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Ménière's Disease (MD) is difficult to diagnose and evaluate objectively over the course of treatment. Recently, several studies have reported MD diagnoses by MRI-based endolymphatic hydrops (EH) analysis. However, this method is time-consuming and complicated. Therefore, a fast, objective, and accurate evaluation tool is necessary. The purpose of this study was to develop an algorithm that can accurately analyze EH on intravenous (IV) gadolinium (Gd)-enhanced inner-ear MRI using artificial intelligence (AI) with deep learning. In this study, we developed a convolutional neural network (CNN)-based deep-learning model named INHEARIT (INner ear Hydrops Estimation via ARtificial InTelligence) for the automatic segmentation of the cochlea and vestibule, and calculation of the EH ratio in the segmented region. Measurement of the EH ratio was performed manually by a neuro-otologist and neuro-radiologist and by estimation with the INHEARIT model and were highly consistent (intraclass correlation coefficient = 0.971). This is the first study to demonstrate that automated EH ratio measurements are possible, which is important in the current clinical context where the usefulness of IV-Gd inner-ear MRI for MD diagnosis is increasing.
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http://dx.doi.org/10.1038/s41598-020-63887-8 | DOI Listing |
J Am Soc Nephrol
September 2025
Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN, USA.
Background: Genetic modifiers are believed to play an important role in the onset and severity of polycystic kidney disease (PKD), but identifying these modifiers has been challenging due to the lack of effective methodologies.
Methods: We generated zebrafish mutants of IFT140, a skeletal ciliopathy gene and newly identified autosomal dominant PKD (ADPKD) gene, to examine skeletal development and kidney cyst formation in larval and juvenile mutants. Additionally, we utilized ift140 crispants, generated through efficient microhomology-mediated end joining (MMEJ)-based genome editing, to compare phenotypes with mutants and conduct a pilot genetic modifier screen.
JCI Insight
September 2025
Edinburgh Medical School: Biomedical Sciences & Euan MacDonald Centre for M, University of Edinburgh, Edinburgh, United Kingdom.
Spinal muscular atrophy (SMA) is a neuromuscular disease caused by low levels of SMN protein. Several therapeutic approaches boosting SMN are approved for human patients, delivering remarkable improvements in lifespan and symptoms. However, emerging phenotypes, including neurodevelopmental comorbidities, are being reported in some treated SMA patients, indicative of alterations in brain development.
View Article and Find Full Text PDFClin J Am Soc Nephrol
September 2025
University College London Great Ormond Street Hospital for Children and Institute of Child Health, London, UK.
Background: Experience with icodextrin use in children on long-term peritoneal dialysis is limited. We describe international icodextrin prescription practices and their impact on clinical outcomes: ultrafiltration, blood pressure control, residual kidney function (RKF), technique and patient survival.
Methods: We included patients under 21 years enrolled in the International Pediatric Peritoneal Dialysis Network (IPPN) between 2007 and 2024, on automated PD with a daytime dwell.
Clin J Am Soc Nephrol
September 2025
Division of Nephrology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland USA.
Socioeconomic, environmental and lifestyle factors shape kidney health. Among the social determinants of health, access to healthy foods is particularly significant. As a basic need, food is integral to an individual's identity, culture, and health.
View Article and Find Full Text PDFClin J Am Soc Nephrol
September 2025
Kidney Division, Peking University First Hospital, Peking University Institute of Nephrology; Key Laboratory of Kidney Disease, Ministry of Health of China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, China.
Background: The Therapeutic Effects of Steroids in IgA Nephropathy Global (TESTING) trial demonstrated that glucocorticoid therapy reduced proteinuria and improved kidney outcomes in patients with Immunoglobulin A Nephropathy (IgAN). Galactose-deficient IgA1 (Gd-IgA1) plays a central role in IgAN pathogenesis by promoting immune complex formation. However, the effects of glucocorticoid on pathogenic IgA levels remain unclear.
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