scholarly journals Primary nephrotic syndrome in children: Clinical significance of histopathologic variants of minimal change and of diffuse mesangial hypercellularity

1981 ◽  
Vol 20 (6) ◽  
pp. 765-771 ◽  
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Significance ◽  
Minimal Change ◽  
Primary Nephrotic Syndrome ◽  
Mesangial Hypercellularity

Immune deposits and mesangial hypercellularity in minimal change nephrotic syndrome: Clinical relevance

1982 ◽  
Vol 100 (2) ◽  
pp. 188-191 ◽  
Author(s):  
William R. Allen ◽  
Luther B. Travis ◽  
Tito Cavallo ◽  
Ben H. Brouhard ◽  
Robert J. Cunningham
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Relevance ◽  
Minimal Change Nephrotic Syndrome ◽  
Minimal Change ◽  
Mesangial Hypercellularity ◽  
Immune Deposits

scholarly journals Identification of a Novel ACTN4 Gene Mutation Which Is Resistant to Primary Nephrotic Syndrome Therapy

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
Lingzhang Meng ◽  
Shan Cao ◽  
Na Lin ◽  
Jingjie Zhao ◽  
Xulong Cai ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Target Gene ◽  
Minimal Change ◽  
Cleavage Sites ◽  
Primary Nephrotic Syndrome ◽  
Minimal Change Nephropathy ◽  
Filtration Barrier ◽  
Capture Method ◽  
Generation Sequencing ◽  
Target Gene Capture

ACTN4, a gene which codes for the protein α-actinin-4, is critical for the maintenance of the renal filtration barrier. It is well known that ACTN4 mutations can lead to kidney dysfunction, such as familial focal segmental glomerulosclerosis (FSGS), a common cause of primary nephrotic syndrome (PNS). To elucidate whether other mutations of ACTN4 exist in PNS patients, we sequenced the ACTN4 gene in biopsies collected from 155 young PNS patients (≤16 years old). The patients were classified into five groups: FSGS, minimal change nephropathy, IgA nephropathy, membranous nephropathy, and those without renal puncture. Ninety-eight healthy people served as controls. Samples were subjected to Illumina’s next generation sequencing protocols using FastTarget target gene capture method. We identified 5 ACTN4 mutations which occurred only in PNS patients: c.1516G > A (p.G506S) on exon 13 identified in two PNS patients, one with minimal change nephropathy and another without renal puncture; c.1442 + 10G > A at the splice site in a minimal change nephropathy patient; c.2191-4G > A at the cleavage site, identified from two FSGS patients; and c.1649A > G (p.D550G) on exon 14 together with c.2191-4G > A at the cleavage sites, identified from two FSGS patients. Among these, c.1649A > G (p.D550G) is a novel ACTN4 mutation. Patients bearing the last two mutations exhibited resistance to clinical therapies.

scholarly journals Primary Nephrotic Syndrome and Risks of End-Stage Kidney Disease, Cardiovascular Events, and Death: The Kaiser Permanente Nephrotic Syndrome Study

2021 ◽  
pp. ASN.2020111583
Author(s):  
Alan Go ◽  
Thida Tan ◽  
Glenn Chertow ◽  
Juan Ordonez ◽  
Dongjie Fan ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Cardiovascular Outcomes ◽  
Minimal Change ◽  
End Stage Kidney Disease ◽  
Kaiser Permanente ◽  
Primary Nephrotic Syndrome ◽  
End Stage

Background Few population-based data exist about adults with primary nephrotic syndrome. Methods To evaluate kidney, cardiovascular, and mortality outcomes in adults with primary nephrotic syndrome, we identified adults within an integrated health care delivery system (Kaiser Permanente Northern California) with nephrotic-range proteinuria or diagnosed nephrotic syndrome between 1996-2012. Nephrologists reviewed medical records for clinical presentation, laboratory findings, and biopsy results to confirm primary nephrotic syndrome and assigned etiology. We identified a 1:100 time-matched cohort of adults without diabetes, diagnosed nephrotic syndrome, or proteinuria as controls to compare rates of end-stage kidney disease (ESKD), cardiovascular outcomes, and death through 2014, using multivariable Cox regression. Results We confirmed 907 cases of primary nephrotic syndrome (655 definite and 252 presumed cases of focal segmental glomerulosclerosis [FSGS, 40%], membranous nephropathy [40%], and minimal change disease [20%]). Mean age was 49 years; 43% were women. Adults with primary nephrotic syndrome had higher adjusted rates of ESKD (adjusted hazard ratio [aHR], 19.63; 95% confidence interval [95% CI], 12.76 to 30.20), acute coronary syndrome (aHR 2.58; 95% CI, 1.89 to 3.52), heart failure (aHR 3.01; 95%CI, 2.16 to 4.19), ischemic stroke (aHR 1.80; 95%CI,1.06 to 3.05), venous thromboembolism (aHR 2.56; 95%CI, 1.35 to 4.85), and death (aHR 1.34; 95%CI, 1.09 to 1.64) versus controls. Excess ESKD risk was significantly higher for FSGS and membranous nephropathy than for presumed minimal change disease. The three etiologies of primary nephrotic syndrome did not differ significantly in terms of cardiovascular outcomes and death. Conclusions Adults with primary nephrotic syndrome experience higher adjusted rates of ESKD, cardiovascular outcomes, and death, with significant variation by underlying etiology in risk for developing ESKD.

The paucity of minimal change disease in adolescents with primary nephrotic syndrome

1998 ◽  
Vol 12 (2) ◽  
pp. 105-107 ◽  
Author(s):  
Noosha Baqi ◽  
Anup Singh ◽  
Shivaiha Balachandra ◽  
Hadi Ahmad ◽  
Anthony Nicastri ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Primary Nephrotic Syndrome
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