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Primary hyperoxaluria type 3: from infancy to adulthood in a genetically unique cohort.
Pediatr Nephrol
October 2024
Azrieli Faculty of Medicine, Bar Ilan University, Safed, Israel.
Background: Primary hyperoxaluria type 3 (PH3) is a rare autosomal recessive disorder caused by bi-allelic genetic variants in the 4 hydroxy-2 oxoglutarate aldolase (HOGA-1) gene. We report the natural history of PH3 in a 16-patient cohort, 15 from a unique genetically isolated population.
Methods: This retrospective single-center study followed PH3 patients between 2003 and 2023 with demographic, clinical, radiographic, genetic, and biochemical parameters.
Background: Focal segmental glomerulosclerosis (FSGS) is a primary podocytopathy characterized by primary podocyte detection and high proteinuria. The search for biomarkers and factors associated with the progression of this disease is an important task nowdays.
Aim: To assess the proteomic profile of urine in patients with FSGS and to isolate urinary biomarkers of podocytopathies.
Case report: Isolated tubular basement membrane deposits in a patient with systemic lupus erythematosus - A diagnostic challenge.
Front Med (Lausanne)
September 2022
University Hospital, Federal University of Maranhão (HU-UFMA), São Luís, Brazil.
Lupus nephritis is one of the most serious and frequent manifestations of systemic lupus erythematosus. It usually presents in the first years of the disease, which suspicion should be raised in cases of elevated serum creatinine, presence of proteinuria above 500 mg/day or active urinary sediment, in the absence of other apparent causes such as urinary tract infection and use of nephrotoxic drugs. In most cases, it affects the glomerulus, and its presentation is rare in the form of isolated tubulo-interstitial disease.
View Article and Find Full Text PDFPMMA-Based Continuous Hemofiltration Modulated Complement Activation and Renal Dysfunction in LPS-Induced Acute Kidney Injury.
Front Immunol
June 2021
Nephrology, Dialysis and Transplantation Unit, Department of Medical and Surgical Science, University of Foggia, Foggia, Italy.
Sepsis-induced acute kidney injury (AKI) is a frequent complication in critically ill patients, refractory to conventional treatments. Aberrant activation of innate immune system may affect organ damage with poor prognosis for septic patients. Here, we investigated the efficacy of polymethyl methacrylate membrane (PMMA)-based continuous hemofiltration (CVVH) in modulating systemic and tissue immune activation in a swine model of LPS-induced AKI.
View Article and Find Full Text PDFUnlabelled: Recent studies have shown a high risk of chronic kidney disease and associated cardiovascular complications in patients with rheumatoid arthritis (RA), which determines the prognosis. However, the prevalence of chronic kidney disease (CKD) in RA has not been established in the Russians.
Aim: Study was to examine the prevalence, risk factors and histological variants of CKD in RA.
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