Article
Pediatrics
Marianne E. Yee, Kristina W. Lai, Nitya Bakshi, Joanna K. Grossman, Preeti Jaggi, Alexander Mallis, Yun F. Wang, Robert C. Jerris, Peter A. Lane, Inci Yildirim
Summary: This retrospective cohort study examined children with SCD who had blood cultures collected from 2010 to 2019, and found that BSI remains a risk for these children with an incidence rate of 0.89 per 100 person-years. Sickle cell anemia genotypes and chronic transfusions were associated with higher odds of BSI, while hydroxyurea was associated with lower odds of BSI.
Review
Hematology
David C. Rees, Valentine A. M. Brousse, John N. Brewin
Summary: Sickle cell disease is a highly variable condition, with outcomes ranging from childhood death to relatively symptom-free living. The co-inheritance of alpha thalassemia and factors determining HbF levels significantly influence the phenotype, but few other significant genetic variants have been identified. Environmental factors, including socioeconomic status and access to medical care, play a significant role in determining outcomes. Exposure to cold and windy weather seems to trigger acute complications in many individuals, although the effects are unpredictable and vary with geography. Several studies have attempted to identify prognostic factors for predicting outcomes, particularly in infancy. Low hemoglobin, low hemoglobin F percentage, and high reticulocytes in childhood are associated with worse outcomes, but these effects are relatively weak and inconsistent.
Article
Medicine, General & Internal
Stephen Rineer, Patrick S. Walsh, Luke R. Smart, Nusrat Harun, David Schnadower, Matthew J. Lipshaw
Summary: This study investigated the absolute risk, risk factors, and outcomes of bacteremia in children and young adults with sickle cell disease presenting with fever. The study found that the risk of bacteremia during fever in these individuals is low, while a history of invasive bacterial infection, central line-associated bloodstream infection, or central venous catheter use may be associated with bacteremia. Age and sickle cell disease genotype are not associated with bacteremia.
Article
Hematology
Filip Ionescu, Nwabundo Anusim, Markie Zimmer, Ishmael Jaiyesimi
Summary: Sickle trait (Hb SA) or sickle disease (Hb SS) patients have an increased risk of venous thromboembolism (VTE), but VTE prophylaxis (VTEP) is underused in these patients. The use of VTEP varies among Hb SS patients based on treatment site, while comorbid conditions play a role in VTEP use in Hb SA patients.
EUROPEAN JOURNAL OF HAEMATOLOGY
(2022)
Article
Hematology
Antonella Meloni, Laura Pistoia, Alessandra Quota, Giuseppe Messina, Paolo Ricchi, Sergio Bagnato, Calogera Gerardi, Roberto Lisi, Liana Cuccia, Stefania Renne, Antonino Vallone, Riccardo Righi, Vincenzo Positano, Alessia Pepe, Filippo Cademartiri
Summary: The aim of this multicenter study was to assess the predictive value of multiparametric cardiac magnetic resonance (CMR) for cardiovascular complications in sickle cell disease (SCD) patients. Reduced left ventricular ejection fraction and increased right ventricular mass index showed a significant prognostic value in patients with SCD.
ANNALS OF HEMATOLOGY
(2023)
Review
Hematology
Giovanna Cannas, Solene Poutrel, Mael Heiblig, Helene Labussiere, Marie-Virginie Larcher, Xavier Thomas, Arnaud Hot
Summary: Population-based studies and case reports indicate an increased risk of acute leukemia in sickle cell disease (SCD) patients. A review of the literature found 51 previously reported cases, with myelodysplastic features and genetic abnormalities identified. The increased leukemogenesis risk in SCD is multifactorial, attributed to chronic inflammation caused by chronic hemolysis and secondary hemochromatosis, leading to persistent marrow stress and genetic damage in hematopoietic stem cells, ultimately resulting in acute myeloid leukemia.
ANNALS OF HEMATOLOGY
(2023)
Review
Biology
A. G. Geffersa, J. J. Burdon, S. Macfadyen, P. H. Thrall, S. J. Sprague, L. G. Barrett
Summary: Genetic resistance is crucial for managing infectious diseases in crops, but the rapid evolution of pathogens is causing resistance breakdown and posing a threat to disease control. Recent research has discovered strategies for deploying resistance genes to disrupt pathogen adaptation and prevent breakdown, but their practical implementation is currently limited.
PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES
(2023)
Article
Economics
David Holdford, Nicholas Vendetti, Daniel M. Sop, Shirley Johnson, Wally R. Smith
Summary: This study aimed to quantify the indirect costs of sickle cell disease in the United States and found that pain events had a significant negative impact on employment status, productivity, and daily lives of adults with the disorder.
Review
Virology
Tianyi Liang, Kaixin Guo, Peng Ni, Guangcai Duan, Rongguang Zhang
Summary: The aim of this meta-analysis was to investigate the relationship between sickle cell disorder and severe COVID-19. The analysis of 21 studies found that sickle cell disease (SCD) was a risk factor for mortality, hospitalization, and intensive care unit (ICU) admission in COVID-19 patients. Sickle cell trait (SCT) was also significantly associated with mortality and hospitalization in COVID-19 patients. These findings highlight the important impact of sickle cell disorder on the progression of COVID-19 to severe cases and associated deaths.
JOURNAL OF MEDICAL VIROLOGY
(2023)
Article
Oncology
Christina A. Rostad, Alexander N. Maillis, Kristina Lai, Nitya Bakshi, Robert C. Jerris, Peter A. Lane, Marianne E. Yee, Inci Yildirim
Summary: This study found that RSV infections are common in children with sickle cell disease, with a higher burden in younger patients. RSV is associated with considerable morbidity, including higher rates of hospitalization compared to the general population.
PEDIATRIC BLOOD & CANCER
(2021)
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
Medicine, General & Internal
Xianbin Xu, Xia Yu, Kai Gong, Huilan Tu, Junjie Yao, Yan Lan, Shaoheng Ye, Haoda Weng, Yu Shi, Jifang Sheng
Summary: This study found an association between acute decompensation events and the risk of nosocomial infections. Jaundice was identified as an independent risk factor for the occurrence of nosocomial infections and increased 90-day mortality rate. Antibiotic prophylaxis may be beneficial for this specific subset of patients.
FRONTIERS IN MEDICINE
(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)
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)