scholarly journals The role of cationic channels of the potential TRPC receptor in the pathogenesis of idiopathic nephrotic syndrome in children

2021 ◽  
Vol 66 (5) ◽  
pp. 67-74
Author(s):  
S. L. Morozov ◽  
V. V. Dlin
Keyword(s):  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
Idiopathic Nephrotic Syndrome ◽  
Pediatric Nephrology ◽  
Urgent Problem ◽  
Steady Decrease ◽  
Segmental Glomerulosclerosis ◽  
Cationic Channels ◽  
Morphological Picture

Idiopathic nephrotic syndrome is the most common glomerulopathy in children, with a prevalence of approximately 16 per 100,000 of child population worldwide. Any chronic glomerular disease has the same type of development mechanism. Regardless of the damaging factor, after the death of a significant part of the nephrons, there occurs a steady decrease in the glomerular filtration rate, while morphologically we most often determine focal segmental glomerulosclerosis. Studying the causes of focal segmental glomerulosclerosis is an urgent problem in pediatric nephrology. Recently, there has been discussed the role of the cation channels of the potential receptor TRPC of podocytes in the development of proteinuria and focal segmental glomerulosclerosis. The article provides data on the role of TRPC receptors in the pathogenesis of focal segmental glomerulosclerosis. The authors present their our own data demonstrating gene expression of the cationic channels family of the potential receptor TRPC1, TRPC3, TRPC4, TRPC5 and TRPC6 in children with idiopathic nephrotic syndrome, depending on the morphological picture of the disease and sensitivity to steroid therapy.

Idiopathic Nephrotic Syndrome Associated with Focal Segmental Glomerulosclerosis

1982 ◽  
pp. 23-30 ◽  
Author(s):  
K. Schärer ◽  
R. Waldherr ◽  
D. E. Müller-Wiefel ◽  
F. Manz ◽  
V. Lenhard ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
Idiopathic Nephrotic Syndrome ◽  
Segmental Glomerulosclerosis

scholarly journals Renal Biopsy: Use of Biomarkers as a Tool for the Diagnosis of Focal Segmental Glomerulosclerosis

2014 ◽  
Vol 2014 ◽  
pp. 1-11 ◽  
Author(s):  
Crislaine Aparecida da Silva ◽  
Mariana Molinar Mauad Cintra ◽  
Eliângela de Castro Côbo ◽  
Marcos Vinícius da Silva ◽  
Fabiano Bichuette Custódio ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Idiopathic Nephrotic Syndrome ◽  
Biological Molecules ◽  
Podocyte Injury ◽  
Segmental Glomerulosclerosis ◽  
Morphological Diagnosis ◽  
Potential Use

Focal segmental glomerulosclerosis (FSGS) is a glomerulopathy associated with nephrotic syndrome and podocyte injury. FSGS occurs both in children and adults and it is considered the main idiopathic nephrotic syndrome nowadays. It is extremely difficult to establish a morphological diagnosis, since some biopsies lack a considerable quantifiable number of sclerotic glomeruli, given their focal aspect and the fact that FSGS occurs in less than half of the glomeruli. Therefore, many biological molecules have been evaluated as potential markers that would enhance the diagnosis of FSGS. Some of these molecules and receptors are associated with the pathogenesis of FSGS and have potential use in diagnosis.

scholarly journals Potential role of Claudin-1 immunohistochemical expression and ultrastructural changes in detecting early focal segmental glomerulosclerosis

2019 ◽  
Vol 8 (3) ◽  
pp. 24-24
Author(s):  
Nadia Galal Elhefnawy ◽  
Nermine Mohamed Adb Raboh ◽  
Ola Hassan Nada ◽  
Esraa Adel Mahmoud ◽  
Waleed Anwar Abd El Mohsen
Keyword(s):  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
Ultrastructural Changes ◽  
Segmental Glomerulosclerosis ◽  
Disease Entities ◽  
Sclerotic Lesions ◽  
Stage Renal Disease ◽  
End Stage ◽  
Parietal Epithelial Cells

Background: Focal segmental glomerulosclerosis (FSGS) and Minimal change disease (MCD) are two disease entities presented mainly by nephrotic syndrome. While 95% of MCD cases showed complete remission on steroid therapy, 50% of FSGS cases progress to end stage renal disease. Early sclerotic lesions in FSGS can be missed in routine H&E examination. Objective: To differentiate early FSGS from MCD by detection of activated parietal epithelial cells (PECs) in early glomerular sclerotic lesions using Claudin-1 immunohistochemical (IHC) staining and by examining podocyte ultrastructural changes. Materials and Methods: This retrospective study included 28 cases diagnosed as MCD and 20 cases diagnosed as early FSGS. Clinicopathologic data collection, claudin-1 IHC staining and reviewing ultrastructural changes were performed and the results were statistically analyzed. Results: A statistically significant correlation was detected between claudin-1 expression and the initial diagnosis of the studied groups (P=0.005). Claudin-1 was expressed in a visceral location in (39.28%) of the biopsies initially diagnosed as MCD thus were reevaluated as early FSGS lesions. 63.64% of these positive cases were presented by steroid resistant nephrotic syndrome and 63.6% of which showed some ultrastructural changes of FSGS in podocytes including abnormalities in mitochondrial shapes, endoplasmic reticulum changes and a decreased number of autophagic vacuoles. Conclusion: Claudin-1 is a novel diagnostic marker that can differentiate between confusing cases of early FSGS versus MCD. Defective autophagy plays a role in the pathogenesis of FSGS.

Sign in / Sign up

Export Citation Format

Share Document