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Related Concept Videos

Chronic Kidney Disease I: Introduction01:25

Chronic Kidney Disease I: Introduction

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Chronic Kidney Disease (CKD) arises when the kidneys progressively lose their ability to function, ultimately leading to end-stage renal disease. At this advanced stage, the kidneys can no longer filter waste or maintain essential body functions, requiring renal replacement therapy (RRT) through dialysis or a kidney transplant for survival.Early-stage chronic kidney disease and detection challengesIn CKD's early stages, symptoms often remain absent because healthy nephrons compensate for...
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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
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
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Chronic Kidney Disease III: Interprofessional Care01:28

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Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...
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Chronic Kidney Disease II: Clinical Manifestations01:24

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Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
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Kidney Structure01:45

Kidney Structure

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The kidneys are two large bean-shaped organs located in the upper abdomen. They filter the blood several times a day to remove toxins and rebalance water and electrolytes of the circulatory system via the renal veins. The kidneys receive blood directly from the heart via the renal arteries. These arteries enter the kidney at the hilum, the concave surface of the bean, where they branch and divide into smaller vessels and capillaries.
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Renal Drug Clearance: Overview01:06

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Renal clearance is a crucial parameter in pharmacokinetics that quantifies the rate at which the kidneys excrete a drug. It represents a constant fraction of the central volume of distribution containing the drug that the kidney eliminates per unit of time.
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Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
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Autosomal Dominant Polycystic Kidney Disease: Core Curriculum 2025.

Craig E Gordon1, Pranav S Garimella2, Ronald D Perrone1

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American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
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PubMed
Summary

Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic cause of kidney failure. Advances in diagnosis, prognosis, and management, including new therapies, offer improved outcomes for patients with ADPKD.

Keywords:
Autosomal dominant polycystic kidney disease

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Area of Science:

  • Nephrology
  • Genetics
  • Internal Medicine

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is the leading genetic cause of kidney failure.
  • Recent scientific progress has enhanced the diagnosis, prognosis, and management strategies for ADPKD's renal and extrarenal complications.
  • Risk stratification for ADPKD progression is now possible by integrating total kidney volume, kidney function, and genetic mutation data.

Purpose of the Study:

  • To provide a comprehensive overview of the diagnosis and management of ADPKD.
  • To discuss the renal and extrarenal manifestations of ADPKD, including pain, cyst infection, polycystic liver disease, and intracranial aneurysms.
  • To cover the management of hypertension, women's health, and pregnancy in ADPKD patients.

Main Methods:

  • Review of current scientific literature and clinical practice guidelines.
  • Integration of data on diagnostic markers, prognostic indicators, and therapeutic interventions.
  • Alignment with the latest Kidney Disease: Improving Global Outcomes (KDIGO) guidelines for ADPKD.

Main Results:

  • Total kidney volume, kidney function, and genetic mutation status predict ADPKD progression to kidney failure.
  • Tolvaptan, a V2 receptor antagonist, is currently the only FDA-approved therapy for slowing ADPKD progression.
  • Multiple other therapies are under active investigation for ADPKD treatment.

Conclusions:

  • Accurate risk assessment allows for timely initiation of disease-modifying therapies.
  • Comprehensive management strategies address both renal and extrarenal manifestations of ADPKD.
  • Current guidelines and emerging therapies offer improved prospects for patients with ADPKD.