Use of corticosteroids in Norwegian patients with immunoglobulin a nephropathy progressing to end-stage kidney disease: a retrospective cohort study.

Background: Despite several clinical trials, the use of corticosteroid therapy for treating immunoglobulin A nephropathy (IgAN) remains controversial. We aimed to describe the use of corticosteroid therapy combined with supportive therapy in Norwegian patients with IgAN who had progressed to end-stage kidney disease.

Methods: We conducted a retrospective cohort study using data from the Norwegian Renal Registry.

Antiglomerular Basement Membrane Disease Possibly Triggered by Undiagnosed Renal Cell Carcinoma: A Case Report.

Antiglomerular basement membrane (anti-GBM) disease is a rare, small-vessel vasculitis that affects the capillary beds of the kidneys and lungs. Although exceedingly rare, several case reports have described anti-GBM disease with a concurrent cancer diagnosis, suggesting a possible correlation between these 2 conditions. Herein, we describe the first known case to our knowledge of a woman in her early 60s with simultaneous anti-GBM disease and clear cell renal cell carcinoma, in which the tumor was thought to have been the substrate for anti-GBM disease.

Proteomic analysis unveils Gb3-independent alterations and mitochondrial dysfunction in a gla zebrafish model of Fabry disease.

Background: Fabry disease (FD) is a rare lysosomal storage disorder caused by mutations in the GLA gene, resulting in reduced or lack of α-galactosidase A activity. This results in the accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in lysosomes causing cellular impairment and organ failures. While current therapies focus on reversing Gb3 accumulation, they do not address the altered cellular signaling in FD.

Gene Expression Analysis in -Mutant Zebrafish Reveals Enhanced Ca Signaling Similar to Fabry Disease.

Fabry disease (FD) is an X-linked inborn metabolic disorder due to partial or complete lysosomal α-galactosidase A deficiency. FD is characterized by progressive renal insufficiency and cardio- and cerebrovascular involvement. Restricted access on Gb3-independent tissue injury experimental models has limited the understanding of FD pathophysiology and delayed the development of new therapies.

A multiomics disease progression signature of low-risk ccRCC.

Clear cell renal cell carcinoma (ccRCC) is the most common renal cancer. Identification of ccRCC likely to progress, despite an apparent low risk at the time of surgery, represents a key clinical issue. From a cohort of adult ccRCC patients (n = 443), we selected low-risk tumors progressing within a 5-years average follow-up (progressors: P, n = 8) and non-progressing (NP) tumors (n = 16).