Review
Biochemistry & Molecular Biology
Pagona Flevari, Ersi Voskaridou, Frederic Galacteros, Giovanna Cannas, Gylna Loko, Laure Joseph, Pablo Bartolucci, Justine Gellen-Dautremer, Emmanuelle Bernit, Corine Charneau, Anoosha Habibi
Summary: This article reports a case of myelodysplastic syndrome and highlights the lack of significant conclusions due to the scarcity of similar cases.
Review
Immunology
Apoorva Pandey, Harpreet Kaur, Sapan Borah, Naveen Khargekar, Vijay Kumar Karra, Tulsi Adhikari, Dipty Jain, Manisha Madkaikar
Summary: This systematic review assessed the efficacy and toxicity of hydroxyurea (HU) in Indian sickle cell patients. The results showed that low-dose HU can reduce the rates of vaso-occlusive crisis and hospitalization, as well as decrease the requirement of blood transfusion in SCD patients. However, there is still insufficient information to determine the long-term or major adverse effects of HU therapy on organ damage, fertility, and pregnancy.
INDIAN JOURNAL OF MEDICAL RESEARCH
(2022)
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
Hematology
Shehu U. Abdullahi, Surayya Sunusi, Mohammed Sani Abba, Saifuddeen Sani, Hauwau Aminu Inuwa, Safiya Gambo, Awwal Gambo, Bilya Musa, Brittany V. Covert Greene, Adetola A. Kassim, Mark Rodeghier, Nafiu Hussaini, Mariana Ciobanu, Muktar H. Aliyu, Lori C. Jordan, Michael R. DeBaun
Summary: This study investigated the effectiveness of fixed oral moderate-dose hydroxyurea compared with fixed oral low-dose hydroxyurea for secondary stroke prevention in children with sickle cell anemia. The results showed that there was no evidence to support the superiority of moderate-dose hydroxyurea in reducing the risk of stroke or death compared to low-dose hydroxyurea.
Review
Medicine, Research & Experimental
Ibrahim Khamees, Fateen Ata, Hassan Choudry, Ashraf T. Soliman, Vincenzo De Sanctis, Mohamed A. Yassin
Summary: Sickle cell disease is commonly encountered in Africa and the Middle East, leading to a spectrum of clinical manifestations and complications. Studies on a rare genotypic variant, HBSE, show both benign and severe complications, including vaso-occlusive crises and strokes.
JOURNAL OF TRANSLATIONAL MEDICINE
(2021)
Article
Hematology
Andrew M. Heitzer, Jennifer Longoria, Victoria Okhomina, Winfred C. Wang, Darcy Raches, Brian Potter, Lisa M. Jacola, Jerlym Porter, Jane E. Schreiber, Allison A. King, Guolian Kang, Jane S. Hankins
Summary: Neurocognitive impairment is common in sickle cell disease, and this study found that older age was associated with a decrease in overall intelligence quotient for patients with HbSS/HbS beta(0)-thalassaemia. Early initiation of hydroxyurea treatment was associated with higher neurocognitive scores across most domains, suggesting its potential in limiting the detrimental effects of SCD on cognitive functions.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Hematology
Philippe Connes, Berenike Mockesch, Emilienne Tudor Ngo Sock, Marie-Dominique Hardy-Dessources, Karen Reminy, Sarah Skinner, Marie Billaud, Elie Nader, Benoit Tressieres, Maryse Etienne-Julan, Nicolas Guillot, Nathalie Lemonne, Olivier Hue, Marc Romana, Sophie Antoine-Jonville
Summary: The study compared plasma markers of inflammation and oxidative stress, as well as microvascular function, between patients with sickle cell disease of different genotypes and under hydroxyurea (HU) treatment or not. Results showed that oxidative stress and inflammation were lower in HbSC patients and HbSS patients receiving HU treatment compared to HbSS patients not treated with HU.
EUROPEAN JOURNAL OF HAEMATOLOGY
(2021)
Review
Hematology
Adrienne D. Mishkin, Elizabeth J. Prince, Elizabeth J. Leimbach, Markus Y. Mapara, C. Patrick Carroll
Summary: Although there is increasing awareness of the impact of sickle cell disease (SCD) on quality of life and mood, there is limited knowledge about the prevalence, presentation, and treatment of psychiatric illness in adults with SCD. This narrative review examines the current understanding of common psychiatric conditions such as depression, anxiety, and cognitive impairment in this population. The review highlights the need for further research, makes provisional recommendations, and identifies areas for improvement in care.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Hematology
Ruth Namazzi, Robert Opoka, Andrea L. Conroy, Dibyadyuti Datta, Abner Tagoola, Caitlin Bond, Michael J. Goings, Moon-Suhn Ryu, Sarah E. Cusick, Nancy F. Krebs, Jeong Hoon Jang, Wanzhu Tu, Russell E. Ware, Chandy C. John
Summary: Data from small clinical trials show that zinc supplementation reduces infection in adolescents and adults with sickle cell anemia (SCA). However, there is a lack of studies on the effects of zinc supplementation for infection prevention in children with SCA in Africa. This randomized trial in Ugandan children aged 1.00-4.99 years with SCA found that daily zinc supplementation did not prevent severe or invasive infections. Zinc deficiency was also prevalent among the supplemented children. Further investigation is needed to determine the optimal zinc dosage and the role of zinc in preventing stroke or death in SCA.
Article
Pharmacology & Pharmacy
Akancha Pandey, Jeremie H. Estepp, Rubesh Raja, Guolian Kang, Doraiswami Ramkrishna
Summary: This study developed pharmacokinetics and pharmacodynamics models to investigate the dosing and efficacy of hydroxyurea treatment in children with sickle cell disease (SCD). The models provided excellent fits for individuals with known or correctly inferred adherence, and can be used to determine optimal dosing regimens and study the impact of non-adherence on treatment outcomes.
Article
Biochemistry & Molecular Biology
Anoosha Habibi, Giovanna Cannas, Pablo Bartolucci, Ersi Voskaridou, Laure Joseph, Emmanuelle Bernit, Justine Gellen-Dautremer, Corine Charneau, Stephanie Ngo, Frederic Galacteros
Summary: Sickle cell disease is a group of inherited hemoglobin disorders characterized by altered deformability of red blood cells and various complications. Hydroxyurea is a major therapeutic agent for sickle cell patients, but its safety during pregnancy is not well-established. This study aims to describe pregnancy outcomes in the European Sickle Cell Disease COhoRT-HydroxyUrea study and share the results with the scientific community.
Article
Medicine, General & Internal
Catherine Segbefia, Seth Kwabena Amponsah, Adwoa K. A. Afrane, Mame Yaa Nyarko, Yvonne Brew, Nihad Salifu, Samuel Yao Ahorhorlu, Abdul Malik Sulley, Lars Hviid, Michael Fokuo Ofori, George Obeng Adjei
Summary: In this study, interactions between hydroxyurea (HU) and antimalarials in sickle cell disease (SCD) were explored. The study found that parasite clearance and adverse event occurrence were similar in SCD children treated with artemether-lumefantrine (AL) regardless of HU status. However, distinct patterns of changes in laboratory indices suggest the potential need for larger, more focused studies in the future.
FRONTIERS IN MEDICINE
(2023)
Article
Hematology
Innocent Safeukui, Russell E. Ware, Narla Mohandas, Kasturi Haldar
Summary: Effective treatment for genetic disorders coevolved with pathogens requires improvement of both conditions. Hydroxyurea (HU) is a safe and effective treatment for sickle cell anemia (SCA) and also reduces clinical malaria. In vitro studies show that HU has significant killing activity against Plasmodium falciparum, the genetic driver of SCA, and it shows additive activity with commonly used antimalarial treatments.
Article
Hematology
David C. Rees, Yurdanur Kilinc, Selma Unal, Carlton Dampier, Betty S. Pace, Banu Kaya, Sara Trompeter, Isaac Odame, Johnny Mahlangu, Sule Unal, Julie Brent, Regine Grosse, Beng R. Fuh, Baba P. D. Inusa, Ariel Koren, Goksel Leblebisatan, Carina Levin, Elizabeth McNamara, Karin Meiser, Douglas Hom, Stephen J. Oliver
Summary: This study found that selective IL-1 beta blockade by canakinumab in patients with sickle cell anemia can reduce inflammation markers, clinical events, and hospitalizations, with potential improvements in symptoms such as pain intensity.
Review
Hematology
Russell E. Ware, Stephen D. Dertinger
Summary: Hydroxyurea is effective in treating SCA by inducing HbF and improving symptoms, but concerns about its mutagenic and carcinogenic risks have limited its broad use. However, current data suggest that hydroxyurea does not directly bind DNA or exhibit mutagenic activity.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Health Policy & Services
Melissa K. Ward, Sofia B. Fernandez, Diana M. Sheehan, Tan Li, Rahel Dawit, Kristopher Fennie, Mary Catherine Beach, Petra Brock, Robert Ladner, Mary Jo Trepka
Summary: This exploratory study examined the sex differences and psychosocial and demographic factors associated with sustained HIV viral suppression. The study found that a higher proportion of females did not achieve sustained viral suppression compared to males. For females, perinatal HIV acquisition and lack of a partner or adult household member were associated with not achieving sustained viral suppression. For males, lack of access to food, Black or Haitian race/ethnicity, problematic substance use, and unknown physician were associated with not achieving sustained viral suppression.
AIDS CARE-PSYCHOLOGICAL AND SOCIO-MEDICAL ASPECTS OF AIDS/HIV
(2023)
Article
Oncology
Megan A. Askew, Arlene M. Smaldone, Melanie A. Gold, Kim Smith-Whitley, John J. Strouse, Zhezhen Jin, Nancy S. Green
Summary: This study aimed to assess the contraceptive practices of pediatric hematology providers for female adolescents and young adults with sickle cell disease (SCD). The results showed that there were variations in provider contraceptive practices, especially based on provider status. Key motivators included patient request and disclosure of sexual activity, while key barriers included inadequate provider training and limited visit time.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Psychology, Multidisciplinary
Charity Oyedeji, John J. Strouse, Rita Masese, Nathan Gray, Tolu O. Oyesanya
Summary: This qualitative descriptive study explored the perspectives of older adults with sickle cell disease (SCD) on preparing for death. Four themes emerged: anticipation of early death, near death experiences, differences in level of comfort with death, and the influence of spirituality. The findings will inform interventions to improve patient-provider communication and provide goal-concordant end-of-life care for adults with SCD.
OMEGA-JOURNAL OF DEATH AND DYING
(2022)
Review
Public, Environmental & Occupational Health
Anna Russell, J. Lee Jenkins, Allen Zhang, Lisa M. Wilson, Eric B. Bass, Edbert B. Hsu
Summary: This review examined the prevalence, incidence, and severity of infections among the emergency medical service (EMS) workforce. The majority of studies focused on SARS-CoV-2 infection, with varying prevalence rates. MRSA and Hepatitis C infections were also reported. Limited studies reported incidence rates. Differences in infection rates were observed between EMS clinicians and firefighters, with higher hospitalization or death rates for SAR-CoV-2 and higher prevalence of Hepatitis C among EMS clinicians. More research is needed to understand the incidence and severity of occupationally-acquired infections in the EMS workforce.
AMERICAN JOURNAL OF INFECTION CONTROL
(2023)
Article
Public, Environmental & Occupational Health
Jillian H. H. Hurst, Amanda Brucker, Congwen Zhao, Hannah Driscoll, Haley P. P. Hostetler, Michael Phillips, Bari Rosenberg, Marc D. D. Samsky, Isaac Smith, Megan E. E. Reller, John J. J. Strouse, Cindy Ke Zhou, Graca M. Dores, Hui-Lee Wong, Benjamin A. A. Goldstein
Summary: Detecting adverse reactions to drugs and biologic agents is important for regulatory approval and post-market safety evaluation. Real-world data, including insurance claims and electronic health records data, are increasingly being used for evaluating potential safety outcomes. However, different types of data elements within these resources can affect the development and performance of computable phenotypes for identifying adverse events (AEs).
Review
Geriatrics & Gerontology
Charity I. I. Oyedeji, Kimberly L. L. Hodulik, Marilyn J. J. Telen, John J. J. Strouse
Summary: People with sickle cell disease are living longer due to various initiatives and improvements in treatment. However, there is a lack of evidence and guidance on how to manage older adults with this condition, as most studies have focused on younger age groups.
Article
Neurosciences
Arvind P. Subramaniam, Charity I. Oyedeji, Jhana S. Parikh, Jody A. Feld, John J. Strouse
Summary: This study aimed to investigate the feasibility and safety of dual-task assessment (DTA) in measuring physical and cognitive function in adults with sickle cell disease (SCD). The results showed that most participants were able to complete the DTA without any adverse events. Specific patterns of cognitive-motor interference were identified. This study supports further evaluation of DTA as a potentially useful tool in assessing physical and cognitive function in ambulatory adults with SCD.
Editorial Material
Medicine, General & Internal
Clement D. Lee, Owen Bradfield, Michelle M. Mello, Mary Catherine Beach
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Article
Health Care Sciences & Services
Ofelia A. Alvarez, Hector Rodriguez-Cortes, E. Leila Jerome Clay, Sandra Echenique, Julie Kanter, John J. Strouse, Talia Buitrago-Mogollon, Cheryl Courtlandt, Laura Noonan, Ifeyinwa Osunkwo
Summary: The EMBRACE SCD project aimed to increase the prescription rate of hydroxyurea (HU) for children with sickle cell anemia (SCA) through a quality improvement framework and patient education. The project successfully increased the prescription rate of HU by assessing reasons for acceptance and refusal of HU.
BMJ QUALITY & SAFETY
(2023)
Editorial Material
Medicine, General & Internal
Suzie A. Noronha, John J. Strouse
Letter
Hematology
Leena Nahata, Gwendolyn P. Quinn, John J. Strouse, Susan E. Creary
Article
Health Care Sciences & Services
Matthew P. Smeltzer, Sarah L. Reeves, William O. Cooper, Brandon K. Attell, John J. Strouse, Clifford M. Takemoto, Julie Kanter, Krista Latta, Allison P. Plaxco, Robert L. Davis, Daniel Hatch, Camila Reyes, Kevin Dombkowski, Angela Snyder, Susan Paulukonis, Ashima Singh, Mariam Kayle
Summary: Population-level data on sickle cell disease (SCD) in the United States is limited. The Centers for Disease Control and Prevention (CDC) has implemented the Sickle Cell Data Collection Programs (SCDC) to address this issue by developing a common informatics infrastructure and data model for standardized reporting. The model allows for the comparison of data across states and the compilation of core surveillance reports. This pilot project serves as a blueprint for similar initiatives in other rare diseases.
Article
Emergency Medicine
Paula Tanabe, Stephanie Ibemere, Ava E. Pierce, Caroline E. Freiermuth, Hayden B. Bosworth, Hongqui Yang, Ifeyinwa Osunkwo, James H. Paxton, John J. Strouse, Joseph Miller, Judith A. Paice, Padmaja Veeramreddy, Patricia L. Kavanagh, R. Gentry Wilkerson, Robert Hughes, Huiman X. Barnhart
Summary: This study compared the effectiveness of a patient-specific pain protocol (PSP) and weight-based (WB) dosing of opioid medication in relieving the pain caused by vaso-occlusive crises. The results showed that both groups experienced significant pain relief without significant side effects. The PSP group had a shorter length of stay in the emergency department.
ACADEMIC EMERGENCY MEDICINE
(2023)
Article
Education & Educational Research
Hosam H. Alkhatib, Mary Catherine Beach, Kelly A. Gebo, Eric B. Bass, Jenny R. Park, Meredith A. Atkinson, Sapna R. Kudchadkar, Stephen M. Sozio
Summary: This study examined the association between medical school Scholarly Concentrations (SC) programs and students' specialty choices. The results showed that students' interest in a specialty at baseline and their research productivity with SC mentors were associated with increased odds of entering a related specialty. However, completing an SC project did not increase the odds of matching into that specialty or a higher ranked program.
MEDICAL EDUCATION ONLINE
(2023)