When treating patients with sickling disorders, hematologists and oncologists should know those eye lesions that require referral to an ophthalmologist, those that should lead to referral, and those that rarely are associated with decreased vision. Hyphema in any patient whose red cells contain hemoglobin S falls into the first category. Retinal neovascularization (proliferative retinopathy) and its consequence vitreous hemorrhage fall into the second. The comma sign, angioid streaks, and abnormal color vision fall into the third category. The roles of preoperative transfusion and experimental antisickling therapy in treatment and prevention of eye lesions are incompletely defined at present.
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http://dx.doi.org/10.1016/s0889-8588(05)70406-8 | DOI Listing |
Hematol Rep
January 2025
Laboratory of Immunobiology and Immunogenetics, Post Graduation Program in Genetics and Molecular Biology (PPGBM), Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre 91501-970, Brazil.
A quarter of a century ago, sickle cell disease (SCD) was mainly viewed as a typical genetic disease inherited as a classical Mendelian trait. Therefore, the main focus concerning SCD was on diagnosis, meaning, genotyping, and identification of homozygous and heterozygous individuals carrying the relevant HbS mutant allele. Nowadays, it is well established that sickle cell disease is indeed the result of homozygosis for the HbS variant, although this single feature is not capable of explaining the highly diverse clinical presentation of SCD.
View Article and Find Full Text PDFTransfusion
January 2025
Ottawa Hospital Research Institute, Ottawa Hospital Center for Transfusion, Ottawa, Ontario, Canada.
Background: Red blood cell (RBC) utilization in patients with sickle cell disease (SCD) in Canada is poorly defined. This study describes RBC utilization in an SCD cohort at a single Canadian center.
Study Design And Methods: All adults with SCD who received care at the Ottawa Hospital between January 2006 and May 2019 were included, and followed until December 2021.
Sickle cell disease (SCD) is the most common genetic disease in the world and a societal challenge. SCD is characterized by multi-organ injury related to intravascular hemolysis. To understand tissue-specific responses to intravascular hemolysis and exposure to heme, we present a transcriptomic atlas in the primary target organs of HbSS vs HbAA transgenic SCD mice.
View Article and Find Full Text PDFBackground And Objective: Sickle cell disease (SCD) is a vascular disease that may affect the retina. This study aimed to evaluate differences in average velocity (AV, mm/s), blood flow (BF, μL/min) and vessel diameter (VD, μm) from the temporal retinal arcades in SCD compared to healthy eyes using Doppler optical coherence tomography (DOCT).
Patients And Methods: A cross-sectional study was conducted between 2021 and 2023.
Front Public Health
January 2025
Center of Excellence for Sickle Cell Disease Research and Training (CESRTA), University of Abuja, Abuja, Nigeria.
Introduction: Newborn Screening (NBS) is a public health program designed to identify and provide early interventions for infants with genetic disorders such as Sickle Cell Disease (SCD). Lack of awareness and unwillingness to participate in the NBS by caregivers and some healthcare workers are major contributing factors impeding NBS for SCD.
Objective: To evaluate the level of awareness and acceptance of NBS for SCD and the determinant factors influencing caregivers in Gwagwalada Area Council of the Federal Capital Territory, Abuja, Nigeria.
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