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This article provides an overview of treatment approaches for chronic kidney disease (CKD) in patients with IgA nephropathy (IgAN). IgAN is the most common primary glomerulonephritis and results from an autoimmune reaction to aberrantly glycosylated immunoglobulin A (IgA) antibodies. Although historically considered largely benign, it is now recognized that a significant percentage of patients develop dialysis-dependent kidney disease over the years.
View Article and Find Full Text PDFRandomised, placebo-controlled, double-blinded, four-way crossover trial to demonstrate the comparative pharmacodynamic equivalence of a non-invasive diagnostic test for adrenal insufficiency in a healthy population: the STARLIT-2 study protocol.
BMJ Open
December 2024
Clinical Medicine, School of Medicine and Population Health, The University of Sheffield, Sheffield, UK
Introduction: Cortisol is an essential stress hormone and failure of its production, known as adrenal insufficiency (AI), is associated with significant mortality due to adrenal crisis. The Short Synacthen Test (SST) is the current diagnostic test of choice for AI, but it is both invasive and resource intensive. Globally, there is an unmet need for a non-invasive, cost-effective test.
View Article and Find Full Text PDFOptical coherence tomography angiography reveals abnormal retinal vascular density and perfusion in patients with X-linked adrenoleukodystrophy: a cross-sectional study.
Orphanet J Rare Dis
January 2025
Department of Ophthalmology, Daping Hospital, Chongqing, China.
Purpose: X-linked adrenoleukodystrophy (XALD) can affect the eyes. Existing therapies are hampered by early quantitative examination methods. This study used an optical coherence tomography angiography system (OCTA) to investigate retinal microvascular density and perfusion in XALD patients.
View Article and Find Full Text PDFInsights From Minnesota on Newborn Screening for Adrenoleukodystrophy: A 5-Year Update.
Am J Med Genet A
January 2025
M Health Fairview Masonic Children's Hospital, Minneapolis, Minnesota, USA.
Our objectives are to report on the outcomes of adrenal insufficiency (AI) and cerebral ALD (cALD) in children diagnosed with X-linked adrenoleukodystrophy (ALD) identified by newborn screening (NBS) in Minnesota in the first 5 years following initiation of NBS in 02/2017. A retrospective chart review was conducted for children diagnosed with ALD via Minnesota NBS from 02/06/2017 through 02/06/2022. Data reviewed included newborn screening data, diagnostic very long chain fatty acid levels, ABCD1 molecular testing results, serial measurements of ACTH and cortisol, and serial brain MRI results.
View Article and Find Full Text PDFCongenital Adrenal Hyperplasia and Adrenal Insufficiency in Children: An Evidence-based Review with Good Practice Points by Adrenal Working Group of The Turkish Society for Pediatric Endocrinology and Diabetes.
J Clin Res Pediatr Endocrinol
January 2025
Ankara University Faculty of Medicine, Department of Pediatric Endocrinology, Ankara, Turkey.
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