scholarly journals Inherited podocytopathies

2008 ◽  
Vol 136 (Suppl. 4) ◽  
pp. 327-339
Author(s):  
Radovan Bogdanovic
Keyword(s):  
Congenital Nephrotic Syndrome ◽  
Structural Studies ◽  
Glomerular Filtration Barrier ◽  
Filtration Barrier ◽  
Glomerular Epithelial Cells ◽  
Causes Of Disease ◽  
Stage Renal Disease ◽  
End Stage ◽  
Made In

Podocytes, the visceral glomerular epithelial cells, are the postmythotic cells that line the outer aspects of the glomerular basement membrane. A number of advances have been made in recent years, linked to the discovery of singlegene defects in hereditary glomerular disease, which highlight the role of these cells in preventing proteinuria. Despite the rarity of hereditary proteinuric syndromes, genetic, biochemical, and structural studies of these diseases have made important contributions to our knowledge of how the normal glomerular filter works and the mechanism of proteinuria. The course of these diseases can vary; some patients present with severe proteinuria and congenital nephrotic syndrome, whereas others have only moderate proteinuria and focal segmental glomerulosclerosis. Regardless of its cause, the disease often progresses to end-stage renal disease. There can be overlap between the diseases: mutations in the same gene can lead to different renal phenotypes. It is important to know that some hereditary podocytopathies respond to therapy, whereas majority does not. For this reason, genetic testing, which is available for some hereditary podocytopathies should be performed whenever possible. This review summarizes recent progress in the eludication of genetic causes of disease and discusses their implication for the understanding of the pathogenic mechanisms which can lead to disruption of the glomerular filtration barrier.

scholarly journals Podocyte Autophagy: A Potential Therapeutic Target to Prevent the Progression of Diabetic Nephropathy

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Na Liu ◽  
Liuqing Xu ◽  
Yingfeng Shi ◽  
Shougang Zhuang
Keyword(s):  
Diabetic Nephropathy ◽  
Glomerular Filtration ◽  
Ultrastructural Changes ◽  
Cell Organelles ◽  
Glomerular Filtration Barrier ◽  
Filtration Barrier ◽  
Patients With Diabetes ◽  
Stage Renal Disease ◽  
End Stage ◽  
Protein Cell

Diabetic nephropathy (DN), a leading cause of end-stage renal disease (ESRD), becomes a worldwide problem. Ultrastructural changes of the glomerular filtration barrier, especially the pathological changes of podocytes, lead to proteinuria in patients with diabetes. Podocytes are major components of glomerular filtration barrier, lining outside of the glomerular basement membrane (GBM) to maintain the permeability of the GBM. Autophagy is a high conserved cellular process in lysosomes including impaired protein, cell organelles, and other contents in the cytoplasm. Recent studies suggest that activation of autophagy in podocytes may be a potential therapy to prevent the progression of DN. Here, we review the mechanisms of autophagy in podocytes and discuss the current studies about alleviating proteinuria via activating podocyte autophagy.

scholarly journals Relevance of VEGF and Nephrin Expression in Glomerular Diseases

2011 ◽  
Vol 2011 ◽  
pp. 1-9 ◽  
Author(s):  
Claudia A. Bertuccio
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Molecular Mechanisms ◽  
Plasma Albumin ◽  
Glomerular Filtration Barrier ◽  
Glomerular Diseases ◽  
Filtration Barrier ◽  
Stage Renal Disease ◽  
End Stage

The glomerular filtration barrier is affected in a large number of acquired and inherited diseases resulting in extensive leakage of plasma albumin and larger proteins, leading to nephrotic syndrome and end-stage renal disease. Unfortunately, the molecular mechanisms governing the development of the nephrotic syndrome remain poorly understood. Here, I give an overview of recent investigations that have focused on characterizing the interrelationships between the slit diaphragm components and podocytes-secreted VEGF, which have a significant role for maintaining the normal podocyte structure and the integrity of the filtering barrier.

The Potential Role of Catheter-Based Renal Sympathetic Denervation in Chronic and End-Stage Kidney Disease

2016 ◽  
Vol 21 (4) ◽  
pp. 344-352 ◽  
Author(s):  
Yusuke Sata ◽  
Markus P. Schlaich
Keyword(s):  
Blood Pressure ◽  
Renal Function ◽  
Kidney Disease ◽  
End Stage Renal Disease ◽  
Renal Denervation ◽  
Potential Role ◽  
Stage Renal Disease ◽  
End Stage ◽  
Renal Sympathetic

Sympathetic activation is a hallmark of chronic and end-stage renal disease and adversely affects cardiovascular prognosis. Hypertension is present in the vast majority of these patients and plays a key role in the progressive deterioration of renal function and the high rate of cardiovascular events in this patient cohort. Augmentation of renin release, tubular sodium reabsorption, and renal vascular resistance are direct consequences of efferent renal sympathetic nerve stimulation and the major components of neural regulation of renal function. Renal afferent nerve activity directly influences sympathetic outflow to the kidneys and other highly innervated organs involved in blood pressure control via hypothalamic integration. Renal denervation of the kidney has been shown to reduce blood pressure in many experimental models of hypertension. Targeting the renal nerves directly may therefore be specifically useful in patients with chronic and end-stage renal disease. In this review, we will discuss the potential role of catheter-based renal denervation in patients with impaired kidney function and also reflect on the potential impact on other cardiovascular conditions commonly associated with chronic kidney disease such as heart failure and arrhythmias.

scholarly journals Successful multiple-exchange peritoneal dialysis in a patient with severe hematological toxicity by methotrexate: case report and literature review

2019 ◽  
Vol 41 (3) ◽  
pp. 427-432 ◽  
Author(s):  
Arbey Aristizabal-Alzate ◽  
John Fredy Nieto-Rios ◽  
Catalina Ocampo-Kohn ◽  
Lina Maria Serna-Higuita ◽  
Diana Carolina Bello-Marquez ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
Therapeutic Option ◽  
Supportive Therapy ◽  
Hematological Toxicity ◽  
High Morbidity ◽  
Multiple Exchange ◽  
Severe Pancytopenia ◽  
Stage Renal Disease ◽  
End Stage

Abstract Methotrexate is an effective medication to control several diseases; however, it can be very toxic, being myelosuppression one of its main adverse effects, which increases in severity and frequency in patients with renal failure. We present the case of a 68-year-old man with chronic, end-stage renal disease associated with ANCA vasculitis, under treatment with peritoneal dialysis, who received the medication at a low dose, indicated by disease activity, which presented as a complication with severe pancytopenia with mucositis that improved with support measures and multiple-exchange peritoneal dialysis. We reviewed 20 cases published to date of pancytopenia associated with methotrexate in patients on dialysis and found high morbidity and mortality, which is why its use in this type of patient is not recommended. However, when this complication occurs, a therapeutic option could be the use of multiple-exchange peritoneal dialysis in addition to supportive therapy for drug-related toxicity, although it is recognized that studies are required to show the role of multiple-exchange peritoneal dialysis in the removal of this medication.

Recent Advances in the Management of the Infant, Child, and Adolescent With Chronic Renal Failure

1990 ◽  
Vol 11 (9) ◽  
pp. 277-282
Author(s):  
Richard N. Fine
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Quarter Century ◽  
Irreversible Reduction ◽  
Age Limits ◽  
Stage Renal Disease ◽  
End Stage

The prognosis of the infant, child, or adolescent with chronic renal failure, defined as an irreversible reduction in glomerular filtration rate, has improved during the past quarter century because of the use of dialysis and renal transplantation. These have prolonged lives in previously fatal situations. Because the potential not only to sustain life but also to effect full rehabilitation exists with the introduction of these treatments, it is now imperative that the multisystem consequences of uremia be either minimized or totally avoided in the pediatric patient with chronic renal failure. The role of the pediatrician in managing the infant, child, or adolescent with chronic renal failure should be directed toward minimizing the potentially devastating consequences of uremia so that the patient is in optimal clinical condition when end stage renal disease occurs. INCIDENCE It is difficult to know the incidence and prevalence of chronic renal failure and end stage renal disease in children. Surveys in Europe and North America have been conducted to obtain precise information regarding these issues; not only have the definitions included in these surveys differed, but the upper and lower age limits defining pediatric patients have not been uniform. The available data suggest a prevalence of chronic renal failure of 18.5 per 1 million child population and an incidence of end stage renal disease of from 3 to 6 children per 1 million total population.

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