Article
Peripheral Vascular Disease
Takanari Kitazono, Kazunori Toyoda, Kazuo Kitagawa, Takehiko Nagao, Hiroshi Yamagami, Shinichiro Uchiyama, Norio Tanahashi, Masayasu Matsumoto, Kazuo Minematsu, Izumi Nagata, Masakatsu Nishikawa, Shinsuke Nanto, Yasuo Ikeda, Toshiaki Shirai, Kenji Abe, Akira Ogawa
Summary: The efficacy of prasugrel may vary among different stroke subtypes, indicating the need for further studies.
JOURNAL OF ATHEROSCLEROSIS AND THROMBOSIS
(2021)
Article
Multidisciplinary Sciences
El Hadji Malick Ndour, Khuthala Mnika, Fatou Gueye Tall, Moussa Seck, Indou Deme Ly, Victoria Nembaware, Gaston Kuzamunu Mazandu, Helene Ange Therese Sagna Bassene, Rokhaya Dione, Aliou Abdoulaye Ndongo, Jean Pascal Demba Diop, Nene Oumou Kesso Barry, Moustapha Djite, Rokhaya Ndiaye Diallo, Papa Madieye Gueye, Saliou Diop, Ibrahima Diagne, Aynina Cisse, Ambroise Wonkam, Philomene Lopez Sall
Summary: This study showed a relatively high proportion of sickle cell nephropathy among patients living with sickle cell anemia in Senegal. Microglucosuria, proteinuria, tubular proteinuria, microalbuminuria, hyposthenuria, and glomerular hyperfiltration were the most prevalent biomarkers of nephropathy in this group of Senegalese patients with sickle cell anemia.
Article
Hematology
Matthew M. Heeney, Miguel R. Abboud, Jessie Githanga, Baba P. D. Inusa, Julie Kanter, Alan D. Michelson, Videlis Nduba, Victor Musiime, Mohini Apte, Adlette Inati, Amar M. Taksande, Marielle Andersson, Magnus astrand, Noha Maklad, Mohammad Niazi, Anders Himmelmann, Anders R. Berggren
Summary: The study aimed to assess the efficacy and safety of ticagrelor in preventing vaso-occlusive crises in pediatric patients with sickle cell disease. However, the results showed that ticagrelor did not reduce the occurrence of vaso-occlusive crises compared to placebo.
Article
Biochemistry & Molecular Biology
Paul W. Buehler, Delaney Swindle, David Pak, Scott K. Ferguson, Susan M. Majka, Vijaya Karoor, Radu Moldovan, Chantal Sintas, Jennifer Black, Thomas Gentinetta, Raphael M. Buzzi, Florence Vallelian, Andreas Wassmer, Monika Edler, Joseph Bain, Daniel Schu, Kathryn Hassell, Rachelle Nuss, Dominik J. Schaer, David C. Irwin
Summary: Hemopexin (Hpx) serves as a crucial defense protein against heme stress in sickle cell disease (SCD) patients, with potential therapeutic benefits in improving cardiopulmonary function and exercise tolerance. Regular administrations of Hpx were found to dose-dependently reduce heme exposure and pulmonary hypertension, while also improving cardiac function and reducing fibrosis and oxidative damage in the lungs and heart. Observations in a murine SCD model support the translational potential of Hpx replacement therapy in SCD patients.
FREE RADICAL BIOLOGY AND MEDICINE
(2021)
Article
Hematology
Simona Stivala, Sara Gobbato, Nicole Bonetti, Giovanni G. Camici, Thomas F. Luescher, Juerg H. Beer
Summary: In summary, the study found that a high-ALA diet could reduce the number of sickle cells in blood smear, liver fibrosis, and expression of adhesion molecules on the endothelium. It also blunted specific parameters of platelet activation and reduced the formation of neutrophil-platelet aggregates, suggesting that dietary ALA may represent a potential therapeutic strategy for SCD.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Amina Nardo-Marino, Andreas Glenthoj, John N. Brewin, Jesper Petersen, Thomas H. Braunstein, Jorgen A. L. Kurtzhals, Thomas N. Williams, David C. Rees
Summary: This study examined spleen size and function in children with sickle cell anemia. The results showed a high prevalence of spleen dysfunction, which was not related to hemoglobin F levels or hydroxyurea treatment. Additionally, there was a strong association between erythrocyte deformability, spleen size, and function.
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Article
Pediatrics
Evans M. Machogu, Monica Khurana, Jennifer Kaericher, Charles C. Clem, James E. Slaven, Joseph E. Hatch, Stephanie D. Davis, Stacey Peterson-Carmichael
Summary: The lung clearance index (LCI) derived from the multiple breath washout test (MBW) did not differentiate sickle cell disease (SCD) from healthy controls in children between the ages of 6 and 18 years. Total lung capacity (TLC) may be a more important pulmonary function measure to follow longitudinally in the pediatric SCD population, as it showed significant differences between healthy controls and SCD participants.
PEDIATRIC PULMONOLOGY
(2021)
Article
Clinical Neurology
Kemar Prussien, Bruce E. Compas, Rachel E. Siciliano, Abagail E. Ciriegio, Chelsea A. Lee, Adetola A. Kassim, Michael R. DeBaun, Manus J. Donahue, Lori C. Jordan
Summary: Elevated cerebral blood flow and oxygen extraction fraction in individuals with sickle cell anemia are associated with deficits in executive function, with significant correlations remaining in multivariate analyses. This may be attributed to hemodynamic impairment.
Review
Urology & Nephrology
Kenneth Ataga, Santosh L. Saraf, Vimal K. Derebail
Summary: Sickle cell syndromes are associated with kidney abnormalities and nephropathy. The pathophysiology of SCD-related nephropathy is multifactorial, including oxidative stress, hyperfiltration, and glomerular hypertension. Patients with SCD experience faster decline in kidney function and often present with albuminuria. Multiple genetic modifiers are also implicated in the development and progression of SCD-related nephropathy. Chronic kidney disease and rapid decline in estimated glomerular filtration rate are associated with increased mortality in SCD patients.
NATURE REVIEWS NEPHROLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Silvia Lee, Patricia P. Wadowski, Timothy Hoberstorfer, Constantin Weikert, Joseph Pultar, Christoph W. Kopp, Simon Panzer, Thomas Gremmel
Summary: The study found that hyperuricemia is associated with increased residual platelet reactivity in patients treated with clopidogrel and prasugrel, but not in those treated with ticagrelor.
CARDIOVASCULAR DRUGS AND THERAPY
(2021)
Article
Oncology
Dania A. Monagel, Gregory Mt Guilcher, Alberto Nettel-Aguirre, Glenda Bendiak
Summary: This study suggests that hematopoietic cell transplantation in children with sickle cell disease may prevent the anticipated decline in pulmonary function over time.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Hematology
Suella Martino, Jean-Benoit Arlet, Marie-Helene Odievre, Vincent Jullien, Martina Moras, Claude Hattab, Thibaud Lefebvre, Laurent Gouya, Mariano A. Ostuni, Sophie D. Lefevre, Caroline Le Van Kim
Summary: This study revealed an abnormal retention of mitochondria in some SCD patients, along with specific biochemical changes. Meanwhile, alterations in levels of related proteins also play a role in the development of the disease.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Hematology
Berengere Koehl, Livia Claude, Karen Reminy, Vanessa Tarer, Veronique Baccini, Marc Romana, Yves Colin-Aronovicz, Vijaya L. Damaraju, Michael Sawyer, Thierry Peyrard, Maryse Etienne-Julan, Caroline Le Van Kim, Slim Azouzi, Luc Reininger
Summary: This study investigated the expression of erythrocyte ENT1 in adult patients with sickle cell disease (SCD) and carriers of sickle cell trait (SCT). The results showed that the expression levels of erythrocyte ENT1 were not significantly different from normal controls in patients with SCD in steady state conditions. However, the expression of erythrocyte ENT1 was significantly decreased in SCD patients during painful vaso-occlusive episodes and in healthy SCT carriers.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Pediatrics
Pritish Mondal, Vishal Midya, Arshjot Khokhar, Shyama Sathianathan, Erick Forno
Summary: This study identified pulmonary function test estimates associated with abnormal diffusing capacity in children with Sickle Cell Disease (C-SCD) using machine learning algorithms and linear mixed-effect models. The results showed that DLCO was associated with PFT estimates representing restrictive lung disease, airflow obstruction, and inflammation in C-SCD patients, highlighting the clinical relevance of prediction models.
FRONTIERS IN PEDIATRICS
(2021)
Review
Medicine, General & Internal
Mohammad S. Akhter, Hassan A. Hamali, Hina Rashid, Gasim Dobie, Aymen M. Madkhali, Abdullah A. Mobarki, Johannes Oldenburg, Arijit Biswas
Summary: Advanced mitochondrial multi-omics reveal the significant involvement of mitochondria in cell physiology, influencing cell structure, function, signaling, and fate. Mitochondrial dysfunction plays a crucial role in acquired and genetic diseases, including sickle cell disease. Mitochondrial anomalies, such as altered mitophagy, increased ROS, and mitochondrial DNA, contribute to the severity of sickle cell disease and its pathological cascade.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Hematology
Jonathan R. Day, Clifford Takemoto, Anjali Sharathkumar, Sarah Makhani, Ashwin Gupta, Stephanie Bitner, Cassandra D. Josephson, Evan M. Bloch, Aaron A. R. Tobian, Lakshmanan Krishnamurti, Ruchika Goel
Summary: Hospitalization patterns for persons with haemophilia (PWH) in the US show that adult admissions are often linked to age-related comorbidities, while pediatric admissions are primarily due to bleeding and catheter-related infections. The costs for haemophilia-related hospitalizations exceed the national average for all-cause hospitalizations.
Correction
Clinical Neurology
S. N. Rodgers-Melnick, L. Lin, K. Gam
JOURNAL OF PAIN RESEARCH
(2022)
Article
Health Care Sciences & Services
Lakshmanan Krishnamurti, Staci D. Arnold, Ann Haight, Allistair Abraham, Gregory M. T. Guilcher, Tami John, Nitya Bakshi, Shalini Shenoy, Karen Syrjala, Paul L. Martin, Sonali Chaudhury, Gretchen Eames, Olusola Festus Olowoselu, Matthew Hsieh, Josu De La Fuente, Kimberly A. Kasow, Elizabeth Stenger, Anne Mertens, Fuad El-Rassi, Peter Lane, Bronwen E. Shaw, Lillian Meacham, David Archer
Summary: This study established an international registry to investigate the long-term effects of hematopoietic cell transplantation (HCT) for sickle cell disease (SCD) and demonstrated the feasibility of recruitment at a single site in the United States. The study found that recruitment and implementation of study procedures were feasible.
JMIR RESEARCH PROTOCOLS
(2022)
Meeting Abstract
Hematology
Christopher Mwaniki Wanjiku, Charity Oyedeji, John J. Strouse
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.
Article
Oncology
Cynthia B. Sinha, Lillian R. Meacham, Nitya Bakshi, Diana Ross, Lakshmanan Krishnamurti
Summary: This study conducted qualitative interviews to understand how caregivers perceive the risk of infertility from allogeneic hematopoietic stem cell transplantation (HCT) for children with sickle cell disease (SCD). The study found that mothers were more concerned about the risk of death and graft-versus-host disease than the risk of infertility. Parents had a realistic understanding of the risk of infertility and considered it in their decision-making process. In addition, parents reported multiple barriers to fertility preservation.
PEDIATRIC BLOOD & CANCER
(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
Biochemical Research Methods
Benjamin E. J. Spurgeon, Andrew L. Frelinger III
Summary: This study developed a 16-color panel for analyzing platelets in human whole blood using spectral flow cytometry. The panel includes clinical relevant markers and an optimized protocol for high-parameter phenotyping, allowing the analysis of platelet phenotype and function.
Article
Hematology
Lakshmanan Krishnamurti, Donna Neuberg, Keith M. Sullivan, Shannon Smith, Mary Eapen, Mark C. Walters
Summary: This study aimed to compare the efficacy of bone marrow transplantation (BMT) with gene therapy in sickle cell disease (SCD), but it was prematurely closed due to recruitment difficulties. Barriers to enrollment included a shortage of suitable donors, exclusion of individuals with previously identified donors, preferences for conventional care among SCD patients, and the impact of the pandemic on recruitment, among others.
TRANSPLANTATION AND CELLULAR THERAPY
(2023)