Glomerular Diseases and Kidney Anatomy
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Questions and Answers

What are glomerular diseases primarily characterized by?

  • Increased red blood cell production
  • Overproduction of urine
  • Excess absorption of nutrients
  • Damage to the glomeruli (correct)

Which of the following problems can result from damage to the glomeruli?

  • Proteinuria (correct)
  • Decreased blood pressure
  • Increased liver function
  • Improved kidney function

What is a significant consequence of untreated glomerular diseases?

  • Enhanced liver metabolism
  • Chronic kidney disease (correct)
  • Increased platelet count
  • Dehydration of the body

Which of the following is classified as Primary Glomerular Nephritis?

<p>Minimal change glomerular disease (C)</p> Signup and view all the answers

Which secondary glomerular disease is associated with autoimmune conditions?

<p>Wegener granulomatosis (A)</p> Signup and view all the answers

What is a common diagnostic method for examining glomerular diseases?

<p>Renal biopsy (A)</p> Signup and view all the answers

Which type of microscope is NOT typically used in the diagnosis of glomerular diseases?

<p>Infrared microscope (D)</p> Signup and view all the answers

What can hypertensive complications from glomerular diseases lead to?

<p>End-stage renal disease (D)</p> Signup and view all the answers

What is the most common malignant renal tumor affecting adults?

<p>Clear cell renal cell carcinoma (B)</p> Signup and view all the answers

Which clinical feature is most commonly associated with renal oncocytoma?

<p>Hematuria (D)</p> Signup and view all the answers

What primary cellular feature is associated with the development of renal oncocytomas?

<p>Accumulation of respiration-defective mitochondria (D)</p> Signup and view all the answers

Which type of tumor is predominantly found in children?

<p>Nephroblastoma (A)</p> Signup and view all the answers

What is the typical gross appearance of renal oncocytoma?

<p>Mahogany-brown cut surface with a fibrous scar (A)</p> Signup and view all the answers

Which stains would be positive in renal oncocytoma?

<p>CD117 / KIT and E-cadherin (B)</p> Signup and view all the answers

Which characteristic is NOT associated with renal oncocytomas?

<p>Presence of pseudocapsule (A)</p> Signup and view all the answers

What is the gender ratio commonly observed in cases of renal oncocytoma?

<p>2:1 male to female ratio (D)</p> Signup and view all the answers

What is the result of sudden and complete obstruction in terms of renal function?

<p>Reduced GFR leading to mild dilation (C)</p> Signup and view all the answers

What is a likely result of chronic obstruction in the kidneys?

<p>Cystic renal structures and significant atrophy (B)</p> Signup and view all the answers

What symptoms are associated with bilateral partial obstruction of the urinary tract?

<p>Polyuria and nocturia (C)</p> Signup and view all the answers

What is a common feature of chronic cases of renal obstruction?

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

How are renal stones primarily classified?

<p>By the minerals that comprise the stones (D)</p> Signup and view all the answers

What percentage of renal stones are made up of calcium oxalate?

<p>75% (B)</p> Signup and view all the answers

What could indicate a surgical emergency in renal obstruction cases?

<p>Acute complete bilateral obstruction (A)</p> Signup and view all the answers

What is likely the outcome if renal obstruction is diagnosed and relieved early?

<p>Return to normal kidney function (A)</p> Signup and view all the answers

What is a risk factor for renal vein thrombosis in patients with nephrotic syndrome?

<p>Increased synthesis of clotting factors (C)</p> Signup and view all the answers

What is the first-line treatment for nephrotic syndrome in children?

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

Which investigation should be performed for adults suspected of nephrotic syndrome?

<p>Renal biopsy (B)</p> Signup and view all the answers

In nephritic syndrome, which symptom is prominently present compared to nephrotic syndrome?

<p>Hypertension (A)</p> Signup and view all the answers

What is one of the main differences between nephrotic and nephritic syndromes?

<p>Presence of hematuria (C)</p> Signup and view all the answers

What is the appropriate management for hyperlipidemia in nephrotic syndrome?

<p>Statins (B)</p> Signup and view all the answers

Which of the following may indicate a need for renal biopsy in children with nephrotic syndrome?

<p>Failure of steroid treatment (A)</p> Signup and view all the answers

What is the standard duration for anticoagulation treatment with warfarin after diagnosed renal vein thrombosis in nephrotic syndrome?

<p>3 to 6 months (C)</p> Signup and view all the answers

What is a distinguishing feature of Membranoproliferative Glomerulonephritis (MPGN) type I?

<p>Lobular accentuation and double-contoured capillary walls (D)</p> Signup and view all the answers

Which microscopy technique is essential to diagnose amyloidosis?

<p>Congo red stain under polarized light (D)</p> Signup and view all the answers

What characterizes Goodpasture’s syndrome in clinical presentation?

<p>Rapidly progressive glomerulonephritis with hemoptysis (A)</p> Signup and view all the answers

What is a common treatment for anti-GBM disease?

<p>Steroids, cytotoxic agents and plasmapheresis (A)</p> Signup and view all the answers

Which pathological finding is typically seen in IgA Nephropathy?

<p>Recurrent gross or microscopic hematuria (D)</p> Signup and view all the answers

In which scenario is renal function likely to improve with therapy in anti-GBM disease?

<p>If intervention occurs early with serum creatinine levels of 4-5 mg/dl (A)</p> Signup and view all the answers

What is the underlying pathogenesis of Goodpasture’s syndrome?

<p>Auto-antibodies against the alpha-3 chain of type IV collagen (B)</p> Signup and view all the answers

Which microscopy finding is NOT associated with Goodpasture’s syndrome?

<p>Amorphous material under light microscopy (C)</p> Signup and view all the answers

What is a notable risk factor for pure squamous cell carcinoma of the urinary bladder?

<p>Indwelling catheter use for over 10 years (C)</p> Signup and view all the answers

Which of the following is a characteristic morphological feature of invasive urothelial carcinoma?

<p>Single cells invading lamina propria (B)</p> Signup and view all the answers

In the context of urothelial carcinoma in-situ, which marker is likely to be positively expressed?

<p>CK20 (A)</p> Signup and view all the answers

What genetic anomalies are associated with the pathogenesis of invasive urothelial carcinoma?

<p>TERT promoter mutations and TP53 mutations (D)</p> Signup and view all the answers

Which of the following statements about cystoscopy findings in urothelial carcinoma in-situ is true?

<p>Presence of multifocal erythematous mucosal patches. (D)</p> Signup and view all the answers

Which immunohistochemical marker is typically negative in pure squamous cell carcinoma of the urinary bladder?

<p>Uroplakins (B)</p> Signup and view all the answers

What is the prognosis for pure squamous cell carcinoma of the urinary bladder compared to conventional urothelial carcinoma?

<p>Worse than conventional urothelial carcinoma (B)</p> Signup and view all the answers

Which of the following is NOT a pathway implicated in the pathogenesis of certain urinary bladder cancers?

<p>WNT pathway (A)</p> Signup and view all the answers

Flashcards

Glomerular Diseases

Conditions that damage the glomeruli, the tiny filters in the kidneys responsible for removing waste and excess fluids from the blood.

Proteinuria

Excess protein in urine, often a sign of glomerular damage.

Hematuria

Blood in urine, another indicator of potential glomerular issues.

Glomerular Filtration Rate (GFR)

A measure of how well your kidneys filter waste products. It's a key indicator of kidney function.

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Primary Glomerular Diseases

Conditions that directly affect only the kidneys.

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Secondary Glomerular Diseases

Conditions that affect both the kidneys and other organs.

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Light Microscopy

A technique used in renal biopsy to examine the structure of glomeruli, tubules, and interstitium.

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Renal Biopsy

A procedure where a small sample of kidney tissue is removed for examination under a microscope.

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Hydronephrosis

Swelling of the kidney due to urine build-up caused by an obstruction in the urinary tract.

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Sudden & Complete Obstruction

A sudden, total blockage in the urinary tract leading to a decrease in GFR, mild dilation, and atrophy of kidney tissue.

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Subtotal or Intermittent Obstruction

A partial or on-and-off blockage in the urinary tract leading to normal GFR and progressive dilation.

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Chronic Hydronephrosis

Long-term kidney swelling caused by persistent obstruction, leading to cortical atrophy, interstitial fibrosis, and significant parenchymal atrophy.

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Renal Colic

Sharp pain in the back and side caused by kidney stones blocking the ureter.

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Urinary Symptoms

Problems with urination like difficulty starting, frequent urination, or pain during urination, often caused by prostatic enlargement.

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Renal Stones

Solid masses made of crystals, protein or other substances that form inside the urinary tract and can cause obstruction.

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Calcium Oxalate Stones

The most common type of kidney stone, accounting for 75% of all stones

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Membranoproliferative Glomerulonephritis (MPGN) Type I

A kidney disease characterized by mesangial and endocapillary proliferation, producing a lobular appearance with double-contoured capillary walls.

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Diabetic Glomerulosclerosis

A complication of diabetes characterized by thickening of the glomerular basement membrane (GBM) and increased mesangial matrix.

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Amyloidosis

A disease caused by the deposition of amyloid protein in tissues, which appears as amorphous material under light microscopy and as 10nm fibrils.

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Anti-GBM Disease (Goodpasture’s Syndrome)

An autoimmune disease where antibodies attack the non-collagenous domain of the alpha-3 chain of type IV collagen, affecting both the glomerular and alveolar basement membranes.

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Goodpasture’s Syndrome: Glomeruli

The glomeruli show fibrinoid necrosis, glomerular necrosis, crescent formation, and fibrin extravasation.

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Goodpasture’s Syndrome: Electron Microscopy (EM)

Electron microscopy shows linear IgG deposition without any deposits.

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Goodpasture’s Syndrome: Clinical Course

Treatment includes steroids, cytotoxic agents, and plasmapheresis. Early intervention improves renal function and resolves pulmonary hemorrhages. Delayed therapy leads to irreversible renal failure.

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IgA Nephropathy

A kidney disease characterized by recurrent hematuria, usually non-nephrotic proteinuria, and no associated systemic disease.

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Nephrotic Syndrome: Hypercoagulability

Increased risk of blood clots due to elevated clotting factors and platelet abnormalities in nephrotic syndrome.

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Nephrotic Syndrome: Hyperlipidemia

High levels of lipids (fats) in the blood, often seen in nephrotic syndrome due to the liver's response to reduced oncotic pressure.

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Renal Biopsy in Nephrotic Syndrome

Usually recommended for adults with nephrotic syndrome to determine the underlying cause. In children, biopsy is only needed if steroid therapy fails.

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Nephrotic Syndrome: Renal Vein Thrombosis

A blood clot forming in the renal vein, a potential complication of nephrotic syndrome due to hypercoagulability.

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Nephritic Syndrome vs Nephrotic Syndrome: Key Difference

Nephritic syndrome is characterized by the presence of blood in the urine (hematuria), whereas nephrotic syndrome primarily involves protein loss.

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Nephritic Syndrome: Clinical Features

Abrupt onset of hematuria (blood in urine), proteinuria, edema, hypertension, and possible kidney impairment.

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Nephritic Syndrome: Causes

Can be caused by various conditions including post-streptococcal infection, primary glomerulonephritis disorders, and secondary conditions like HSP or vasculitis.

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Nephritic Syndrome: RBC Casts

Red blood cells clumped together in the urine, a characteristic finding in nephritic syndrome due to inflammation in the kidney's filtering units.

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Urothelial Carcinoma In-Situ (CIS)

A non-invasive tumor confined to the urothelial lining of the bladder, characterized by cytologic atypia, but not full invasion. It frequently recurs and can progress to invasive cancer.

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What are some common IHC markers for CIS?

Positive: CK20 (full thickness), p53, increased Ki67. Negative: CD44 (or decreased).

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Conventional Urothelial Carcinoma

Invasive carcinoma of the bladder, characterized by nests, sheets, cords, or single cells invading beyond the lamina propria. It has a mixed architectural pattern and often occurs in the 7th decade or later.

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Key IHC markers in conventional urothelial carcinoma

Positive: GATA3, HMWCK, CK7, CK20, P63, uroplakin. Negative: PAX8.

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Pure Squamous Cell Carcinoma of the Bladder

A rare bladder cancer characterized by 100% squamous morphology with intercellular bridges, keratin pearls, and keratinized cells. It shouldn't contain conventional urothelial carcinoma.

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Risk factors for pure squamous cell carcinoma

Indwelling catheter >10 years, bladder stones, smoking, Schistosoma haematobium infection.

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Adenocarcinoma of the Bladder

A rare bladder cancer characterized by 100% adenocarcinoma morphology with varying patterns (enteric/colonic, mucinous, signet ring cell, or mixed). It shouldn't contain conventional urothelial carcinoma.

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What is a key difference between conventional urothelial carcinoma and CIS?

Conventional urothelial carcinoma invades the lamina propria and beyond, while CIS is confined to the urothelial lining.

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Study Notes

Glomerular Diseases

  • Glomerular diseases are a group of conditions that damage the glomeruli, the kidney's microscopic filtration units.
  • Damage impairs kidney function, leading to proteinuria (excess protein in urine), hematuria (blood in urine), reduced glomerular filtration rate (GFR), and in severe cases, chronic kidney disease or kidney failure.
  • Glomerular diseases have significant morbidity and mortality, leading to complications like hypertension, nephrotic syndrome, and end-stage renal disease.

Kidney Anatomy

  • The kidney has an outer cortex and an inner medulla.
  • Blood vessels (renal artery and vein) and the ureter connect to the kidney.
  • The nephron is the functional unit, consisting of the glomerulus, Bowman's capsule, proximal convoluted tubule, loop of Henle, distal convoluted tubule, and collecting duct.
  • The glomerulus is a network of capillaries that filters blood.
  • The Bowman's capsule surrounds the glomerulus.

Renal Anatomy

  • The glomerulus is a network of capillaries filtering blood.
  • The proximal convoluted tubule is the first part of the tubule system.
  • The loop of Henle is a U-shaped loop that moves the filtrate from the cortex to the medulla.
  • The distal convoluted tubule is the last part of the tubule system
  • The collecting duct collects the filtrate from the distal convoluted tubules.
  • Nephrons are the functional units of the kidney.

Glomerulus Detail

  • The glomerulus consists of capillary loops with fenestrations in the endothelium.
  • The glomerular basement membrane sits between the endothelium and podocytes.
  • Podocytes have foot processes that wrap around the capillaries, creating filtration slits.
  • Mesangial cells are found within the glomerulus.

Classification of Glomerular Diseases

  • Primary glomerulonephritis (GN): disease affects the kidney only.
    • Minimal change disease (Lipoid nephrosis)
    • Acute diffuse proliferative GN: Post-streptococcal GN, Non-post-streptococcal GN
    • Rapidly progressive GN
    • Membranous GN
    • Membranoproliferative GN
    • Chronic GN
  • Secondary GN: disease affects the kidney and other organs.
    • Systemic lupus erythematosus (SLE)
    • Polyarteritis nodosa (PAN)
    • Wegener granulomatosis
    • Diabetes mellitus (diabetic nephropathy)
    • Goodpasture syndrome
    • Amyloidosis

Diagnosis of Glomerular Diseases

  • Renal biopsy is taken to study glomerular disease.
  • Light microscopy examines glomeruli, tubules, and interstitium.
  • Immunofluorescence microscopy detects immunoglobulin deposits in the glomeruli.
  • Electron microscopy detects the site of the immune complex deposits, such as sub-epithelial, sub-endothelial, mesangial or basement membrane.

Minimal Change Disease

  • Often affects children
  • Characterized by an excellent prognosis
  • Usually responds well to corticosteroids
  • Histological findings include fusion of foot processes of podocytes without immune complex deposits.

Post-Streptococcal GN

  • Often begins after a streptococcal infection.
  • Characterized by an acute inflammatory response.
  • Light microscopy shows proliferation of endothelial and mesangial cells.
  • Immune complex deposition occurs, leading to acute inflammation

Membranous Glomerulopathy

  • Characterized by thickening of the glomerular basement membrane.

Membranoproliferative GN (MPGN) (Type I)

  • Includes mesangial and endocapillary proliferation, seen as lobular accentuation and double-contoured capillary walls.
  • Glomerular immune complex deposits are seen.

Diabetic Glomerulosclerosis

  • Results from long-term diabetes
  • Thickened glomerular basement membranes with mesangial matrix increase

Amyloidosis

  • Deposition of amyloid protein in the kidneys
  • Amorphous material is visible on light microscopy.

Anti-GBM Disease (Goodpasture's Syndrome)

  • Characterized by circulating antibodies against non-collagenous domains of collagen type IV.
  • Leads to glomerular and alveolar basement membrane damage.
  • Renal biopsy will show glomeruli necrosis.

IgA Nephropathy

  • Characterized by frequent gross/microscopic hematuria
  • Mesangioproliferative (most common) is a defining feature on IF.

Henoch-Schönlein Purpura

  • Systemic vasculitis that commonly manifests with purpuric rash, abdominal pain, arthralgias, and renal manifestations (IgA nephropathy).

Systemic Lupus Erythematosus (SLE)

  • Multi-system autoimmune disease.
  • Common in women.
  • Kidney involvement (lupus nephritis) causes variable morphological changes.

Chronic Glomerulonephritis

  • Associated with chronic kidney damage to glomeruli, tubules, and blood vessels.
  • Often characterized by diffuse interstitial fibrosis and bilateral kidney contraction.

Nephrotic Syndrome

  • Characterized by: proteinuria, hypoproteinemia, edema, and hyperlipidemia.
  • Minimal change disease (MCD) is a common cause in children.
  • Membranous nephropathy is a common cause in adults.
  • Diagnosis often requires a renal biopsy.
  • Complications may include an elevated risk of infection, thromboembolism, and hyperlipidaemia

Nephritic Syndrome

  • Characterized by: haematuria, oliguria, periorbital edema, and hypertension.
  • Post-streptococcal GN is a typical cause in children.

Pyelonephritis

  • Infection of the kidney tissue (tubules, interstitium, and renal pelvis)
  • Acute pyelonephritis is typically caused by bacterial infection;
  • Chronic pyelonephritis is frequently caused by urinary tract reflux or obstruction.
  • Symptoms typically include fever, flank pain.

Acute Interstitial Nephritis (AIN)

  • Characterized by diffuse interstitial infiltrates with numerous inflammatory cells, particularly lymphocytes and eosinophils.
  • Often triggered by medications (e.g., antibiotics, NSAIDs) or infections.
  • Typically associated with an abrupt decline in kidney function, often needing immediate treatment.

Lower Urinary Tract Infections (UTIs)

  • Infections affecting the bladder/ureters/urethra
  • UTIs usually present with symptoms like painful urination, urgency, frequency

Obstructive Uropathy

  • Obstruction to the flow of urine in the urinary tract resulting in hydronephrosis
  • Causes can include kidney stones, tumors, and strictures (narrowing within the urinary tract)

Urolithiasis (Renal Calculi)

  • Formation of masses of crystals, protein, or various foreign bodies within the urinary tract that can lead to obstruction.
  • Common types include Calcium oxalate, Struvite, Uric acid and Cystine
  • Factors promoting stone formation include stasis, high concentration of stone components, dehydration, and pH alteration.
  • Typical presentation is pain (renal colic), hematuria, and recurrent UTIs

Renal Tumors

  • Includes benign and malignant cases.
  • The most common malignant tumor is the clear cell renal cell carcinoma.
  • Other types include oncocytoma, nephroblastoma.

Tumors of Urinary Bladder/Urothelial Tract

  • Primarily affect urothelial cells lining the urinary bladder and ureter.
  • Includes benign and malignant tumors, with malignant tumors presenting with more severe clinical outcomes
  • Commonly include Urothelial Papilloma, Invasive Urothelial Carcinoma, Pure squamous cell carcinoma and Adenocarcinoma

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Glomerular Diseases - PDF

Description

This quiz covers glomerular diseases, their impact on kidney function, and the anatomy of the kidney. Participants will learn about kidney structures such as the glomerulus and the nephron, as well as the implications of glomerular damage. Test your knowledge and understanding of these critical aspects of renal health.

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