Literature DB >> 29318647

Realizing effectiveness across continents with hydroxyurea: Enrollment and baseline characteristics of the multicenter REACH study in Sub-Saharan Africa.

Patrick T McGann1, Thomas N Williams2,3, Peter Olupot-Olupot4, George A Tomlinson5, Adam Lane1, José Luís Reis da Fonseca6, Robert Kitenge7, George Mochamah2, Ham Wabwire4, Susan Stuber1, Thad A Howard1, Kathryn McElhinney1, Banu Aygun8, Teresa Latham1, Brígida Santos6, Léon Tshilolo7, Russell E Ware1.   

Abstract

Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, ClinicalTrials.gov NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries. Following comprehensive training of local research teams, REACH was approved by local Ethics Committees and achieved full enrollment ahead of projections with 635 participants enrolled over a 30-month period, despite half of families living >12 km from their clinical site. At enrollment, study participants (age 5.4 ± 2.4 years) had substantial morbidity, including a history of vaso-occlusive pain (98%), transfusion (68%), malaria (85%), and stroke (6%). Significant differences in laboratory characteristics were noted across sites, with lower hemoglobin concentrations (P < .01) in Angola (7.2 ± 1.0 g/dL) and the DRC (7.0 ± 0.9 g/dL) compared to Kenya (7.4 ± 1.1 g/dL) and Uganda (7.5 ± 1.1 g/dL). Analysis of known genetic modifiers of SCA demonstrated a high frequency of α-thalassemia (58.4% with at least a single α-globin gene deletion) and G6PD deficiency (19.7% of males and 2.4% of females) across sites. The CAR β-globin haplotype was present in 99% of participants. The full enrollment to REACH confirms the feasibility of conducting high-quality SCA research in Africa; this study will provide vital information to guide safe and effective dosing of hydroxyurea for children with SCA living in Africa.
© 2018 Wiley Periodicals, Inc.

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Year:  2018        PMID: 29318647      PMCID: PMC5870803          DOI: 10.1002/ajh.25034

Source DB:  PubMed          Journal:  Am J Hematol        ISSN: 0361-8609            Impact factor:   10.047


  48 in total

1.  Polymerase chain reaction amplification applied to the determination of beta-like globin gene cluster haplotypes.

Authors:  M Sutton; E E Bouhassira; R L Nagel
Journal:  Am J Hematol       Date:  1989-09       Impact factor: 10.047

2.  Is low dose hydroxyurea the solution to the global epidemic of sickle cell disease?

Authors:  John J Strouse
Journal:  Pediatr Blood Cancer       Date:  2015-03-08       Impact factor: 3.167

Review 3.  Pharmacotherapeutical strategies in the prevention of acute, vaso-occlusive pain in sickle cell disease: a systematic review.

Authors:  Joep W R Sins; David J Mager; Shyrin C A T Davis; Bart J Biemond; Karin Fijnvandraat
Journal:  Blood Adv       Date:  2017-08-22

4.  Efficacy of fixed low dose hydroxyurea in Indian children with sickle cell anemia: a single centre experience.

Authors:  Dipti L Jain; Mohini Apte; Roshan Colah; Vijaya Sarathi; Saumil Desai; Amruta Gokhale; Amol Bhandarwar; Harshwardhan L Jain; Kanjaksha Ghosh
Journal:  Indian Pediatr       Date:  2013-04-05       Impact factor: 1.411

5.  Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group.

Authors:  T R Kinney; R W Helms; E E O'Branski; K Ohene-Frempong; W Wang; C Daeschner; E Vichinsky; R Redding-Lallinger; B Gee; O S Platt; R E Ware
Journal:  Blood       Date:  1999-09-01       Impact factor: 22.113

6.  Negative epistasis between the malaria-protective effects of alpha+-thalassemia and the sickle cell trait.

Authors:  Thomas N Williams; Tabitha W Mwangi; Sammy Wambua; Timothy E A Peto; David J Weatherall; Sunetra Gupta; Mario Recker; Bridget S Penman; Sophie Uyoga; Alex Macharia; Jedidah K Mwacharo; Robert W Snow; Kevin Marsh
Journal:  Nat Genet       Date:  2005-10-16       Impact factor: 38.330

7.  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).

Authors:  Ersi Voskaridou; Dimitrios Christoulas; Antonios Bilalis; Eleni Plata; Konstantinos Varvagiannis; George Stamatopoulos; Klio Sinopoulou; Aggeliki Balassopoulou; Dimitris Loukopoulos; Evangelos Terpos
Journal:  Blood       Date:  2009-11-10       Impact factor: 22.113

8.  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.

Authors:  Dilip K Patel; Ranjeet S Mashon; Siris Patel; Bhabani S Das; Prasanta Purohit; Subasa C Bishwal
Journal:  Hemoglobin       Date:  2012       Impact factor: 0.849

9.  Genetic modifiers of sickle cell anemia in the BABY HUG cohort: influence on laboratory and clinical phenotypes.

Authors:  Vivien A Sheehan; Zhaoyu Luo; Jonathan M Flanagan; Thad A Howard; Bruce W Thompson; Winfred C Wang; Abdullah Kutlar; Russell E Ware
Journal:  Am J Hematol       Date:  2013-05-30       Impact factor: 10.047

10.  Rapid and reliable detection of α-globin copy number variations by quantitative real-time PCR.

Authors:  Runa M Grimholt; Petter Urdal; Olav Klingenberg; Armin P Piehler
Journal:  BMC Hematol       Date:  2014-01-24
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  11 in total

1.  The population dynamics of hemoglobins A, A2, F and S in the context of the hemoglobinopathies HbS and α-thalassemia in Kenyan infants.

Authors:  Alex W Macharia; Sophie Uyoga; Carolyne Ndila; Gideon Nyutu; Johnstone Makale; Metrine Tendwa; Emily Nyatichi; John Ojal; Sarah Atkinson; Thomas N Williams
Journal:  Haematologica       Date:  2018-11-22       Impact factor: 9.941

2.  Real-world experience using hydroxyurea in children with sickle cell disease in Lilongwe, Malawi.

Authors:  Tisungane Mvalo; Hillary M Topazian; Portia Kamthunzi; Jane S Chen; Isobel Kambalame; Pilirani Mafunga; Noel Mumba; Msandeni Chiume; Khadija Paseli; Gerald Tegha; Wiza Kumwenda; J Brett Heimlich; Graham Ellis; Nigel Key; Satish Gopal; Irving Hoffman; Kenneth I Ataga; Kate D Westmoreland
Journal:  Pediatr Blood Cancer       Date:  2019-08-09       Impact factor: 3.167

3.  Strategies to increase access to basic sickle cell disease care in low- and middle-income countries.

Authors:  Meghna Dua; Halima Bello-Manga; Yvonne M Carroll; Aisha Amal Galadanci; Umma Abdulsalam Ibrahim; Allison A King; Ayobami Olanrewaju; Jeremie H Estepp
Journal:  Expert Rev Hematol       Date:  2022-04-12       Impact factor: 2.819

4.  Sickle cell anaemia and severe Plasmodium falciparum malaria: a secondary analysis of the Transfusion and Treatment of African Children Trial (TRACT).

Authors:  Sophie Uyoga; Peter Olupot-Olupot; Roisin Connon; Sarah Kiguli; Robert O Opoka; Florence Alaroker; Rita Muhindo; Alexander W Macharia; Arjen M Dondorp; Diana M Gibb; A Sarah Walker; Elizabeth C George; Kathryn Maitland; Thomas N Williams
Journal:  Lancet Child Adolesc Health       Date:  2022-07-02

5.  A predictive algorithm for identifying children with sickle cell anemia among children admitted to hospital with severe anemia in Africa.

Authors:  Peter Olupot-Olupot; Roisin Connon; Sarah Kiguli; Robert O Opoka; Florence Alaroker; Sophie Uyoga; Margret Nakuya; William Okiror; Julius Nteziyaremye; Tonny Ssenyondo; Eva Nabawanuka; Juliana Kayaga; Cynthia Williams Mukisa; Denis Amorut; Rita Muhindo; Gary Frost; Kevin Walsh; Alexander W Macharia; Diana M Gibb; A Sarah Walker; Elizabeth C George; Kathryn Maitland; Thomas N Williams
Journal:  Am J Hematol       Date:  2022-02-16       Impact factor: 13.265

6.  Improvement of SCD morbimortality in children: experience in a remote area of an African country.

Authors:  Benoît Mukinayi Mbiya; Didier Kalenda Kalombo; Yannick Nkesu Mukendi; Valery Daubie; John Kalenda Mpoyi; Parola Mukendi Biboyi; Ghislain Tumba Disashi; Béatrice Gulbis
Journal:  BMC Health Serv Res       Date:  2021-04-01       Impact factor: 2.655

7.  Genotypic Diversity among Angolan Children with Sickle Cell Anemia.

Authors:  Mariana Delgadinho; Catarina Ginete; Brígida Santos; Armandina Miranda; Miguel Brito
Journal:  Int J Environ Res Public Health       Date:  2021-05-19       Impact factor: 3.390

Review 8.  Effective use of hydroxyurea for sickle cell anemia in low-resource countries.

Authors:  Alexandra Power-Hays; Russell E Ware
Journal:  Curr Opin Hematol       Date:  2020-05       Impact factor: 3.218

9.  Prospective Newborn Screening for Sickle Cell Disease and Other Inherited Blood Disorders in Central Malawi.

Authors:  Gerald Tegha; Hillary M Topazian; Portia Kamthunzi; Thad Howard; Zondwayo Tembo; Tisungane Mvalo; Nelecy Chome; Wiza Kumwenda; Tawonga Mkochi; Arielle Hernandez; Kenneth I Ataga; Irving F Hoffman; Russell E Ware
Journal:  Int J Public Health       Date:  2021-06-29       Impact factor: 3.380

10.  β-Thalassemia pathogenic variants in a cohort of children from the East African coast.

Authors:  Alexander W Macharia; George Mochamah; Sophie Uyoga; Carolyne M Ndila; Gideon Nyutu; Metrine Tendwa; Emily Nyatichi; Johnstone Makale; Russell E Ware; Thomas N Williams
Journal:  Mol Genet Genomic Med       Date:  2020-05-11       Impact factor: 2.473

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