Nephrotic syndrome in infants and children: pathophysiology and management

ML Downie, C Gallibois, RS Parekh… - … and international child …, 2017 - Taylor & Francis
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥ 40 mg/m2/hour or urine
protein/creatinine ratio≥ 200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (< …

Exploring the clinical and genetic spectrum of steroid resistant nephrotic syndrome: the PodoNet registry

A Trautmann, BS Lipska-Ziętkiewicz… - Frontiers in …, 2018 - frontiersin.org
Background: Steroid resistant nephrotic syndrome (SRNS) is a rare condition, accounting for
10–15% of all children with idiopathic nephrotic syndrome. SRNS can be caused by genetic …

Precision nephrology identified tumor necrosis factor activation variability in minimal change disease and focal segmental glomerulosclerosis

LH Mariani, S Eddy, FM AlAkwaa, PJ McCown… - Kidney international, 2023 - Elsevier
The diagnosis of nephrotic syndrome relies on clinical presentation and descriptive patterns
of injury on kidney biopsies, but not specific to underlying pathobiology. Consequently, there …

Interstitial fibrosis scored on whole-slide digital imaging of kidney biopsies is a predictor of outcome in proteinuric glomerulopathies

LH Mariani, S Martini, L Barisoni… - Nephrology Dialysis …, 2018 - academic.oup.com
Background Interstitial fibrosis (IF), tubular atrophy (TA) and interstitial inflammation (II) are
known determinants of progression of renal disease. Standardized quantification of these …

Angiotensin II up-regulates sodium-glucose co-transporter 2 expression and SGLT2 inhibitor attenuates Ang II-induced hypertensive renal injury in mice

KN Miyata, CS Lo, S Zhao, MC Liao, Y Pang… - Clinical …, 2021 - portlandpress.com
Clinical trials indicate that sodium/glucose co-transporter 2 (SGLT2) inhibitors (SGLT2i)
improve kidney function, yet, the molecular regulation of SGLT2 expression is incompletely …

CureGN study rationale, design, and methods: establishing a large prospective observational study of glomerular disease

LH Mariani, AS Bomback, PA Canetta… - American journal of …, 2019 - Elsevier
Rationale & Objectives Glomerular diseases, including minimal change disease, focal
segmental glomerulosclerosis, membranous nephropathy, and immunoglobulin A (IgA) …

Transethnic, genome-wide analysis reveals immune-related risk alleles and phenotypic correlates in pediatric steroid-sensitive nephrotic syndrome

H Debiec, C Dossier, E Letouzé… - Journal of the …, 2018 - journals.lww.com
Background Steroid-sensitive nephrotic syndrome (SSNS) is a childhood disease with
unclear pathophysiology and genetic architecture. We investigated the genomic basis of …

Comparing kidney health outcomes in children, adolescents, and adults with focal segmental glomerulosclerosis

DS Gipson, JP Troost, C Spino, S Attalla… - JAMA network …, 2022 - jamanetwork.com
Importance Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage
kidney disease (ESKD) across the lifespan. While 10% to 15% of children and 3% of adults …

Proteinuria reduction and kidney survival in focal segmental glomerulosclerosis

JP Troost, H Trachtman, C Spino, FJ Kaskel… - American Journal of …, 2021 - Elsevier
Rationale & Objective Remission of proteinuria has been shown to be associated with lower
rates of kidney disease progression among people with focal segmental glomerulosclerosis …

[HTML][HTML] The relatively poor correlation between random and 24-hour urine protein excretion in patients with biopsy-proven glomerular diseases

MC Hogan, HN Reich, PJ Nelson, SG Adler… - Kidney international, 2016 - Elsevier
Random urine protein creatinine ratios are used to estimate 24-hour urine protein excretion,
which is considered a diagnostic gold standard. However, few studies are available of the …