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Related Concept Videos

Nephrons01:10

Nephrons

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The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
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Nephrotic Syndrome I : Introduction01:24

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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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Cystic Fibrosis: Pathogenesis01:23

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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
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Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

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IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
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Urinary Tract Calculi I: Introduction01:28

Urinary Tract Calculi I: Introduction

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Renal calculi, or kidney stones, are solid deposits of minerals and salts formed inside the kidneys. In medical terminology, "calculus" refers to the stone itself, while "lithiasis" describes the process of stone formation. Depending on their location within the urinary system, these stones may be classified as either urolithiasis, when situated within the urinary tract, or nephrolithiasis, when located within the kidneys. Each term signifies the specific impact of the stone.Predisposition...
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Renal Corpuscle01:20

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The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
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Updated: Mar 8, 2026

Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
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Nephropathic cystinosis: an update.

Koenraad R Veys1, Mohamed A Elmonem, Fanny O Arcolino

  • 1aDepartment of Pediatric Nephrology, University Hospitals Leuven bDepartment of Development and Regeneration, KU Leuven, Leuven, Belgium cDepartment of Clinical and Chemical Pathology, Faculty of Medicine, Cairo University, Cairo, Egypt dDepartment of Pediatric Nephrology, Radboud University Medical Center, Nijmegen, The Netherlands.

Current Opinion in Pediatrics
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Summary
This summary is machine-generated.

Cystinosis, a rare metabolic disease, is now treatable with new therapies. Future treatments may include stem cell therapy and gene editing for a potential cure.

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Area of Science:

  • Biochemistry
  • Genetics
  • Cellular Biology

Background:

  • Cystinosis is a rare lysosomal storage disorder.
  • It is a treatable metabolic disease with increasing understanding of its pathophysiology.
  • Cystinosis serves as a prototype for studying fundamental biochemical and cellular processes.

Purpose of the Study:

  • To provide an overview of the latest advances in cystinosis research.
  • To cover pathogenetic, clinical, and therapeutic aspects of cystinosis.

Main Methods:

  • Literature review of recent advances in cystinosis.
  • Analysis of pathogenetic, clinical, and therapeutic developments.

Main Results:

  • Cystinosis pathophysiology is increasingly understood.
  • New systemic and ocular cysteamine formulations are emerging.
  • Alternative therapeutic monitoring strategies are being developed.

Conclusions:

  • Improved outcomes for cystinosis patients are anticipated with new formulations and monitoring.
  • Stem cell-based therapy and gene-editing technologies offer novel tools for a potential cure.