Background: The aim of this study is to examine the thickness of choroidal, macular and peripapillary retinal nerve fiber layer by spectral-domain optical coherence tomography (SD-OCT) in pediatric patients with sickle cell anemia (SCA) without retinopathy.
Methods: A total of 75 children (30 SCA patients (Group 1) and 45 healthy individuals (Group 2) were included in the study. Macular (central, superior, inferior, nasal, temporal), choroidal (subfoveal, at nasal distances from the central fovea of 1000 μm [N1], 2000 μm [N2], 3000 μm [N3], at temporal distances from the central fovea of 1000 μm [T1], 2000 μm [T2], 3000 μm [T3]) and RNFL (average, temporal, superotemporal, inferotemporal, nasal, inferonasal and superonasal) measurements were performed by SD-OCT. These parameters were compared with healthy children with similar demographic characteristics.
Results: The mean age was 14.11±3.86 (11-18) in sickle cell anemia patients and 13.15± 2.69 (10-18) in the healthy control group. Of the patients, 56.6% (n=17) of Group 1 and 44.4% (n=20) of Group 2 were male. Choroidal measurements made in the subfoveal, N1, N2, N3, T1, T2 and T3 quadrants showed that the choroid was thinner in 6 quadrants in SCA patients compared to the healthy group (p = 0.003, p = 0.039, p = 0.035, p = 0.595, p = 0.006, p = 0.005, p = 0.047, respectively). In RNFL measurements, there was significant thinning in the temporal, inferotemporal, and nasal quadrants of SCA patients compared to the healthy group. Changes in other quadrants were not significant.
Conclusions: SD-OCT is a useful imaging method in the diagnosis and screening in patients with SCA without retinopathy. Early diagnosis of retinopathy during subclinical disease will prevent visual loss in these patients.
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http://dx.doi.org/10.24953/turkjped.2021.05.015 | DOI Listing |
Mediterr J Hematol Infect Dis
January 2025
Center for Human Genetics, Faculty of Medicine, KU Leuven, Leuven, Belgium.
Mediterr J Hematol Infect Dis
January 2025
Pediatric Intensive Care Unit, Hospital Professor Doutor Fernando Fonseca, Unidade Local de Saúde Amadora/Sintra, Portugal.
Background: Red Blood Cell Exchange (RBCX) is a common treatment for pediatric sickle cell disease (SCD). Since inflammation with elevated proinflammatory cytokines plays a crucial role in SCD, this study hypothesized that RBCX might lower these cytokines and aimed to assess the impact of this technique on these markers.
Methods: Prospective and observational study of pediatric SCD patients (HbSS genotype) enrolled in a chronic RBCX program at a Portuguese Hospital from October 2022 to August 2024.
Ann Hematol
January 2025
Department of Internal Medicine, Section of Hematology/Oncology, University of Missouri-Kansas City, Kansas City, MO, 64108, USA.
Sickle cell disease (SCD) is an inherited hematologic disease caused by sickle hemoglobin as the predominant RBC hemoglobin or by sickle hemoglobin in combination with other abnormal β-hemoglobin variants like HbC, HbD and others. Sickling of erythrocytes under deoxygenated conditions is the basis of inflammatory and thrombotic cascades which result in multiple serious complications, leading to early morbidity and mortality. While HLA-matched allogeneic bone marrow transplantation is potentially curative, it has considerable limitations due to potential severe toxicities.
View Article and Find Full Text PDFBlood Cells Mol Dis
January 2025
Red Blood Cell Research Group, Central Diagnostic Laboratory-Research, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.
Pyruvate kinase (PK), a key ATP-generating enzyme in glycolysis, is a target for novel sickle cell disease (SCD) therapies. Enhancing PK activity lowers 2,3-diphosphyglycerate (2,3-DPG), increases adenosine triphosphate (ATP), and may prevent red blood cell (RBC) sickling. Townes and Berkeley SCD mouse models are commonly used for the development of novel drugs for SCD, but differ from humans in 2,3-DPG and ATP levels, which could be related to underlying differences in PK properties.
View Article and Find Full Text PDFJ Nanobiotechnology
January 2025
College of Pharmacy, Chosun University, 309 Pilmun-Daero, Dong-Gu, Gwangju, 61452, Republic of Korea.
Artificial oxygen carriers have emerged as potential substitutes for red blood cells in situations of major blood loss, including accidents, surgical procedures, trauma, childbirth, stomach ulcers, hemorrhagic shock, and blood vessel ruptures which can lead to sudden reduction in blood volume. The therapeutic delivery of oxygen utilizing artificial oxygen carriers as red blood cell substitutes presents a promising avenue for treating a spectrum of disease models. Apart from that, the recent advancement of artificial oxygen carriers intended to supplant conventional blood transfusions draws significant attention due to the exigencies of warfare and the ongoing challenges posed by the COVID-19 pandemic.
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