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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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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...
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Pyelonephritis is a bacterial infection that primarily affects the renal parenchyma and collecting system, including the renal pelvis, tubules, and interstitial tissue of one or both kidneys. It can be classified as either acute—a sudden, severe infection—or chronic, which refers to long-term or recurrent kidney infections.The primary cause of acute pyelonephritis (APN) is bacterial infection, with Escherichia coli accounting for approximately 70-80% of cases. Other bacteria, such...
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Acute Kidney Injury (AKI) progresses through distinct clinical phases: the oliguric, diuretic, and recovery phases, each marked by unique manifestations and challenges.Oliguric Phase:The oliguric phase is the initial stage of AKI, typically lasting 10 to 14 days. This phase is marked by a significant reduction in urine output, usually less than 400 mL per day, indicating decreased kidney function. Fluid retention is a prominent feature, leading to symptoms such as edema, hypertension, and...
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Rapidly progressive glomerulonephritis in children.

Khemchand N Moorani1, Madiha Aziz2, Farhana Amanullah3

  • 1Khemchand N Moorani, MBBS, FCPS (Pediatrics and Nephrology). Professor of Pediatric Nephrology, The Kidney Center Postgraduate Training Institute (TKCPGTI), Karachi, Pakistan.

Pakistan Journal of Medical Sciences
|March 21, 2022
PubMed
Summary
This summary is machine-generated.

Rapidly progressive glomerulonephritis (RPGN) requires urgent diagnosis and treatment. Aggressive immunosuppression improves outcomes, but factors like fibrous crescents predict poor renal survival in children.

Keywords:
Acute glomerulonephritisAcute kidney injuryCrescentic GNImmunosuppressive therapyRPGN

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Area of Science:

  • Nephrology
  • Pediatric Nephrology
  • Immunology

Background:

  • Rapidly progressive glomerulonephritis (RPGN) is a critical kidney disease characterized by rapid loss of function.
  • Histologically, RPGN shows crescents in most glomeruli and presents a diagnostic and therapeutic emergency.
  • Management challenges are amplified in low-resource settings, necessitating clear clinical guidance.

Purpose of the Study:

  • To review the latest evidence on RPGN management in children.
  • To formulate clinical practice guidance for pediatric RPGN, especially in resource-limited areas.
  • To address the need for prompt diagnosis and urgent treatment to prevent poor outcomes.

Main Methods:

  • Systematic literature search of PubMed, Cochrane Library, and Google Scholar up to December 2020.
  • Inclusion of studies on "Rapidly progressive glomerulonephritis," "children," "crescentic glomerulonephritis," and "management."
  • Independent review by three investigators, including reference backtracking.

Main Results:

  • Aggressive immunosuppression has improved outcomes, but predictors of poor renal survival include nephrotic syndrome, severe acute kidney injury, fibrous crescents, and chronicity.
  • Post-infectious glomerulonephritis (PIGN) in children generally has a good prognosis without immunosuppression.
  • Immune-complex-mediated GN and lupus nephritis (LN) in children are associated with poor prognosis and high rates of end-stage kidney disease (ESKD).

Conclusions:

  • Prompt diagnosis and urgent treatment are crucial for improving outcomes in pediatric RPGN.
  • Current pediatric RPGN management guidelines are adapted from adult protocols.
  • Further research and tailored guidance are needed, particularly for low-resource settings, to optimize the management of pediatric RPGN.