Article
Pediatrics
Asmaa M. Zahran, Khaled Saad, Khalid Elsayh, Shaimaa M. Khalaf, Khaled Hashim Mahmoud, Amira Elhoufey, Helal F. Hetta
Summary: Among children with SCD, HU treatment exhibited significant qualitative and quantitative effects on Tregs. HU treatment in SCD decreases the frequency of Tregs, as well as the levels of the most suppressive Tregs: HLA-DR+, CD39+, and CD69+. At the same time, HU increases the proportion of naive CD45RA+ Tregs. Our study showed the impact of HU therapy on Tregs in children with SCD.
PEDIATRIC RESEARCH
(2022)
Article
Biochemistry & Molecular Biology
Mariana Delgadinho, Catarina Ginete, Brigida Santos, Carolina Fernandes, Carina Silva, Armandina Miranda, Jocelyne Neto de Vasconcelos, Miguel Brito
Summary: This study investigated the impact of hydroxyurea treatment on the gut microbiome of Angolan children with sickle cell anemia (SCA). The results showed that hydroxyurea increased the abundance of beneficial bacteria and reduced the abundance of pathogenic bacteria. These microorganisms could potentially serve as candidate biomarkers for disease severity and hydroxyurea efficacy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Hematology
Yan Zheng, Jeffrey M. Gossett, Pei-Lin Chen, Martha Barton, Missy Ryan, Jing Yu, Guolian Kang, Jane S. Hankins, Stella T. Chou
Summary: This study found that the inflammatory state of transfusion recipients affects the risk of red blood cell alloimmunization, which is not modified by hydroxyurea (HU) therapy. Judicious use of transfusion during proinflammatory events is critical for preventing alloimmunization.
Review
Chemistry, Medicinal
Joelma Santana dos Santos Neres, Setondji Cocou Modeste Alexandre Yahouedehou, Marilda Souza Goncalves
Summary: Inconsistent therapeutic responses have been observed in SCA patients undergoing HU treatment. Personalized dosages based on individual pharmacokinetic profiles have shown improved effectiveness. However, further research is needed in this area.
Article
Multidisciplinary Sciences
Rita Masese, Dominique Bulgin, Mitchell R. Knisely, Liliana Preiss, Eleanor Stevenson, Jane S. Hankins, Marsha J. Treadwell, Allison A. King, Victor R. Gordeuk, Julie Kanter, Robert Gibson, Jeffrey A. Glassberg, Paula Tanabe, Nirmish Shah
Summary: The study found that female patients had worse reports of pain severity, more vaso-occlusive episodes, and a higher occurrence of 3 or more hospital admissions in the past year, while male patients were more likely to experience acute chest syndrome, cardiovascular and musculoskeletal complications, but less likely to have depression.
Article
Biochemistry & Molecular Biology
Catarina Ginete, Mariana Delgadinho, Brigida Santos, Vera Pinto, Carina Silva, Armandina Miranda, Miguel Brito
Summary: Sickle cell anemia is a common inherited disease in sub-Saharan Africa with heterogeneous phenotypes. Hydroxyurea is the main treatment for these patients, but response to treatment varies. Identifying genetic variants associated with hydroxyurea response is important for predicting treatment outcomes. A pharmacogenetic study in Angolan children identified 30 variants in 18 genes that may be associated with drug response and found associations with hematological, biochemical, and clinical parameters. Further research is needed to confirm these findings.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Health Care Sciences & Services
Daniela Anderson, Katie Lien, Chibueze Agwu, Phillip S. Ang, Nabil Abou Baker
Summary: This article explores the historical context of sickle cell disease in the United States, discusses specific issues related to biases, social and self-stigma, inequities in access to care, and research funding, and highlights recent interventions that address racial biases and stigma.
JOURNAL OF GENERAL INTERNAL MEDICINE
(2023)
Article
Hematology
Monica L. Hulbert, Melanie E. Fields, Kristin P. Guilliams, Priyesha Bijlani, Shalini Shenoy, Slim Fellah, Alison S. Towerman, Michael M. Binkley, Robert C. McKinstry, Joshua S. Shimony, Yasheng Chen, Cihat Eldeniz, Dustin K. Ragan, Katie Vo, Hongyu An, Jin-Moo Lee, Andria L. Ford
Summary: This study investigated the effect of hematopoietic stem cell transplant (HSCT) on cerebral hemodynamics in children with sickle cell disease (SCD). It found that HSCT normalized cerebral blood flow and oxygen extraction fraction in SCD children, reducing the risk of strokes.
Article
Pharmacology & Pharmacy
Abhishek Gour, Dilpreet Kour, Ashish Dogra, Diksha Manhas, Priya Wazir, Sanjeev Kumar Digra, Ajay Kumar, Utpal Nandi
Summary: This study suggests that epicatechin may be a promising supplementation therapy for the symptomatic management of sickle cell anemia (SCA) under hydroxyurea (HU) treatment. Epicatechin exhibits anti-sickling, polymerization inhibition, and erythrocyte membrane stability effects, and can reduce the adverse effects of HU. The study also found that epicatechin can enhance the plasma exposure of HU in rats.
TOXICOLOGY AND APPLIED PHARMACOLOGY
(2022)
Review
Cell Biology
Ambroise Wonkam
Summary: Sickle cell disease (SCD) is a common recessive genetic disease affecting nearly 300,000 newborns annually, with the majority in Africa. Genomics research can accelerate the development of SCD therapies by exploring the heritability of fetal hemoglobin, investing in RNA therapies, and identifying genetic risk factors for SCD cardiovascular complications.
DISEASE MODELS & MECHANISMS
(2023)
Article
Oncology
Kristine Karkoska, Kevin Todd, Omar Niss, Kelly Clapp, Lynette Fenchel, Theodosia A. Kalfa, Punam Malik, Charles T. Quinn, Russell E. Ware, Patrick T. McGann
Summary: Universal and early initiation of hydroxyurea therapy for children with sickle cell anemia has led to significant increase in utilization, younger age of initiation, and reduced SCA-related admissions, demonstrating measurable improvements in clinical outcomes. This approach can serve as a model for other programs to expand hydroxyurea use for more children with SCA.
PEDIATRIC BLOOD & CANCER
(2021)
Review
Pharmacology & Pharmacy
Min Dong, Russell E. Ware, Andre Dallmann, Alexander A. Vinks
Summary: Sickle cell anemia (SCA) is a genetic condition that can contribute to high-risk pregnancies. With improved management, SCA has become a chronic disease of adults and many patients are now considering parenthood. It is important to provide continuous management and avoid SCA-related complications for a healthy pregnancy.
Article
Biochemical Research Methods
Davis B. Zhou, Maria Castanos, Alexander Pinhas, Peter Gillette, Justin Migacz, Richard B. Rosen, Jeffrey Glassberg, Toco Y. P. Chui
Summary: This study introduces imaging metrics to capture dynamic vascular changes in sickle cell disease patients, revealing significant variations in capillary perfusion compared to controls. These measurements could be useful for monitoring disease state and evaluating treatment efficacy.
BIOMEDICAL OPTICS EXPRESS
(2021)
Review
Medical Laboratory Technology
Melissa Azul, Eudorah F. Vital, Wilbur A. Lam, David K. Wood, Joan D. Beckman
Summary: This article provides an overview of the etiology and biological consequences of sickle cell disease. It also discusses the application and potential development of microfluidic technologies in sickle cell disease research.
TRANSLATIONAL RESEARCH
(2022)
Review
Clinical Neurology
Pallab Bhattacharya, Deepaneeta Sarmah, Kunjan R. Dave, Avirag Goswami, Mitsuyoshi Watanabe, Xin Wang, Kiran Kalia, Nikolaus Plesnila, Dileep R. Yavagal, Ofelia Alvarez
Summary: This comprehensive review delves into the pathophysiology, epidemiology, evaluation, and treatment of sickle cell anemia (SCA)-related stroke in both developed and developing countries. Different interventions and preventative measures are used in various countries, emphasizing the need for personalized approaches based on individual patient characteristics. Expanding the implementation of Transcranial Doppler ultrasound (TCD) should be a priority in developing countries where resources may be limited.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2021)
Letter
Environmental Sciences
Pedro Aurio Maia Filho, Tarcisio Paulo de Almeida Filho, Caroline de Fatima Aquino Moreina-Nunes, Rommel Rodriguez Burbano, Jose Alexandre Rodrigues de Lemos, Edivaldo Herculano Correa de Oliveira, Bruno Coelho Cavalcanti, Jamilly Florencio Pereira, Maritza Cavalcante Barbosa, Fernando Barroso Duarte, Marilena Facundo de Castro, Acy Telles de Souza Quixada, Romelia Pinheiro Goncalves Lemes
ENVIRONMENTAL AND MOLECULAR MUTAGENESIS
(2018)