Glomerular Diseases Overview
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Questions and Answers

What is the primary function of the glomeruli in the kidneys?

  • To regulate blood pressure
  • To produce hormones
  • To filter waste and excess fluids from the blood (correct)
  • To maintain electrolyte balance

    • Which of the following is a consequence of damage to the glomeruli?

  • Proteinuria (correct)
  • Hyperfiltration
  • Fluid retention in the bladder
  • Urinary incontinence

    • What is a significant characteristic of primary glomerular disease?

  • It usually affects multiple organs
  • It leads to immediate kidney failure
  • It is always caused by diabetes
  • It primarily affects the kidneys only (correct)

    • Which of the following diseases is classified as a secondary glomerular disease?

    <p>Systemic lupus erythematosus</p> Signup and view all the answers

    What is the significance of early detection of glomerular diseases?

    <p>It improves patient outcomes and prevents progression</p> Signup and view all the answers

    Which type of microscope is used to examine the structure of glomeruli and tubules in a renal biopsy?

    <p>Light microscope</p> Signup and view all the answers

    What can chronic glomerular diseases potentially lead to?

    <p>Renal replacement therapy necessity</p> Signup and view all the answers

    Which of the following is NOT seen as a complication of glomerular diseases?

    <p>Diabetic ketoacidosis</p> Signup and view all the answers

    What type of microscopy is used to detect the site of immune complex in the glomeruli?

    <p>Electron microscopy</p> Signup and view all the answers

    What is the primary pathological change observed in minimal change disease?

    <p>Fusion of foot processes of podocytes</p> Signup and view all the answers

    Which of the following is associated with post-streptococcal glomerulonephritis?

    <p>Neutrophil casts in tubules</p> Signup and view all the answers

    Which feature is NOT typically found in minimal change disease under light microscopy?

    <p>Proliferation of endothelial cells</p> Signup and view all the answers

    What is a common clinical presentation of post-streptococcal glomerulonephritis?

    <p>Mild bilateral kidney enlargement with petechial hemorrhages</p> Signup and view all the answers

    What typically happens to proteinuria in patients with minimal change disease after corticosteroid treatment?

    <p>Shows complete resolution</p> Signup and view all the answers

    What type of immune deposits are typically observed in post-streptococcal glomerulonephritis under immunofluorescence?

    <p>Granular C3 and Ig G</p> Signup and view all the answers

    Which of these is a possible fate of minimal change disease?

    <p>Resolution with corticosteroids</p> Signup and view all the answers

    What is the most common age group for the occurrence of Nephroblastoma (Wilms tumor)?

    <p>3 - 4 years</p> Signup and view all the answers

    Which genetic alteration is implicated in the tumorigenesis of Nephroblastoma?

    <p>WT1 gene at chromosome 11p13</p> Signup and view all the answers

    What is a common clinical presentation of Nephroblastoma?

    <p>Abdominal mass</p> Signup and view all the answers

    Which syndrome is associated with aniridia in Nephroblastoma patients?

    <p>WAGR Syndrome</p> Signup and view all the answers

    What is the estimated percentage of Nephroblastoma cases that are familial?

    <p>1 - 2%</p> Signup and view all the answers

    What is indicated by an increase in alkaline phosphatase in Nephroblastoma patients?

    <p>Presence of bone metastasis</p> Signup and view all the answers

    Which of the following is NOT a symptom associated with Nephroblastoma?

    <p>Nausea</p> Signup and view all the answers

    Nephroblastoma has a slight female preponderance. What could this imply?

    <p>More cases in girls than boys</p> Signup and view all the answers

    Which risk factor is commonly associated with both low-grade and high-grade non-invasive papillary urothelial carcinoma?

    <p>Smoking</p> Signup and view all the answers

    What histopathological feature distinguishes high-grade non-invasive papillary urothelial carcinoma from low-grade?

    <p>Marked cytologic atypia</p> Signup and view all the answers

    What is the typical age group for patients diagnosed with high-grade non-invasive papillary urothelial carcinoma?

    <p>Older than 70 years</p> Signup and view all the answers

    Which of the following IHC markers would be positive in high-grade non-invasive papillary urothelial carcinoma?

    <p>CK20</p> Signup and view all the answers

    What is the prognosis for patients with high-grade non-invasive papillary urothelial carcinoma regarding progression to invasive urothelial carcinoma?

    <p>25%</p> Signup and view all the answers

    What is one of the distinguishing features of urothelial carcinoma in-situ compared to non-invasive papillary tumors?

    <p>Flat lesion with no papillary architecture</p> Signup and view all the answers

    Which mutation is a significant part of the pathogenesis of high-grade non-invasive papillary urothelial carcinoma?

    <p>TERT promoter mutations</p> Signup and view all the answers

    Which of the following features would NOT be expected in the morphology of low-grade non-invasive papillary urothelial carcinoma?

    <p>Marked cytologic atypia</p> Signup and view all the answers

    What is the characteristic cellular infiltrate associated with Acute Interstitial Nephritis (AIN)?

    <p>Eosinophils</p> Signup and view all the answers

    Which symptom is NOT typically associated with cystitis?

    <p>Fever</p> Signup and view all the answers

    What can potentially reverse Acute Interstitial Nephritis (AIN)?

    <p>Identification and treatment of the underlying cause</p> Signup and view all the answers

    What is the minimum bacterial count in urine that is considered significant for diagnosing a urinary tract infection (UTI)?

    <p>10⁵ cfu/ml</p> Signup and view all the answers

    Which factor does NOT normally protect against urinary tract infections?

    <p>Presence of bacteria in urine</p> Signup and view all the answers

    Which type of cystitis is characterized by inflammation not resulting from an infection?

    <p>Non-bacterial cystitis (Interstitial Cystitis)</p> Signup and view all the answers

    What is one of the major manifestations of interstitial cystitis commonly seen in women aged 20 to 30?

    <p>Chronic pelvic pain</p> Signup and view all the answers

    In terms of urine characteristics, what symptom may indicate a urinary tract infection?

    <p>Foul-smelling urine</p> Signup and view all the answers

    What is a defining characteristic of nephrotic syndrome related to protein levels?

    <p>Proteinuria of greater than 3.5g/24 hrs</p> Signup and view all the answers

    Which statement about the pathophysiology of nephrotic syndrome is correct?

    <p>Alternative pathology is responsible for oedema instead of changes in oncotic pressure.</p> Signup and view all the answers

    Which of the following is NOT considered a primary cause of nephrotic syndrome?

    <p>Diabetes mellitus</p> Signup and view all the answers

    Which complication is commonly associated with nephrotic syndrome?

    <p>Increased susceptibility to infections</p> Signup and view all the answers

    What type of oedema is commonly observed in nephrotic syndrome?

    <p>Pitting oedema particularly in the limbs and around the eyes</p> Signup and view all the answers

    Which of the following is a secondary cause of minimal change disease?

    <p>HIV infection</p> Signup and view all the answers

    What is a potential differential diagnosis for nephrotic syndrome?

    <p>Cardiac failure</p> Signup and view all the answers

    Which of the following clinical signs is associated with nephrotic syndrome?

    <p>Genital oedema</p> Signup and view all the answers

    Signup and view all the answers

    Study Notes

    Glomerular Diseases

    • Glomerular diseases are a group of conditions that damage the glomeruli, the kidney's microscopic filtration units.
    • Damage to the glomeruli impairs kidney function, causing issues like proteinuria, hematuria, reduced glomerular filtration rate (GFR), and chronic kidney disease or failure.
    • Glomerular diseases can result in significant morbidity and mortality. Complications include hypertension, nephrotic syndrome, and end-stage renal disease, necessitating dialysis or kidney transplantation.
    • Early detection and appropriate management are crucial to prevent progression and improve patient outcomes.

    Human Kidney Anatomy

    • The kidney has an external and internal view. Diagrams of both are shown. Cortical blood vessels, interlobular blood vessels, renal artery, renal vein, ureter, minor calyx, major calyx, medulla, and capsule are depicted.

    Renal Anatomy

    • The functional unit of the kidney is the nephron; a diagram is provided. Components include the glomerulus, proximal convoluted tubule, distal convoluted tubule, collecting duct. Blood flow is depicted from renal artery to renal vein.

    Glomerulus Structure

    • Diagrams illustrating the microscopic structure of the glomerulus are present. Components include capillary loops, red blood cells, proximal tubule, urinary space, mesangium, mesangial cells, mesangial matrix, parietal epithelium, endothelium, foot processes, basement membrane and fenestrae in endothelium. All components are labeled directly.

    Classification of Glomerular Diseases

    • Primary Glomerular Diseases: affect only the kidneys.
      • Minimal change disease (lipoid nephrosis)
      • Acute diffuse proliferative GN (post-streptococcal and non-post-streptococcal)
      • Rapidly progressive GN
      • Membranous GN
      • Membranoproliferative GN
      • Chronic GN
    • Secondary Glomerular Diseases: affect other organs and also affect the kidneys.
      • Systemic lupus erythematosus (SLE)
      • Polyarteritis nodosa (PAN)
      • Wegener granulomatosis
      • Diabetes mellitus (diabetic nephropathy)
      • Goodpasture syndrome
      • Amyloidosis

    Minimal Change Glomerular Disease

    • Etiology & Pathogenesis: Chemical change in glomerular basement membrane causing protein loss.
    • Grossly: Mild bilateral kidney enlargement.
    • LM (Light Microscope): No abnormalities.
    • IF (Immunofluorescence): No immune deposits.
    • EM (Electron Microscope): Fusion of foot processes of epithelial cells (podocytes).
    • Clinical Picture (CP): Affects children and young adults; causes nephrotic syndrome.
    • Fate: Excellent prognosis; most patients respond to corticosteroids with complete resolution of proteinuria.

    Post-streptococcal Glomerular Nephritis

    • Etiology & Pathogenesis: Immune complex reaction; nephrogenic strains of group A beta hemolytic streptococci + IgG, complex deposited in glomeruli with subsequent complement activation -> acute inflammation.
    • Grossly: Mild bilateral kidney enlargement with petechial hemorrhages.
    • LM (Light Microscope):
      • Glomeruli: proliferation of endothelial and mesangial cells; glomerular capillaries containing neutrophils; Bowman's space shows neutrophils, RBCs, and some albumin.
      • Tubules: lining cells swollen; lumens show casts (RBC casts, neutrophil casts, and hyaline casts).
      • Interstitium: acute inflammatory reaction.
    • IF (Immunofluorescence): Deposition of IgG and C3.
    • EM (Electron Microscope): Subepithelial immune complex deposit (humps)
    • Clinical Picture (CP): Young child abruptly develops malaise, fever, nausea, oliguria, and hematuria (smoky or cocoa-colored urine) 1-2 weeks after recovery from a sore throat.
    • Fate: More than 95% recover with therapy. The rest may develop rapidly progressive glomerulonephritis or chronic glomerulonephritis. In adults, prognosis is bad.

    Diagnosis of Glomerular Diseases

    • A renal biopsy is taken and examined using three types of microscopes:
      • Light microscope (LM): to examine the structure of glomeruli, tubules, and interstitium.
      • Immunofluorescent microscope (IF): to identify the type of deposited immunoglobulin in the glomeruli.
      • Electron microscope (EM): to determine the site of immune complexes (sub-epithelial, sub-endothelial, mesangial or basement membrane).

    Other Glomerular Diseases (Brief Overviews)

    • Presents information on both light and electron microscopy views for various aspects of kidney disease. Includes Membranous glomerulopathy, Membranoproliferative GN (Type 1), Diabetic glomerulosclerosis, Amyloidosis, Anti-GBM disease (Goodpasture's syndrome), IgA nephropathy, Henoch-Schönlein Purpura, SLE, Chronic Glomerulonephritis, and other, more general information.

    Nephrotic and Nephritic Syndromes

    • Nephrotic Syndrome: formed of: proteinuria (>3.5g/24 hrs), hypoproteinemia (<30g/L), edema, and hypercholesterolemia. Minimal change disease in children and membranous nephropathy in adults are common causes. Potential complications = infections, thromboembolism and hyperlipidaemia.
    • Nephritic Syndrome: formed of: hematuria, oliguria, periorbital edema, and hypertension. Post-streptococcal GN is a common cause. Electrolyte imbalances and hypertension may be present possibly because of glomerular damage.

    Pyelonephritis and Acute Interstitial Nephritis (AIN)

    • Pyelonephritis: Inflammation of the tubules, interstitium, and renal pelvis. Two forms:
      • Acute pyelonephritis: Often caused by bacterial infection.
      • Chronic pyelonephritis: More complex, infection plays a role, but reflux and obstruction are also factors. Symptoms of acute pyelonephritis: Fever, chills, tachycardia, tachypnea, flank pain, abdominal pain, nausea, vomiting, dysuria, nocturia, symptoms of recent cystitis or UTI treatment. Predisposing factors = Urinary tract obstruction, Instrumentation, Vesicoureteral reflex, Pregnancy, Preexisting renal lesions, Diabetes mellitus and Immunosuppression, possible complications = Papillary necrosis, Pyonephrosis, and Perinephric abscess.
    • Acute Interstitial Nephritis (AIN): Inflammatory condition affecting kidney interstitium (area surrounding renal tubules). Causes = Medications (antibiotics, NSAIDs), Infections, Autoimmune Diseases. Histology = Presence of inflammatory cells (lymphocytes, eosinophils, and sometimes plasma cells) within the interstitium, possible damage to tubules and edema.

    Lower Urinary Tract Infections

    • Urinary Tract Infections (UTIs): Defined by the presence of microorganisms in bladder urine with or without symptoms or renal disease. Factors normally protecting against UTIs: Washing out bacteria, Low pH, High osmolarity, Tamm-Horsfall protein, Ureterovesical junction valve action.
    • Cystitis: Inflammation of bladder; two forms:
      • Bacterial (acute): common, predisposing factors = bladder calculi, urinary obstruction, diabetes mellitus, instrumentation, immune deficiency.
      • Non-bacterial (Interstitial Cystitis): Chronic, non-infectious. Symptoms = Frequency, Urgency, Dysuria, Suprapubic Pain, Cloudy or foul-smelling urine. Etiology includes women, tuberculous cystitis, Candida albicans infections, Schistosomiasis, Chlamydia and Mycoplasma infections.
    • Relevant other lower UTI's are also described.

    Obstructive Uropathy

    • Obstructive uropathy: Functional or anatomic obstruction of urinary tract flow.
      • Causes = Calculus, Chronic infection, Pyogenic granulomatous, Ureter anomalies, Stricture stenosis, Neoplasm, Compression, Trauma.
      • Location
      • Completeness
      • Involvement of one or both upper urinary tracts
      • Duration
      • Cause of any obstruction
      • Physiologic view of urine flow.
      • Pathophysiology (mechanisms) of obstruction related to causes.
    • Lower Urinary Tract Obstruction: Urethral stricture, Prostate enlargement, pelvic organ prolapse, Low bladder wall compliance.
      • Neurogenic bladder - Dyssynergia, Detrusor hyperreflexia-overactive, Detrusor areflexia-underactive and Overactive bladder syndrome (OBS) = Frequency, urgency, nocturia.

    Urolithiasis (Renal Calculi)

    • Definition: Masses of crystals, protein, or other substances forming within and obstructing the urinary tract.
    • Types of Renal Stones:
      • Calcium oxalate/phosphate (75%)
      • Struvite (10-15%)
      • Uric acid (6%)
      • Cystine (1-2%)
      • Unknown (?10%)
    • Pathogenesis: Variables related to each type of stone.
    • Risk Factors: Increased concentration of stone constituents (changes in urinary pH, low pH higher risk), Decrease in urine volume, and urinary tract infection.
    • Sites: Unilateral in 80%; common sites = renal calyces, pelvis; stones can be small (2-3 mm), or large (Staghorn).
    • Clinical Presentation: Symptoms may occur from obstruction, ulceration, and subsequent bleeding. Smaller stones = pain, hematuria, recurrent UTI. Asymptomatic can occur.
    • Laboratory Diagnosis:
      • UEC (Urinalysis shows hematuria, crystals, and pyuria).
      • BUN
      • Other modes of investigation = USS, X-rays, CT scans.
    • Prognosis: Generally good; depends on the underlying cause.

    Renal Tumors

    • Renal Cell Carcinoma: Most common malignant tumor. Epidemiology = Affects adults, 6th/7th decade. Risk factors = smoking, obesity, HTN, exposure to chemicals, estrogen therapy and secondary to dialysis. Clinical presentation = Silent, hematuria, flank pain.

    • Renal Oncocytoma: Rare, benign tumor. Epidemiology = More common in men. Clinical presentation = Asymptomatic, flank pain, and hematuria.

    • Nephroblastoma (Wilms Tumor): Malignant embryonal tumor occurring in children; common in 3-4 year-old children. Etiology = Genetic alterations. Clinical presentation = Abdominal mass, with sometimes associated pain/hematuria.

      • Associated syndromes = WAGR, Denys-Drash, Beckwith-Wiedemann

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    Description

    This quiz covers essential concepts related to glomerular diseases, including their functions, characteristics, and consequences of damage. Learn about microscopy techniques used in renal biopsies and the clinical presentations associated with various glomerular conditions. Test your knowledge on both primary and secondary glomerular diseases.

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