Sickle Cell Disease in Children and Adolescents: A Review of the Historical, Clinical, and Public Health Perspective of Sub‐Saharan Africa and Beyond
Sickle cell disease (SCD) is an umbrella term for a group of life‐long debilitating autosomal
recessive disorders that are caused by a single‐point mutation (Glu→ Val) that results in …
recessive disorders that are caused by a single‐point mutation (Glu→ Val) that results in …
[HTML][HTML] A cell stress signaling model of fetal hemoglobin induction: what doesn't kill red blood cells may make them stronger
R Mabaera, RJ West, SJ Conine, ER Macari… - Experimental …, 2008 - Elsevier
A major goal of hemoglobinopathy research is to develop treatments that correct the
underlying molecular defects responsible for sickle cell disease and β-thalassemia. One …
underlying molecular defects responsible for sickle cell disease and β-thalassemia. One …
Barriers to therapeutic use of hydroxyurea for sickle cell disease in Nigeria: a cross-sectional survey
Background: Sickle cell disease, the inherited blood disorder characterized by anemia,
severe pain and other vaso-occlusive complications, acute chest syndrome …
severe pain and other vaso-occlusive complications, acute chest syndrome …
Induction of human fetal hemoglobin via the NRF2 antioxidant response signaling pathway
ER Macari, CH Lowrey - Blood, The Journal of the American …, 2011 - ashpublications.org
Although hematopoietic stem cell transplantation and gene therapy have the potential to
cure β-thalassemia and sickle cell disease, they are not currently available to most people …
cure β-thalassemia and sickle cell disease, they are not currently available to most people …
Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled …
Background Despite~ 90% of sickle cell disease (SCD) occurring in low-and middle-income
countries (LMICs), the vast majority of people are not receiving evidence-based …
countries (LMICs), the vast majority of people are not receiving evidence-based …
Simvastatin and t-butylhydroquinone suppress KLF1 and BCL11A gene expression and additively increase fetal hemoglobin in primary human erythroid cells
ER Macari, EK Schaeffer, RJ West… - Blood, The Journal of …, 2013 - ashpublications.org
Although increased fetal hemoglobin (HbF) levels have proven benefit for people with β-
hemoglobinopathies, all current HbF-inducing agents have limitations. We previously …
hemoglobinopathies, all current HbF-inducing agents have limitations. We previously …
Hydroxyurea in children with sickle cell disease in a resource‐poor setting: Monitoring and effects of therapy. A practical perspective
U Nnebe‐Agumadu, I Adebayo… - Pediatric Blood & …, 2021 - Wiley Online Library
Background Although effectiveness of hydroxyurea (HU) in sickle cell disease is well
established, unanswered questions persist about its use in African children. We determined …
established, unanswered questions persist about its use in African children. We determined …
Level of utilization and provider-related barriers to the use of hydroxyurea in the treatment of sickle cell disease patients in Jos, North-Central Nigeria
Background: Hydroxyurea is underutilized by sickle cell health-care providers in Nigeria
despite available evidence of its effectiveness in reducing the manifestations and …
despite available evidence of its effectiveness in reducing the manifestations and …
Improvement of SCD morbimortality in children: experience in a remote area of an African country
BM Mbiya, DK Kalombo, YN Mukendi, V Daubie… - BMC health services …, 2021 - Springer
Background Sickle cell disease (SCD) is a public health problem in the Democratic Republic
of Congo. While reference sickle cell centers have been implemented in capital cities of …
of Congo. While reference sickle cell centers have been implemented in capital cities of …
Understanding patient-related barriers to hydroxyurea use among adolescent and adult patients with sickle cell disease in Mulago and Kiruddu hospitals, Uganda, a …
P Namaganda, P Nantume, KR Mubiru… - BMC Health Services …, 2024 - Springer
Abstract Background In 2016, Uganda added Hydroxyurea (HU) to the list of essential drugs
to treat sickle cell disease SCD. However, Hydroxyurea utilization has been low for several …
to treat sickle cell disease SCD. However, Hydroxyurea utilization has been low for several …