H. K., Dover, G. J., Sebastiani, P., & Alsultan, A. (2014) · Blood · doi:10.1182/blood-2013-09-528067 · Cited by 267 · see citing works
Five years of experience with hydroxyurea in children and young adults with sickle cell disease
Ferster, A., Tahriri, P., Vermylen, C., Sturbois, G., Corazza, F., Fondu, P., Devalck, C., Dresse, M. F., Feremans, W., Hunninck, K., et al. (2001) · Blood · doi:10.1182/blood.v97.11.3628 · Cited by 218 · see citing works
Suboptimal medication adherence in Parkinson's disease
Grosset, K. A., Bone, I., & Grosset, D. G. (2005) · Movement Disorders · doi:10.1002/mds.20602 · Cited by 206 · see citing works
Hydroxyurea for sickle cell disease in children and for prevention of cerebrovascular events: the Belgian experience
Gulbis, B. (2004) · Blood · doi:10.1182/blood-2004-07-2704 · Cited by 164 · see citing works
The effect of hydroxcarbamide therapy on survival of children with sickle cell disease
Lobo, C. L. D. C., Pinto, J. F. D. C., do Nascimento, E. M., de Moura, P. G., Cardoso, G. P., & Hankins, J. S. (2013) · British Journal of Haematology · doi:10.1111/bjh.12323 · Cited by 163 · see citing works
Self-citation
Hydroxyurea adherence and associated outcomes among Medicaid enrollees with sickle cell disease
Candrilli, S. D., O’Brien, S. H., Ware, R. E., Nahata, M. C., Seiber, E. E., & Balkrishnan, R. (2010) · American Journal of Hematology · doi:10.1002/ajh.21968 · Cited by 157 · see citing works
Self-citation
Advances in the use of hydroxyurea
Ware, R. E., & Aygün, B. (2009) · Hematology · doi:10.1182/asheducation-2009.1.62 · Cited by 143 · see citing works
Self-citation
Glomerular hyperfiltration and albuminuria in children with sickle cell anemia
Aygün, B., Mortier, N. A., Smeltzer, M. P., Hankins, J. S., & Ware, R. E. (2011) · Pediatric Nephrology · doi:10.1007/s00467-011-1857-2 · Cited by 132 · see citing works
Self-citation
Electronic monitoring and counseling to improve medication adherence
Rosen, M. I., Rigsby, M. O., Salahi, J. T., Ryan, C. E., & Cramer, J. A. (2003) · Behaviour Research and Therapy · doi:10.1016/s0005-7967(03)00149-9 · Cited by 129 · see citing works
Hydroxyurea treatment decreases glomerular hyperfiltration in children with sickle cell anemia
Aygün, B., Mortier, N. A., Smeltzer, M. P., Shulkin, B. L., Hankins, J. S., & Ware, R. E. (2012) · American Journal of Hematology · doi:10.1002/ajh.23365 · Cited by 113 · see citing works
Self-citation
Reference Values and Hematologic Changes From Birth to 5 Years in Patients With Sickle Cell Disease
Brown, A. K. (1994) · Archives of Pediatrics and Adolescent Medicine · doi:10.1001/archpedi.1994.02170080026005 · Cited by 108 · see citing works
Fetal hemoglobin and clinical severity of homozygous sickle cell disease in early childhood
Stevens, M. C. G., Hayes, R., Vaidya, S., & Serjeant, G. R. (1981) · The Journal of Pediatrics · doi:10.1016/s0022-3476(81)80529-x · Cited by 89 · see citing works
Acute hemolytic vascular inflammatory processes are prevented by nitric oxide replacement or a single dose of hydroxyurea
Almeida, C. B., de Souza, L. E. B., Leonardo, F. C., Costa, F. T. M., WERNECK, C. C., Covas, D. T., Costa, F. F., & Conran, N. (2015) · Blood · doi:10.1182/blood-2014-12-616250 · Cited by 82 · see citing works
Hydroxyurea for Children with Sickle Cell Disease
Heeney, M. M., & Ware, R. E. (2010) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2009.11.002 · Cited by 80 · see citing works
Self-citation
New developments in anti‐sickling agents: can drugs directly prevent the polymerization of sickle haemoglobin in vivo?
Oder, E., Safo, M. K., Abdulmalik, O. Y., & Kato, G. J. (2016) · British Journal of Haematology · doi:10.1111/bjh.14264 · Cited by 72 · see citing works
Hydroxyurea therapy requires HbF induction for clinical benefit in a sickle cell mouse model
Lebensburger, J. D., Pestina, T. I., Ware, R. E., Boyd, K., & Persons, D. A. (2010) · Haematologica · doi:10.3324/haematol.2010.023325 · Cited by 70 · see citing works
Self-citation
Improved hydroxyurea effect with the use of text messaging in children with sickle cell anemia
Estepp, J. H., Winter, B., Johnson, M. R., Smeltzer, M. P., Howard, S. C., & Hankins, J. S. (2014) · Pediatric Blood & Cancer · doi:10.1002/pbc.25177 · Cited by 52 · see citing works
Self-citation
Inherited thrombotic disorders: An update
Florell, S. R., & Rodgers, G. M. (1997) · American Journal of Hematology · doi:10.1002/(sici)1096-8652(199701)54:1 · Cited by 41 · see citing works
Self-citation
Hydroxycarbamide treatment and brain MRI/MRA findings in children with sickle cell anaemia
Nottage, K. A., Ware, R. E., Aygün, B., Smeltzer, M. P., Kang, G., Moen, J., Wang, W. C., Hankins, J. S., & Helton, K. J. (2016) · British Journal of Haematology · doi:10.1111/bjh.14235 · Cited by 40 · see citing works
Self-citation
Predictors of splenic function preservation in children with sickle cell anemia treated with hydroxyurea
Nottage, K. A., Ware, R. E., Winter, B., Smeltzer, M. P., Wang, W. C., Hankins, J. S., Dertinger, S. D., Shulkin, B. L., & Aygün, B. (2014) · European Journal Of Haematology · doi:10.1111/ejh.12361 · Cited by 38 · see citing works
Self-citation
Population pharmacokinetics of hydroxyurea for children and adolescents with sickle cell disease
Wiczling, P., Liem, R. I., Panepinto, J. A., Garg, U. C., Abdel‐Rahman, S. M., Kearns, G. L., & Neville, K. A. (2014) · The Journal of Clinical Pharmacology · doi:10.1002/jcph.303 · Cited by 28 · see citing works
Pharmacokinetics and bioequivalence of a liquid formulation of hydroxyurea in children with sickle cell anemia
Estepp, J. H., Melloni, C., Thornburg, C. D., Wiczling, P., Rogers, Z. R., Rothman, J., Green, N., Liem, R. I., Brandow, A. M., Crary, S. E., et al. (2015) · The Journal of Clinical Pharmacology · doi:10.1002/jcph.598 · Cited by 27 · see citing works
Self-citation
Physiologic Decline in Fetal Hemoglobin Parameters in Infants With Sickle Cell Disease
Marcus, S. J., & Ware, R. E. (1999) · Journal of Pediatric Hematology/Oncology · doi:10.1097/00043426-199909000-00013 · Cited by 23 · see citing works
Self-citation
Amelioration of murine sickle cell disease by nonablative conditioning and γ-globin gene-corrected bone marrow cells
Pestina, T. I., Hargrove, P. W., Zhao, H., Mead, P. E., Smeltzer, M. P., Weiss, M. J., Wilber, A. C., & Persons, D. A. (2015) · Molecular Therapy — Methods & Clinical Development · doi:10.1038/mtm.2015.45 · Cited by 18 · see citing works
Self-citation
Association between baseline fetal hemoglobin levels and incidence of severe vaso‐occlusive pain episodes in children with sickle cell anemia
Bhatnagar, P., Keefer, J. R., Casella, J. F., Barron‐Casella, E. A., Bean, C. J., Hooper, C. W., Payne, A. B., Arking, D. E., & DeBaun, M. R. (2013) · Pediatric Blood & Cancer · doi:10.1002/pbc.24588 · Cited by 12 · see citing works

Flagged first, then by citations

Sorted by citations

Therapeutic strategies for sickle cell disease: towards a multi-agent approach
Telen, M. J., Malik, P., & Vercellotti, G. M. (2018) · Nature Reviews Drug Discovery · doi:10.1038/s41573-018-0003-2 · Cited by 184 · see citing works
Fetal hemoglobin in sickle cell anemia
Steinberg, M. H. (2020) · Blood · doi:10.1182/blood.2020007645 · Cited by 136 · see citing works
Robust clinical and laboratory response to hydroxyurea using pharmacokinetically guided dosing for young children with sickle cell anemia
McGann, P. T., Niss, O., Dong, M., Marahatta, A., Howard, T. A., Mizuno, T., Lane, A., Kalfa, T. A., Malik, P., Quinn, C. T., et al. (2019) · American Journal of Hematology · doi:10.1002/ajh.25510 · Cited by 105 · see citing works
Self-citation
Advances in the Treatment of Sickle Cell Disease
Kapoor, S., Little, J. A., & Pecker, L. H. (2018) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2018.08.001 · Cited by 104 · see citing works
Hydroxyurea treatment and neurocognitive functioning in sickle cell disease from school age to young adulthood
Heitzer, A. M., Longoria, J. N., Okhomina, V. I., Wang, W. C., Raches, D., Potter, B., Jacola, L. M., Porter, J. S., Schreiber, J. E., King, A. A., et al. (2021) · British Journal of Haematology · doi:10.1111/bjh.17687 · Cited by 64 · see citing works
Self-citation
Progress and challenges towards CRISPR/Cas clinical translation
Rosenblum, D., Gutkin, A., Dammes, N., & Peer, D. (2020) · Advanced Drug Delivery Reviews · doi:10.1016/j.addr.2020.07.004 · Cited by 60 · see citing works
Healthcare utilization and hydroxyurea adherence in youth with sickle cell disease
Badawy, S. M., Thompson, A. A., Holl, J. L., Penedo, F. J., & Liem, R. I. (2018) · Pediatric Hematology and Oncology · doi:10.1080/08880018.2018.1505988 · Cited by 56 · see citing works
Prospective longitudinal follow‐up of children with sickle cell disease treated with hydroxyurea since infancy
Thomas, R., Dulman, R. Y., Lewis, A., Notarangelo, B., & Yang, E. (2019) · Pediatric Blood & Cancer · doi:10.1002/pbc.27816 · Cited by 54 · see citing works
Clinical applications of CRISPR‐based genome editing and diagnostics
Foss, D. V., Hochstrasser, M. L., & Wilson, R. C. (2019) · Transfusion · doi:10.1111/trf.15126 · Cited by 50 · see citing works
Drug Therapies for the Management of Sickle Cell Disease
Rai, P., & Ataga, K. I. (2020) · F1000Research · doi:10.12688/f1000research.22433.1 · Cited by 43 · see citing works
Sickle cell disease: progress towards combination drug therapy
Pace, B. S., Starlard‐Davenport, A., & Kutlar, A. (2021) · British Journal of Haematology · doi:10.1111/bjh.17312 · Cited by 43 · see citing works
Post-translational modification as a response to cellular stress induced by hemoglobin oxidation in sickle cell disease
Strader, M. B., Jana, S., Meng, F., Heaven, M. R., Shet, A. S., Thein, S. L., & Alayash, A. I. (2020) · Scientific Reports · doi:10.1038/s41598-020-71096-6 · Cited by 40 · see citing works
Voxelotor: alteration of sickle cell disease pathophysiology by a first-in-class polymerization inhibitor
Glaros, A. K., Razvi, R., Shah, N., & Zaidi, A. U. (2021) · Therapeutic Advances in Hematology · doi:10.1177/20406207211001136 · Cited by 40 · see citing works
CRISPR-Cas12a Gene Editing of HBG1 and HBG2 Promoters to Treat Sickle Cell Disease
Hanna, R., Frangoul, H., Piñeiro, L., McKinney, C. M., Mapara, M. Y., Dalal, J. D., Rangarajan, H. G., Atkins, H., Sharma, A., Chang, K., et al. (2026) · New England Journal of Medicine · doi:10.1056/nejmoa2415550 · Cited by 27 · see citing works
Targeting Genetic Modifiers of HBG Gene Expression in Sickle Cell Disease: The miRNA Option
Starlard‐Davenport, A., Gu, Q., & Pace, B. S. (2022) · Molecular Diagnosis & Therapy · doi:10.1007/s40291-022-00589-z · Cited by 27 · see citing works
A Multidimensional Electronic Hydroxyurea Adherence Intervention for Children With Sickle Cell Disease: Single-Arm Before-After Study
Creary, S. E., Chisolm, D. J., Stanek, J. R., Hankins, J. S., & O’Brien, S. H. (2019) · JMIR mhealth and uhealth · doi:10.2196/13452 · Cited by 24 · see citing works
Self-citation
Treating sickle cell anemia: A new era dawns
Steinberg, M. H. (2020) · American Journal of Hematology · doi:10.1002/ajh.25724 · Cited by 25 · see citing works
Elevated fetal haemoglobin levels are associated with decreased incidence of retinopathy in adults with sickle cell disease
Mian, U. K., Tang, J., Allende, A., Heo, M., Bernstein, N., Vattappally, L., Schoenfeld, D., & Minniti, C. P. (2018) · British Journal of Haematology · doi:10.1111/bjh.15617 · Cited by 23 · see citing works
Higher hydroxyurea adherence among young adults with sickle cell disease compared to children and adolescents
Reddy, P. S., Cai, S. W., Barrera, L., King, K., & Badawy, S. M. (2022) · Annals of Medicine · doi:10.1080/07853890.2022.2044509 · Cited by 19 · see citing works
Association between clinical outcomes and metformin use in adults with sickle cell disease and diabetes mellitus
Badawy, S. M., & Payne, A. B. (2019) · Blood Advances · doi:10.1182/bloodadvances.2019000838 · Cited by 19 · see citing works
Oxygen gradient ektacytometry–derived biomarkers are associated with acute complications in sickle cell disease
Rab, M. A. E., Kanne, C. K., Boisson, C., Bos, J., van Oirschot, B. A., Houwing, M. E., Renoux, C., Bartels, M., Rijneveld, A. W., Nur, E., et al. (2023) · Blood Advances · doi:10.1182/bloodadvances.2023011013 · Cited by 18 · see citing works
PUM1 mediates the posttranscriptional regulation of human fetal hemoglobin
Elagooz, R., Dhara, A. R., Gott, R. M., Adams, S. E., White, R. A., Ghosh, A., Ganguly, S., Man, Y., Owusu-Ansah, A. T., Mian, O. Y., et al. (2022) · Blood Advances · doi:10.1182/bloodadvances.2021006730 · Cited by 18 · see citing works
Red blood cell transfusion therapy for sickle cell patients with frequent painful events
Hilliard, L., Kulkarni, V., Sen, B., Caldwell, C., Bemrich‐Stolz, C. J., Howard, T. H., Brandow, A. M., Waite, E. S., & Lebensburger, J. D. (2018) · Pediatric Blood & Cancer · doi:10.1002/pbc.27423 · Cited by 18 · see citing works
Targeted Hydroxyurea Education after an Emergency Department Visit Increases Hydroxyurea Use in Children with Sickle Cell Anemia
Pecker, L. H., Kappa, S. S., Greenfest, A., Darbari, D. S., & Nickel, R. S. (2018) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2018.05.019 · Cited by 18 · see citing works
Delta‐globin gene expression improves sickle cell disease in a humanised mouse model
Porcu, S., Simbula, M., Marongiu, M. F., Perra, A., Poddie, D., Perseu, L., Kowalik, M. A., Littera, R., Barella, S., Caria, C. A., et al. (2021) · British Journal of Haematology · doi:10.1111/bjh.17561 · Cited by 18 · see citing works
Measuring hydroxyurea adherence by pharmacy and laboratory data compared with video observation in children with sickle cell disease
Creary, S. E., Chisolm, D. J., Stanek, J. R., Neville, K. A., Garg, U. C., Hankins, J. S., & O’Brien, S. H. (2020) · Pediatric Blood & Cancer · doi:10.1002/pbc.28250 · Cited by 16 · see citing works
Self-citation
Time to rethink haemoglobin threshold guidelines in sickle cell disease
Ballas, S. K., Kuypers, F. A., Gordeuk, V. R., Hankins, J. S., Thompson, A. A., & Vichinsky, E. P. (2021) · British Journal of Haematology · doi:10.1111/bjh.17578 · Cited by 15 · see citing works
Self-citation
Discovery and Optimization of First-in-Class Molecular Glue Degraders of the WIZ Transcription Factor for Fetal Hemoglobin Induction to Treat Sickle Cell Disease
Kerrigan, J. R., Thomsen, N. M., Cernijenko, A., Kochanek, S. E., Dewhurst, J., O’Brien, G., Ware, N. F., Sanchez, C. C., Manning, J. R., Ma, X., et al. (2024) · Journal of Medicinal Chemistry · doi:10.1021/acs.jmedchem.4c02251 · Cited by 15 · see citing works
HABIT efficacy and sustainability trial, a multi-center randomized controlled trial to improve hydroxyurea adherence in youth with sickle cell disease: a study protocol
Smaldone, A. M., Manwani, D. G., Aygün, B., Smith‐Whitley, K., Jia, H., Bruzzese, J., Findley, S. E., Massei, J. R., & Green, N. (2019) · BMC Pediatrics · doi:10.1186/s12887-019-1746-6 · Cited by 15 · see citing works
An integrated therapeutic approach to sickle cell disease management beyond infancy
Ribeil, J., Pollock, G., Frangoul, H., & Steinberg, M. H. (2023) · American Journal of Hematology · doi:10.1002/ajh.26956 · Cited by 13 · see citing works
Hydroyxurea improves cerebral oxygen saturation in children with sickle cell anemia
Karkoska, K. A., Quinn, C. T., Niss, O., Pfeiffer, A., Dong, M., Vinks, A. A. T. M. M., & McGann, P. T. (2021) · American Journal of Hematology · doi:10.1002/ajh.26120 · Cited by 12 · see citing works
Social determinants of health affect disease severity among preschool children with sickle cell disease
Khan, H., Kang, G., Porter, J. S., Ding, J., Wang, W. C., Estepp, J. H., Gurney, J. G., Davis, R. L., Hankins, J. S., & Hodges, J. (2024) · Blood Advances · doi:10.1182/bloodadvances.2023012379 · Cited by 11 · see citing works
Self-citation
The effects of hydroxycarbamide on the plasma proteome of children with sickle cell anaemia
Brewin, J. N., Tewari, S., Menzel, S., Kirkham, F. J., Inusa, B. P. D., Renney, G., Ward, M. A., & Rees, D. C. (2019) · British Journal of Haematology · doi:10.1111/bjh.15996 · Cited by 11 · see citing works
A novel mouse model of hemoglobin SC disease reveals mechanisms underlying beneficial effects of hydroxyurea
Setayesh, T., Chi, M., Oestreicher, Z., Sakabe, M., Seu, K. G., Zhu, Z., Kaur, H., Tijani, A., Xin, M., Shova, A., et al. (2025) · Blood · doi:10.1182/blood.2024028136 · Cited by 10 · see citing works
Sickle cell nephropathy. Clinical manifestations and new mechanisms involved in kidney injury
Payán‐Pernía, S., Llobet, A. R., Remacha, A. F., Egido, J., Ballarín, J., & Moreno, J. A. (2021) · Nefrología (English Edition) · doi:10.1016/j.nefroe.2021.10.001 · Cited by 10 · see citing works
A Review of Gene Therapies for Hemoglobinopathies
Jones-Wonni, B., Kelkar, A. H., & Achebe, M. (2024) · Hemoglobin · doi:10.1080/03630269.2024.2369534 · Cited by 9 · see citing works
Managing patients with sickle cell disease in primary care
Hehre, R. J. (2020) · JAAPA · doi:10.1097/01.jaa.0000697232.25913.b1 · Cited by 9 · see citing works
Hydroxyurea Use Associated with Nonverbal and Executive Skills in Sickle Cell Anemia
Tarazi, R. A., Patrick, K. E., Iampietro, M. C., & Apollonsky, N. (2021) · Journal of Pediatric Psychology · doi:10.1093/jpepsy/jsab015 · Cited by 9 · see citing works
Trienone analogs of curcuminoids induce fetal hemoglobin synthesis via demethylation at Gγ-globin gene promoter
Nuamsee, K., Chuprajob, T., Pabuprapap, W., Jintaridth, P., Munkongdee, T., Phannasil, P., Vadolas, J., Chaichompoo, P., Suksamrarn, A., & Svasti, S. (2021) · Scientific Reports · doi:10.1038/s41598-021-87738-2 · Cited by 9 · see citing works
Efficacy and safety of oral hydroxyurea in transfusion-dependent β-thalassaemia: a protocol for randomised double-blind controlled clinical trial
Yasara, N., Wickramarathne, N., Mettananda, C. D., Manamperi, A., Premawardhena, A., & Mettananda, S. (2020) · BMJ Open · doi:10.1136/bmjopen-2020-041958 · Cited by 9 · see citing works
Hydroxyurea treatment of sickle cell disease: towards a personalized model-based approach
Pandey, A. (2021) · Journal of Translational Genetics and Genomics · doi:10.20517/jtgg.2020.45 · Cited by 8 · see citing works
A systems pharmacology model for gene therapy in sickle cell disease
Zheng, B. J., Wille, L., Peppel, K., Hagen, D., Matteson, A., Ahlers, J. D., Schaff, J. C., Hua, F., Yuraszeck, T. M., Cobbina, E., et al. (2021) · CPT Pharmacometrics & Systems Pharmacology · doi:10.1002/psp4.12638 · Cited by 7 · see citing works
Efficacy, safety, and pharmacokinetics of a new, ready-to-use, liquid hydroxyurea in children with sickle cell anemia
Rankine‐Mullings, A., Keenan, R., Chakravorty, S., Inusa, B. P. D., Telfer, P. T., Velangi, M., Ware, R. E., Moss, J. J., Lloyd, A. L., Edwards, S., et al. (2023) · Blood Advances · doi:10.1182/bloodadvances.2023010099 · Cited by 7 · see citing works
Self-citation
The Effects of Hydroxyurea Therapy on the Six-Minute Walk Distance in Patients with Adult Sickle Cell Anemia: An Echocardiographic Study
Garadah, T. S., Mandeel, F. H., Jaradat, A., & Thani, K. B. (2019) · Journal of Blood Medicine · doi:10.2147/jbm.s203828 · Cited by 7 · see citing works
Impact of telehealth visits on hydroxyurea response in sickle cell anemia
Shaner, S., Hilliard, L., Howard, T. H., Pernell, B. M., Bhatia, S., & Lebensburger, J. D. (2021) · Pediatric Blood & Cancer · doi:10.1002/pbc.29354 · Cited by 7 · see citing works
How I approach disease‐modifying therapy in children with sickle cell disease in an era of novel therapies
Karkoska, K. A., & McGann, P. T. (2021) · Pediatric Blood & Cancer · doi:10.1002/pbc.29363 · Cited by 6 · see citing works
In-Depth Immunological Typization of Children with Sickle Cell Disease: A Preliminary Insight into Its Plausible Correlation with Clinical Course and Hydroxyurea Therapy
Giulietti, G., Zama, D., Conti, F., Moratti, M., Presutti, M. T., Belotti, T., Cantarini, M. E., Facchini, E., Bassi, M., Selva, P., et al. (2022) · Journal of Clinical Medicine · doi:10.3390/jcm11113037 · Cited by 7 · see citing works
Delta-Globin Gene Expression Is Enhanced in vivo by Interferon Type I
Manchinu, M. F., Simbula, M., Caria, C. A., Musu, E., Perseu, L., Porcu, S., Steri, M., Poddie, D., Frau, J., Cocco, E. E., et al. (2020) · Frontiers in Medicine · doi:10.3389/fmed.2020.00163 · Cited by 6 · see citing works
MicroRNA29B induces fetal hemoglobin via inhibition of the HBG repressor protein MYB in vitro and in humanized sickle cell mice
Gu, Q., Palani, C. D., Smith, A., Li, B., Amos-Abanyie, E. K., Ogu, U. O., Lu, L., Pace, B. S., & Starlard‐Davenport, A. (2022) · Frontiers in Medicine · doi:10.3389/fmed.2022.1043686 · Cited by 6 · see citing works
Nefropatía falciforme. Manifestaciones clínicas y nuevos mecanismos implicados en el daño renal
Payán‐Pernía, S., Llobet, A. R., Remacha, A. F., Egido, J., Ballarín, J., & Moreno, J. A. (2021) · Nefrología · doi:10.1016/j.nefro.2020.10.012 · Cited by 6 · see citing works
Metal Antagonists and Metals
Gray, J. P., Suhali-Amacher, N., Ford, C., & Ray, S. D. (2018) · Side effects of drugs annual · doi:10.1016/bs.seda.2018.08.013 · Cited by 5 · see citing works
Impact of sickle cell anaemia on reproductive health: a narrative review
Kotam, G. P., Morrison, D. B., Nyarkoh, J. K., Mawunyo, A. O., Kotam, J., Osei, G. N., & Adu, P. (2025) · Reproductive Health · doi:10.1186/s12978-025-02169-w · Cited by 4 · see citing works
Mathematical Modeling of Hydroxyurea Therapy in Individuals with Sickle Cell Disease
Pandey, A., Estepp, J. H., Raja, R., Kang, G., & Ramkrishna, D. (2022) · Pharmaceutics · doi:10.3390/pharmaceutics14051065 · Cited by 5 · see citing works
Self-citation
Effectiveness of hydroxycarbamide in children with sickle cell disease – Analysis of dose‐response metrics in a large birth cohort in a tertiary sickle cell centre
Park, H., Bhatti, S. A., & Chakravorty, S. (2019) · Pediatric Blood & Cancer · doi:10.1002/pbc.27615 · Cited by 5 · see citing works
BCL11A-targeted γ-globin gene induction by triterpenoid glycosides of Fagonia indica: A preclinical scientific validation of indigenous herb for the treatment of β-hemoglobinopathies
Iftikhar, F., Khan, M. B. N., Tehreem, S., Kanwal, N., & Musharraf, S. G. (2023) · Bioorganic Chemistry · doi:10.1016/j.bioorg.2023.106768 · Cited by 4 · see citing works
miR-365-3p mediates BCL11A and SOX6 erythroid-specific coregulation: A new player in HbF activation
Simbula, M., Manchinu, M. F., Mingoia, M., Pala, M., Asunis, I., Caria, C. A., Perseu, L., Shah, M., Crossley, M., Moi, P., et al. (2023) · Molecular Therapy — Nucleic Acids · doi:10.1016/j.omtn.2023.09.002 · Cited by 3 · see citing works
Intensive hydroxyurea dosing in very young children with sickle cell anemia
Wang, W. C., Brown, C., McNaull, M. A., Rogers, Z. R., Barton, M., Dua, M., Hankins, J. S., Gossett, J. M., Richardson, J. L., Porter, J. S., et al. (2023) · Blood Advances · doi:10.1182/bloodadvances.2022009613 · Cited by 3 · see citing works
Self-citation
The HBG2 rs7482144 (C > T) Polymorphism is Linked to HbF Levels but not to the Severity of Sickle Cell Anemia
Bhaskar, L. V., & Pattnaik, S. (2021) · Journal of Pediatric Genetics · doi:10.1055/s-0041-1733950 · Cited by 3 · see citing works
Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness
Mkwambe, M. C., Deng, Y., & Dongchi, Z. (2024) · International Journal of Clinical Medicine · doi:10.4236/ijcm.2024.151001 · Cited by 3 · see citing works
Randomized pilot trial of cell phone support to improve medication adherence among adolescents and young adults with chronic health conditions
Sayegh, C. S., MacDonell, K. K., Iverson, E. F., Beard, B., CHANG, N. T., Vu, M. H., & Belzer, M. E. (2024) · BMC Digital Health · doi:10.1186/s44247-024-00069-w · Cited by 2 · see citing works
Structure-based development of novel substrate-type G9a inhibitors as epigenetic modulators for sickle cell disease treatment
Nishigaya, Y., Takase, S., Sumiya, T., Sato, T., Niwa, H., Sato, S., Nakata, A., Matsuoka, S., Maemoto, Y., Hashimoto, N., et al. (2024) · Bioorganic & Medicinal Chemistry Letters · doi:10.1016/j.bmcl.2024.129856 · Cited by 2 · see citing works
Post-GWAS Validation of Target Genes Associated with HbF and HbA2 Levels
Caria, C. A., Faà, V., Porcu, S., Marongiu, M. F., Poddie, D., Perseu, L., Meloni, A., Vaccargiu, S., & Ristaldi, M. S. (2024) · Cells · doi:10.3390/cells13141185 · Cited by 2 · see citing works
Optimization of hydroxyurea in sickle cell disease in Brazil
Lobo, C. L. D. C., Silva‐Pinto, A. C., & Cançado, R. D. (2025) · Hematology Transfusion and Cell Therapy · doi:10.1016/j.htct.2025.103826 · Cited by 2 · see citing works
Hydroxycarbamide decreases the free alpha‐hemoglobin pool in red blood cells of adult patients with sickle cell anemia
Domingues‐Hamdi, E., Vasseur, C., Pakdaman, S., Moutereau, S., Habibi, A., Bartolucci, P., Galactéros, F., & Baudin‐Creuza, V. (2020) · American Journal of Hematology · doi:10.1002/ajh.25947 · Cited by 2 · see citing works
Is Hydroxyurea Treatment Changing the Life of Children with Sickle Cell Disease?
Sabbah, M. A. A., Radaideh, M. A., Saleh, S. M., Al-Doory, S. A., Abdalqader, A. M., Mir, F. F., & Mohammed, A. (2023) · Dubai Medical Journal · doi:10.1159/000531257 · Cited by 2 · see citing works
Hydroxyurea mobile directly observed therapy versus standard monitoring in patients with sickle cell anemia: a phase 2 randomized trial
Sasi, P. G., Makubi, A. N., Sangeda, R. Z., Ngaeje, M., Mmbando, B. P., Soka, J., Rosano, C., Magesa, A. S., Cox, S. E., Makani, J., et al. (2024) · Communications Medicine · doi:10.1038/s43856-024-00552-5 · Cited by 2 · see citing works
Gene polymorphisms predicting response to hydroxyurea treatment in Bahraini patients with sickle cell disease
Al-Shaikh, F. S., Deifalla, A., Sequeira, R. P., & Woodman, A. (2025) · Expert Review of Hematology · doi:10.1080/17474086.2025.2546575 · Cited by 1 · see citing works
Factors associated with hemoglobin response to hydroxyurea in patients with sickle cell disease
Saber, C., Tukakira, J., Kiriza, N. K., Oluwole, O. B., Castro, L. M. D., Brooks, M. M., Novelli, E. M., & Xu, J. Z. (2026) · Blood Red Cells & Iron · doi:10.1016/j.brci.2026.100048 · Cited by 1 · see citing works
Variability of Prognostic Results Based on Biological Parameters in Sickle Cell Disease Cohort Studies in Children: What Should Clinicians Know?
Sommet, J., Roux, É. L., Koehl, B., Haouari, Z., Mohamed, D., Baruchel, A., Benkerrou, M., & Alberti, C. (2021) · Children · doi:10.3390/children8020143 · Cited by 1 · see citing works
A Versatile and Efficient Novel Approach for Mendelian Randomization Analysis with Application to Assess the Causal Effect of Fetal Hemoglobin on Anemia in Sickle Cell Anemia
Liyanage, J. S. S., Estepp, J. H., Srivastava, K., Rashkin, S. R., Sheehan, V., Hankins, J. S., Takemoto, C. M., Li, Y., Cui, Y., Mori, M., et al. (2022) · Mathematics · doi:10.3390/math10203743 · Cited by 1 · see citing works
Self-citation
Leveraging mathematical modeling to analyze nonadherence for hydroxyurea therapy in sickle cell disease
Pandey, A., Raja, R., Estepp, J. H., & Ramkrishna, D. (2023) · CPT Pharmacometrics & Systems Pharmacology · doi:10.1002/psp4.12945 · Cited by 1 · see citing works
Self-citation
Sickle Cell Disease
Menon, N., & Frei‐Jones, M. J. (2020) · Springer eBooks · doi:10.1007/978-3-030-49980-8_5
Impact of Telehealth Visit and Socioeconomic Status (SES) on Hydroxyurea Response in Sickle Cell AnemiaPreprint
Shaner, S., Hilliard, L., Howard, T. H., Pernell, B. M., Bhatia, S., & Lebensburger, J. D. (2021) · doi:10.22541/au.162041376.61578469/v1
Evaluation of persistence and fate of ex vivo edited-HSC modified with donor template and Its role in correcting Sickle Cell DiseasePreprint
Pattabhi, S., Lotti, S. N., Berger, M. P., Singh, S., & Rawlings, D. J. (2021) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2021.06.30.450644
Health-related quality of life and drug therapy problems in patients with sickle cell disease on hydroxyurea at Kenyatta National Hospital.
Kagiri, M. W. (2020)
Sickle Cell Disease
Taylor, J. (2022) · Springer eBooks · doi:10.1007/978-3-030-96542-6_58
Impact of Hydroxyurea Starting Dose on Pain Outcomes in Patients with Sickle Cell Disease
Dayer, L. E., Wagner, R., King, D., Lakkad, M., Wilson, L. A., Montgomery, C., & Painter, J. T. (2022) · Journal of Pain & Palliative Care Pharmacotherapy · doi:10.1080/15360288.2022.2128154
The Association of Sleep Quality, Sociodemographic, and Disease‐Related Factors in Youth Living With Sickle Cell Disease
Tucker, T. D., Padmanabhan, D., Alishlash, A., Mrug, S., Lebensburger, J. D., Thomas, S., Guest, K. C., & Murdaugh, D. L. (2025) · Pediatric Blood & Cancer · doi:10.1002/pbc.32115
Can platelet to neutrophil ratio (PNR) serve as a viable alternative tool for monitoring sickle cell disease patients on hydroxyurea in low income countries?
Chibuife, E. C., Adaobi, N.-E. C., Odini, F., Deepika, H. D., Aduni, N.-B., & Andrew, C. (2025) · Annals of Hematology · doi:10.1007/s00277-025-06598-2
“Does the dose of hydroxyurea correlate with shorter hospital stay and higher fetal hemoglobin levels in patients with sickle cell disease?”
Alzahrani, M. F., Albacker, A. B., Alshabanat, A., Alharbi, M., Abuhaimed, J. M., Arnous, N. K., Alzahrani, Y., Aloraier, H. A., Alamri, M. Z., Altamimi, R., et al. (2026) · Future Science OA · doi:10.1080/20565623.2025.2610223
Roxadustat induced fetal hemoglobin in anemic CKD patients: a dual-center real-world observational cohort study
Gou, W., Chen, M., Li, Z., Tan, C., Hu, L., Wang, X., Jiang, Y., Yu, T., Yin, X., Yin, X., et al. (2026) · BMC Nephrology · doi:10.1186/s12882-026-04860-x
Facteurs psychosociaux influençant l’observance thérapeutique des adultes atteints de drépanocytose
Doglioni, D. O. (2021) · theses.fr (ABES) · doi:10.70675/9897b5b1z2953z487cza41aza3d226b9216f
AI-assisted decision support for sickle cell disease severity stratification using routine blood tests: a systematic review and meta-analysis
Ali, N., Mehdi, M. A. H., Abdullah, R. S., Ali, G. S., Ali, H. M., Gubran, A. N. M., & Al-Abd, N. (2026) · BMC Medical Informatics and Decision Making · doi:10.1186/s12911-026-03679-8
Perspective Chapter: Fetal Hemoglobin Modulators
Walke, V. A. (2026) · IntechOpen eBooks · doi:10.5772/intechopen.1017037
Evaluation of The Use of Hydroxyurea in Treating Children With Sickle Cell Anemia in Central Africa's Rural Area
Mbayabo, G., Lumbala, P. K., Ngole, M., Lumaka, A. Z., Race, V., Matthijs, G., Mikobi, T. M., Devriendt, K., Geet, C. V., & Lukusa-Tshilobo, P. (2026) · eJHaem · doi:10.1002/jha2.70378
Systematic Data Fitness Assessment Improves Validity and Replicability of Research Using Real-World DataPreprint
Razzaghi, H., Wieand, K., Pinkney, A., & Bailey, L. C. (2026) · medRxiv · doi:10.64898/2026.08.05.26359818
Holographic cytometry assesses therapeutic effects in sickle cell disease
Highland, R. E., Wang, W., Chen, C. X., Fan, J., Delahunty, M. D., Miller, D. A., Telen, M. J., & Wax, A. P. (2026) · Blood Red Cells & Iron · doi:10.1016/j.brci.2026.100116
Self-citation
BindingDB Entry 50021967: Structure-based development of novel substrate-type G9a inhibitors as epigenetic modulators for sickle cell disease treatment.
BindingDB (2025) · UC San Diego · doi:10.7270/q25t3r5x

Higher Fetal Hemoglobin Following Escalation of Hydroxyurea to Maximum Tolerated Dose Provides Clinical Benefit to Children with Sickle Cell Anemia
Estepp, J. H., Smeltzer, M. P., Kang, G., Aygün, B., Ware, R. E., & Nottage, K. A. (2014) · Blood · doi:10.1182/blood.v124.21.85.85 · Cited by 7 · see citing works
Self-citation
Clinical and Laboratory Effects of Hydroxyurea in Children and Adolescents with Sickle Cell Anemia: A Portuguese Hospital Study
Braga, L., Ferreira, A. C., Guimarães, M., Nazario, C., Pacheco, P. R., Miranda, A., Picanço, I., Seixas, T., Rosado, L., & Amaral, J. M. V. (2005) · Hemoglobin · doi:10.1081/hem-200066299 · Cited by 14 · see citing works
The modern use of hydroxyurea for children with sickle cell anemia
Quinn, C. T., & Ware, R. E. (2025) · Haematologica · doi:10.3324/haematol.2023.284633 · Cited by 29 · see citing works
Self-citation
Hydroxyurea for treatment of severe sickle cell anemia: a pediatric clinical trial
Ferster, A., Vermylen, C., Cornu, G., Buyse, M. E., Corazza, F., Devalck, C., Fondu, P., Toppet, M., & Sariban, E. (1996) · Blood · doi:10.1182/blood.v88.6.1960.bloodjournal8861960 · Cited by 276 · see citing works
Hydroxyurea at Maximal Tolerated Dose (MTD) Prior to Completion of the β-Globin Switch Has Additive but Not Sustained Benefits in Fetal Hemoglobin Production
Abrams, C. M., Moen, J., Kang, G., Wang, W. C., Hankins, J. S., & Estepp, J. H. (2016) · Blood · doi:10.1182/blood.v128.22.125.125
Self-citation

Authors: Estepp, J. H., Smeltzer, M. P., Kang, G., Li, C., Wang, W. C., Abrams, C., Aygun, B., Ware, R. E., Nottage, K., & Hankins, J. S.
Affiliations: St. Jude Children's Research Hospital (US)
University of Memphis (US)
University of Tennessee Health Science Center (US)
Division of Hematology/ Oncology Steven and Alexandra Cohen Children's Medical Center New Hyde Park New York
Cincinnati Children's Hospital Medical Center (US)
Janssen Research & Development (United States) (US)
Corresponding author: Estepp, Jeremie H.
ORCID: Estepp, Jeremie H.
Smeltzer, Matthew P.
Kang, Guolian
Li, Chen
Wang, Winfred C.
Abrams, Christina
Ware, Russell E.
Hankins, Jane S.
Venue: American Journal of Hematology
Publisher: Wiley
Published: 2017-09-28
Volume: 92
Issue: 12
Pages: 1333-1339
ISSN: 0361-8609 (ISSN-L)
1096-8652
DOI: 10.1002/ajh.24906
PMC: PMC5675769
OpenAlex: W2755262585
Semantic Scholar: a34199eaa09261ecdbaeb71d79763492a29c10df
Cited by: 88
References: 46
Citations by year: 2026: 9 · 2025: 7 · 2024: 8 · 2023: 8 · 2022: 9 · 2021: 19 · 2020: 12 · 2019: 9 · 2018: 7
FWCI: 4.40
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations: American Journal of Hematology (publishedVersion)
PubMed (publishedVersion)
Europe PMC (PubMed Central) (acceptedVersion)
PubMed Central (acceptedVersion)
Funders: National Heart, Lung, and Blood Institute (R01 HL‐0990941, R34 HL‐127162)
Topics: Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
Health Sciences › Medicine › Hematology › Blood groups and transfusion
Health Sciences › Medicine › Hematology › Hemophilia Treatment and Research
Keywords: sickle cell disease, hydroxyurea, fetal hemoglobin, pediatric patients, maximum tolerated dose, hospitalization, vaso-occlusive pain, acute chest syndrome, fever, neutropenia
MeSH terms: Adolescent, Anemia, Sickle Cell, Antisickling Agents, Blood Cell Count, Child, Child, Preschool, Dose-Response Relationship, Drug, Female, Fetal Hemoglobin, Hospitalization, Humans, Hydroxyurea, Infant, Male, Prospective Studies, Maximum Tolerated Dose

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

A clinically meaningful fetal hemoglobin threshold for children with sickle cell anemia during hydroxyurea therapy

Abstract

Integrity signals

Claims the paper makes

Claims other papers make about this one

Self-check

Cites

Cited by

Related

Record

About these checks