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Related Concept Videos

Renal Tubule and Collecting Duct01:24

Renal Tubule and Collecting Duct

The renal tubule is divided into three parts: the proximal convoluted tubule (PCT), the Loop of Henle (LOH), and the distal convoluted tubule (DCT).
Proximal Convoluted Tubule (PCT):
The PCT is the initial segment of the renal tubule, extending from the Bowman's capsule that encloses the glomerulus. Its convoluted structure and microvilli-lined cells increase the surface area for reabsorption. The PCT reabsorbs glucose, amino acids, sodium, and water from the filtrate, ensuring essential...
Nephrons01:10

Nephrons

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 happens...
Physiology of the Genitourinary System II: Tubular Reabsorption and Secretion01:22

Physiology of the Genitourinary System II: Tubular Reabsorption and Secretion

The kidneys maintain homeostasis through filtration, reabsorption, and secretion. Tubular reabsorption and secretion are crucial in forming urine and regulating electrolytes, water balance, and waste elimination.Tubular Reabsorption and Secretion ProcessesTubular reabsorption is the process that reclaims essential substances such as electrolytes, glucose, amino acids, and water from the glomerular filtrate back into the bloodstream. This is achieved through passive and active transport...
Acute Kidney Injury II: Pathophysiology01:29

Acute Kidney Injury II: Pathophysiology

Acute kidney injury (AKI) causes are categorized into three primary categories based on the location of the injury: prerenal, intrarenal (or intrinsic), and postrenal causes. This classification guides clinical management and illustrates how different pathways can impair kidney function.Etiology and Pathophysiology of Acute Kidney Injury1. Prerenal causesEtiology: Prerenal Acute Kidney Injury, the most common type, occurs when reduced blood flow to the kidneys decreases filtration capacity...
Renal Drug Excretion: Tubular Secretion01:28

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Active tubular secretion is a robust, energy-demanding process that utilizes carrier systems to transport drugs into renal tubules. The active renal secretion systems include the organic anion transporter (OAT) for weak acids and the organic cation transporter (OCT) for weak bases. Structurally similar drugs can compete for the same transporter, potentially leading to drug accumulation and toxicity. However, this principle can be exploited therapeutically. One example is probenecid (Probalan),...
Tubular Reabsorption and Secretion01:28

Tubular Reabsorption and Secretion

Tubular secretion and reabsorption are two critical processes in the nephron tubule of the kidneys. When the fluid filtered from the glomerulus enters the proximal convoluted tubule, it is referred to as filtrate, and its composition changes due to tubular reabsorption and secretion.
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Related Experiment Video

Updated: May 11, 2026

Generation of Human Kidney Tubuloids from Tissue and Urine
08:34

Generation of Human Kidney Tubuloids from Tissue and Urine

Published on: April 16, 2021

Renal tubular dysgenesis.

Marie-Claire Gubler1

  • 1INSERM, U983, 149 rue de Sèvres, 75743, Paris cedex 15, France, marie-claire.gubler@inserm.fr.

Pediatric Nephrology (Berlin, Germany)
|May 3, 2013
PubMed
Summary

Renal tubular dysgenesis (RTD) is a severe fetal disorder impacting kidney development. Early diagnosis of RTD in an anuric fetus is crucial for management and genetic counseling.

Area of Science:

  • Perinatology
  • Nephrology
  • Genetics

Background:

  • Renal tubular dysgenesis (RTD) is a severe fetal disorder characterized by absent or poorly developed proximal tubules, leading to anuria and Potter sequence.
  • Affected fetuses often experience early death due to pulmonary hypoplasia, anuria, and refractory arterial hypotension.
  • RTD can be inherited as an autosomal recessive disease or acquired during fetal development.

Purpose of the Study:

  • To summarize the key features, causes, and implications of renal tubular dysgenesis (RTD).
  • To highlight the critical role of the renin-angiotensin system (RAS) in fetal renal development and blood pressure regulation.
  • To emphasize the importance of diagnosing RTD in anuric fetuses for appropriate management and counseling.

Main Methods:

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  • Review of existing literature on renal tubular dysgenesis (RTD).
  • Analysis of genetic mutations associated with inherited RTD within the renin-angiotensin system (RAS).
  • Examination of secondary causes of RTD, including twin-to-twin transfusion syndrome and exposure to RAS blockers.
  • Main Results:

    • Mutations in RAS components (angiotensinogen, renin, ACE, AT1R) cause inherited RTD by disrupting angiotensin II production or function.
    • Secondary RTD results from conditions causing fetal renal hypoperfusion, such as twin-to-twin transfusion syndrome or exposure to RAS inhibitors.
    • A functional RAS is vital for maintaining fetal blood pressure and renal blood flow.

    Conclusions:

    • Accurate diagnosis of RTD in an anuric fetus with normal renal sonography is essential for neonatal management.
    • Understanding the cause of RTD allows for genetic counseling and strategies to prevent recurrence in future pregnancies.
    • RTD underscores the critical importance of the renin-angiotensin system for normal fetal development and survival.