Nephritic Syndrome Overview

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Questions and Answers

What is a key characteristic of nephritic syndrome?

  • Hypoalbuminemia with ascites
  • Presence of RBC casts in urine (correct)
  • Elevated creatinine without oliguria
  • Massive proteinuria (> 3.5 g/day)

Which condition is commonly associated with crescent formation in Bowman space?

  • Poststreptococcal glomerulonephritis (PSGN)
  • Rapidly Progressive Glomerulonephritis (RPGN) (correct)
  • Minimal Change Disease
  • Chronic Kidney Disease

Which immunofluorescence finding is associated with Goodpasture syndrome?

  • Granular pattern
  • Crescentic pattern
  • Linear pattern (correct)
  • Negative IF

What is the most likely outcome for children with Poststreptococcal Glomerulonephritis (PSGN)?

<p>Rarely progress to renal failure (1%) (B)</p> Signup and view all the answers

Which feature is commonly observed in the urinalysis of patients with nephritic syndrome?

<p>Dysmorphic red blood cells (A)</p> Signup and view all the answers

What clinical sign is most indicative of salt retention in nephritic syndrome?

<p>Periorbital edema (C)</p> Signup and view all the answers

Poststreptococcal Glomerulonephritis is mediated by which mechanism?

<p>Immune complex deposition (B)</p> Signup and view all the answers

Which demographic is most commonly affected by Poststreptococcal Glomerulonephritis?

<p>Young children (C)</p> Signup and view all the answers

Flashcards

Nephritic Syndrome

A group of kidney diseases characterized by inflammation and bleeding in the glomeruli, resulting in limited protein in urine (proteinuria), decreased urine output (oliguria), and increased waste in blood (azotemia).

Poststreptococcal Glomerulonephritis (PSGN)

A type of nephritic syndrome that develops after infection with group A β-hemolytic streptococcus, commonly causing skin (impetigo) or throat infections.

Immune Complex Deposition

The process in PSGN where antibodies and antigens (from the streptococcus) clump together and deposit in the glomeruli, triggering inflammation.

Rapidly Progressive Glomerulonephritis (RPGN)

A serious form of nephritic syndrome where the glomeruli rapidly deteriorate, leading to kidney failure within weeks or months.

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Crescents

A characteristic feature of RPGN, where a crescent-shaped structure forms in Bowman's space (surrounding the glomerulus). This crescent is composed of fibrin (a clotting protein) and macrophages (immune cells).

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Goodpasture Syndrome

An autoimmune disease targeting the basement membranes of the glomeruli (in the kidneys) and lungs. It leads to bleeding in urine (hematuria) and lungs (hemoptysis).

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Diffuse Proliferative Glomerulonephritis

The most common type of RPGN, caused by immune complex deposition. This occurs in a variety of conditions, including PSGN and Lupus.

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Pauci-Immune RPGN

A group of diseases causing RPGN where the cause is not fully understood. They are often linked to vasculitis (inflammation of blood vessels).

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Study Notes

Nephritic Syndrome

  • Characterized by glomerular inflammation and bleeding
  • Limited proteinuria (less than 3.5 g/day)
  • Oliguria and azotemia
  • Salt retention causing periorbital edema and hypertension
  • Red blood cell (RBC) casts and dysmorphic RBCs in urine
  • Biopsy shows hypercellular, inflamed glomeruli (Figure 12.12)

Poststreptococcal Glomerulonephritis (PSGN)

  • Arises after group A beta-hemolytic streptococcal infection (skin or pharynx)
  • Occurs 2-3 weeks post-infection
  • Presents with hematuria (cola-colored urine), oliguria, hypertension, and periorbital edema
  • Primarily affects children, but can affect adults
  • Immune complex deposition causes granular IF and subepithelial "humps" on electron microscopy (EM)
  • Treatment is supportive
  • Rare progression to renal failure in children
  • Some adults may develop rapidly progressive glomerulonephritis (RPGN)

Rapidly Progressive Glomerulonephritis (RPGN)

  • Nephritic syndrome progressing to renal failure within weeks to months
  • Immunofluorescence patterns vary depending on the disease, with linear deposits indicative of Goodpasture syndrome, granular deposits for diseases like PSGN, and negative deposits in some cases.
  • Goodpasture syndrome is characterized by antibodies against glomerular basement membrane collagen resulting in hematuria and hemoptysis.
  • Diffuse proliferative glomerulonephritis (most common immune complex mediated), Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome are examples of diseases that can have a granular IF pattern.

IgA Nephropathy (Berger Disease)

  • Most common nephropathy worldwide
  • Characterized by IgA immune complex deposition in the glomerular mesangium
  • Presents during childhood, often following mucosal infections (e.g., gastroenteritis)
  • Increased IgA production during infection
  • Characterized by episodic hematuria and presence of RBC casts
  • IgA immune complex deposition in the mesangium is visible on immunofluorescence
  • May progress slowly to renal failure

Alport Syndrome

  • Inherited defect in type IV collagen, often X-linked
  • Results in thinning and splitting of the glomerular basement membrane
  • Presents with isolated hematuria, sensory hearing loss, and ocular disturbances

Membranous Nephropathy

  • Thick glomerular basement membranes
  • Subepithelial deposits with 'spike and dome' appearance (Figure 12.9)

Kimmelstiel-Wilson nodules

  • Found in diabetic nephropathy (Figure 12.11)

Subendothelial deposits

  • Characteristic of membranoproliferative glomerulonephritis (Figure 12.10)

Hypercellular inflamed glomerulus

  • Characteristic of nephritic syndrome (Figure 12.12)

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