The pathogenesis of acute painful crisis in children with sickle cell disease is poorly understood; suggested risk factors include sickle cell type, severity of anemia, fetal hemoglobin concentration, and hypoxemia from upper airway obstruction. In a cohort study of 95 patients the relationship between clinical, laboratory, and sleep study data and frequency of painful crisis was investigated. Both univariate and multiple regression modeling showed that low nocturnal oxygen saturation was highly significantly associated with a higher rate of painful crisis in childhood (P <.0001). Screening and treatment for hypoxemia may reduce the frequency of this and other complications of the disease.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1182/blood-2002-05-1392 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Alterations of T Cell Subsets Associated with Sickle Cell Trait.
Blood Genom Discov
October 2024
Department of Pathology and Laboratory Medicine, University of Vermont Larner College of Medicine, Burlington, VT 05405, USA.
Sickle cell trait (SCT) has been associated with alterations in various immune-related laboratory parameters including lower circulating lymphocyte counts. To further characterize the impact of SCT on the immune system, we performed flow cytometry of monocyte and lymphocyte immune cell subsets from peripheral blood mononuclear cells collected in a large, community-based cohort of SCT-positive (n = 68) and SCT-negative (n = 959) Black adults. SCT was significantly associated with lower proportions of CD8 and CD4 T cell subsets that include senescent-like markers of repeated immune system challenges.
View Article and Find Full Text PDFMalaria prophylaxis in sickle cell anaemia: some answers, more questions.
Lancet Infect Dis
December 2024
Cincinnati Children's Hospital, Cincinnati, OH, USA.
Weekly dihydroartemisinin-piperaquine versus monthly sulfadoxine-pyrimethamine for malaria chemoprevention in children with sickle cell anaemia in Uganda and Malawi (CHEMCHA): a randomised, double-blind, placebo-controlled trial.
Lancet Infect Dis
December 2024
Training and Research Unit of Excellence, Blantyre, Malawi; School of Global and Public Health, Kamuzu University of Health Sciences, Blantyre, Malawi.
Background: In many sub-Saharan African countries, it is recommended that children with sickle cell anaemia receive malaria chemoprevention with monthly sulfadoxine-pyrimethamine or daily proguanil as the standard of care. However, the efficacy of these interventions is compromised by high-grade antifolate resistance of Plasmodium falciparum and poor adherence. We aimed to compare the efficacy of weekly dihydroartemisinin-piperaquine and monthly sulfadoxine-pyrimethamine for the prevention of clinical malaria in children with sickle cell anaemia in areas with high-grade sulfadoxine-pyrimethamine resistance of P falciparum in Uganda and Malawi.
View Article and Find Full Text PDFHydroxyurea therapy for neurological and cognitive protection in pediatric sickle cell anemia in Uganda (BRAIN SAFE II): Protocol for a single-arm open label trial.
Contemp Clin Trials Commun
December 2024
Department of Pediatrics, Columbia University Irving Medical Center, New York, USA.
Background: Children with sickle cell anemia (SCA) in Sub-Saharan Africa are at high risk of sickle cerebrovascular injury (SCVI). Hydroxyurea, a commonly used disease-modifying therapy, may reduce SCVI resulting in potential impact on reducing stroke and cognitive dysfunction. We aim to test the impact of daily hydroxyurea therapy on these outcomes in Ugandan children with SCA.
View Article and Find Full Text PDFDescriptive epidemiology demonstrating the All of Us database as a versatile resource for the rare and undiagnosed disease community.
J Am Med Inform Assoc
December 2024
Department of Biological and Health Sciences, Crown College, St Bonifacius, MN 55375, United States.
Objective: We aim to demonstrate the versatility of the All of Us database as an important source of rare and undiagnosed disease (RUD) data, because of its large size and range of data types.
Materials And Methods: We searched the public data browser, electronic health record (EHR), and several surveys to investigate the prevalence, mental health, healthcare access, and other data of select RUDs.
Results: Several RUDs have participants in All of Us [eg, 75 of 100 rare infectious diseases (RIDs)].
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!