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This study aimed to evaluate foveal avascular zone (FAZ) features and macular flow density (FD) in various retinal layers in a cohort of patients with foveal hypoplasia (FH) using optical coherence tomography angiography (OCTA), in order to characterize microvascular abnormalities and explore their potential clinical significance. FAZ parameters and FD, as well as retinal thickness and volume values were analyzed and compared between patients with FH and an age- and gender-matched control cohort. Correlations between disease severity and visual acuity (VA), as well as between disease severity and FAZ features were evaluated. A total of 19 eyes with FH and 19 control eyes were included. The study group showed significantly higher FD values in the foveal sectors of the superficial and deep capillary plexus compared to controls. FAZ area, perimeter, and acircularity index (ACI) were noticeably altered in eyes with FH; however, they did not correlate with disease severity. Visual acuity was negatively correlated with disease severity. The results of this study provide evidence of altered microvasculature architecture specifically in the foveal sectors of patients with FH. The higher FD values in the foveal sectors of FH patients suggest a potential compensatory response of the retinal microvasculature. FAZ parameters and FD values of the foveal sectors could be used as part of an OCTA-based grading system in FH patients.
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http://dx.doi.org/10.3390/jcm12154992 | DOI Listing |
Epilepsia Open
December 2024
Integrated Diagnostics for Epilepsy, Department of Diagnostic and Technology, European Reference Network EPIcare, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Neuronal ceroid lipofuscinoses (NCLs) are genetically heterogeneous neurodegenerative disorders, characterized by progressive cognitive and motor decline, epilepsy, visual impairment, and shortened life-expectancy. CLN6-related NCLs include both late-infantile and adult myoclonic form. We report a 21-year-old patient, with mild developmental delay, who developed occipital seizures at 14 years, and subsequently cognitive decline, cortical myoclonus, and photosensitivity at low and higher frequencies.
View Article and Find Full Text PDFRheumatology (Oxford)
December 2024
Department of Rheumatology, Erasmus MC, Rotterdam, The Netherlands.
Objectives: To evaluate the short and long term effects of an online, interactive, multifactorial lifestyle intervention program (Leef! Met Reuma) on health risk and all ICHOM-recommended patient reported outcome measures(PROMs) in patients with an Inflammatory Arthritis(IA), OsteoArthritis(OA) or FibroMyalgia(FM).
Methods: Patients with an IA, OA or FM, could register for the lifestyle intervention program. The program consists of a 3-month intensive part followed by a 21-month aftercare period and focuses on 4 pillars, namely nutrition, exercise, relaxation and sleep.
Blood Res
December 2024
Division of Hematology-Oncology, Hanyang University Seoul Hospital, Seoul, Republic of Korea.
Purpose: This study investigated the occurrence of subsequent malignancies (SM) in adult patients with severe aplastic anemia (SAA) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) to address the lack of large-scale, long-term data on this complication.
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Clin Rheumatol
December 2024
Department of Rheumatology, Faculty of Medicine, Dokuz Eylul University, İnciraltı Mahallesi Mithatpaşa Cad. no:1606, Balçova, İzmir, Türkiye.
Objectives: To evaluate the incidence and characteristics of severe infections in rheumatic patients receiving biologic disease-modifying anti-rheumatic drugs (bDMARDs) after kidney transplantation.
Methods: This multicenter, retrospective study included 38 patients who had undergone kidney transplantation and received bDMARDs for rheumatic diseases. Demographic, clinical, and treatment data were collected.
Mol Biol Rep
December 2024
State Key Laboratory of Cell Differentiation and Regulation, College of Life Sciences, Henan Normal University, Xinxiang, 453007, China.
Yes-associated protein (YAP) and transcriptional coactivator with PDZ-binding motif (TAZ) are key downstream effectors of the Hippo pathway that regulate organ size, tissue homeostasis, and cancer development. YAP/TAZ play crucial regulatory roles in organ growth, cell proliferation, cell renewal, and regeneration. Mechanistically, YAP/TAZ influence the occurrence and progression of liver regeneration (LR) through various signaling pathways, including Notch, Wnt/β-catenin, TGF-β/Smad.
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