3rd Shifting Kidneys, Lower Urinary Tract and Male Urinary Tract Flashcards
Four basic morphologic components of the kidneys
Glomeruli
Interstitium
Tubules
Blood vessels
biochemical abnormality that refers to elevation of the BUN and creatinine levels
Azotemia
Azotemia + clinical signs and symptoms
Uremia
Examples of uremic symptoms
anorexia, lethargy, dec mental acuity, coma
Nephritic syndrome is a glomerular disease dominated by the acute onset of what symptoms?
▪grossly visible hematuria
▪mild to moderate proteinuria
▪hypertension
Examples of diseases associated/presenting with Nephritic syndrome
- Post streptococcal glomerulonephritis
- Rapidly progressive (Crescentic) glomerulonephritis
- Goodpasture’s syndrome
- Glomerulonephritis with vasculitis
Characteristic symptoms in Nephrotic Syndrome
Heavy proteinuria (>3.5 g/day), hypoalbuminemia, severe edema, hyperlipidemia and lipiduria
Examples of diseases associated/presenting with Nephrotic syndrome
- Lipoid nephrosis
- Focal segmental glomerulosclerosis
- Membranous glomerulosclerosis
- Diabetic nephropathy
- Amyloidosis
Oliguria or anuria and recent onset of azotemia
Acute renal failure
Prolonged signs and symptoms of uremia
Chronic renal failure
End result of chronic renal parenchymal diseases
Chronic renal failure
Characteristic symptoms of Renal tubular defects
polyuria, nocturia and electrolytes disorders
examples of diseases presenting with renal tubular defects
- Inherited (familial nephrogenic diabetes, cystinuria, renal tubular acidosis)
- Acquired (lead nephropathy)
Characteristic symptoms of UTI
Bacteriuria, Pyuria
Characteristic symptoms of Nephrolithiasis
Severe spasm of pain (renal colic) and hematuria
What disease is characterized by Nephritic symptoms with a rapid decline in GFR (hours-days)
Rapid Progressive (Crescentic) Glomerulonephritis
major cause of death from renal diseases
Chronic Renal Failure
4 stages of renal disease
- Diminisehed renal reserve (50%GFR)
- Renal insufficiency (20%-50% GFR)
- CRF (20%-25% GFR)
- End stage renal Disease (<5% GFR)
What are the symptoms of Renal insufficiency (Stage II)
polyuria, hypertension, nocturia, anemia, azotemia
What are the symptoms of CRF (Stage III)
Uremia, edema, metabolic acidosis, hyperkalemia
most common causes of Chronic Kidney Disease
Chronic Glumerulonephritis
What are the clinical manifestations of Glomerular Disease
Nephritic, Nephrotic, RPGN, CRF, isolated urinary abnormalities
Histologic alterations in glomerular diseases (4)
- Hypercellularity
- Basement Membrane Thickening
- Hyalinization/Hyalinosis
- Sclerosis
Proliferating parietal cells and infiltrating leukocytes
Crescents (Present in hypercellularity)
Deposition of amorphous electron dense materials in the endothelial or epithelial side of the basement membrane
Basement Membrane Thickening
Hyalinization of glomerulus is a common feature in what disease?
Focal segmental glomerolusclerosis
Accumulation of extracellular collagenous matrix, either confined to the mesangial areas (DM) or involving the capillary loops
Sclerosis
Pathogenesis of Glomerular Injury (How are they injured?)
ANTIBODY-MEDIATED INJURY
CYTOTOXIC ANTIBODIES
CELL-MEDIATED IMMUNE INJURY
ACTIVATION OF ALTERNATIVE COMPLEMENT PATHWAY
Forms of Antibody Mediated Glomerular Injury:
- Intrinsic (Immune complex deposition involving intrinsic and in situ renal antigens)
- Extrinsic (Circulating Immune complex Glumerulonephritis)
Examples “Immune complex deposition involving intrinsic and in situ renal antigens” form of glomerular injury
- Heymann Nephritis (granular)
- Ab against planted Antigens (granular)
- Anti GBM Ab-induced GN (linear)
Granular immune deposits in the visceral epithelium
Heymann Nephritis
Ab to component of the GBM
Masugi or nephrotoxic nephritis
Anti GBM Ab-induced GN
what happens to:
a. Cationic immunogens
b. Anionic immunogens
c. Neutral immunogens
a. cross the BM and are deposited in the subepithelium
b. trapped subendothelially or NOT nephritogenic
c. deposited in the mesangium
Mediators of Glomerular Cellular Injury
Celllular mediators:
Neutrophils, T cells, Platelets, mesangial cells
Soluble mediators:
Complement, eicosanoids, cytokines, chemokines, coagulation system
Two major histologic characteristics of progressive renal damage:
- Focal Segmental Glomerulosclerosis (FSGS)
2. Tubulointersititial fibrosis
develop proteinuria even if the primary dse was non glomerular
Focal Segmental Glomerulosclerosis (FSGS)
adaptive change that occurs in the unaffected glomeruli of diseased kidneys; Compensatory hypertrophy of the remaining glomeruli
Glomerulosclerosis
in Tubulointerstitial Fibrosis, what is the cause of direct injury to and activation of tubular cells
Proteinuria
Primary Glomerulopathies (8)
Nephritic syndrome Nephrotic syndrome Membranous nephropathy Minimal-change disease HIV-associated nephropathy RPGN FSGS MPGN
2 types of Acute Proliferative GN
- Poststreptococcal GN
2. Post-infectious GN
Syndrome associated with severe glomerular injury ;NOT a specific etiologic form of glomerulonephritis
RPGN (Crescentic GN)
Rapid and progrssive loss of renal function associated with severe oliguria and signs of nephritic syndrome
RPGN (Crescentic GN)
Enlarged kidneys with petechial hemorrhages on its cortical surface
RPGN (Crescentic GN)
Type 1 RPGN characteristics:
- Anti-GBM Antibody
- Antigen: noncollagenous portion of the α3 chain of collagen type IV
- Linear deposits of IgG
- C3 in the GBM
Type 2 RPGN characteristics:
- Immune Complex deposition
- Granular pattern of staining
Type 3 RPGN characteristics:
- Pauci-Immune
- ANCA-associated
- Lack of anti-GBM antibodies or immune complexes
amount of massive proteinuria in Nephrotic syndrome
3.5 g/day
Most common cause of nephrotic syndrome among adults
Focal Segmental Glomerulosclerosis
Diffuse thickening of the glomerular capillary wall due to accumulation of electron dense, Ig containing deposits along the subepithelial side of the basement membrane
Membranous Nephropathy
Most frequent cause of nephrotic syndrome in children
Minimal change disease
Diffuse loss of foot processes (podocytes) in most glomeruli (“Fusion” of foot processes)
Minimal change disease
Cells of proximal tubules are often laden with lipid and protein which reflect tubular reabsorption of lipoproteins passing through diseased glomeruli
Lipoid Nephrosis
Morphologic variant of FSGS; Retraction and/or collapse of the entire glomerular tuft, with or without additional FSGS lesions
Collapsing Glomerulopathy
Characteristic feature of Collapsing Glomerulopathy
proliferation and hypertrophy of glomerular visceral epithelial cells
Secondary form of FSGS
RENAL ABLATION FSGS
Striking focal cystic dilation of tubule segments
HIV-associated Nephropathy
Presence of large numbers of tubuloreticular inclusions within endothelial cells
HIV-associated Nephropathy
Proliferation is dominant in the mesangium but may involve the capillary loops
MPGN or Mesangiocapillary glomerulonephritis
Type 1 MPGN characteristics:
- Activation of both classical and alternative complement pathways
- Granular pattern
- Antigens are believed to be proteins derived from infectious agents such as hepatitis C and B viruses
Type 2 MPGN characteristics:
- activation of the alternative complement pathway
- Lamina densa of GBM – ribbon-like
- Mesangial rings – characteristic circular aggregates In the mesangium
ISOLATED URINARY ABNORMALITIES
IgA nephropathy (Berger’s disease) Hereditary nephritis Alport syndrome Thin basement membrane lesion (Benign Familial Hematuria) Chronic glomerulonephritis
- Presence of prominent IgA deposits in the mesangial regions
- most common type of glomerulonephritis worldwide
IgA nephropathy (Berger’s disease)
Refers to a group of heterogeneous familial renal diseases associated primarily with glomerular injury
Hereditary nephritis
Hereditary nephritis (2)
- Alport Syndrome
2. Thin basement membrane lesion
Symptoms of Alport syndrome
- Nerve Deafness
- Lens Dislocation
- Posterior cataracts
- Corneal Dystrophy
- Due to abnormal α3 (COL4A3), α4 (COL4A4), or α5 (COL4A5) chain of type IV collagen
- X-linked
- Basket-weave appearance of the GBM
Alport Syndrome
- familial asymptomatic hematuria
- Mutations in genes encoding α3 or α4 chains of type IV collagen
Thin basement membrane lesion
- Considered a pool of end-stage glomerular disease
- Obliteration of glomeruli, transforming them into acellular eosinophilic masses
Chronic GN
GLOMERULAR LESIONS ASSOCIATED WITH SYSTEMIC DISEASES
Lupus Nephritis Henoch-Schönlein Purpura Bacterial Endocarditis–Associated Glomerulonephritis Diabetic Nephropathy Amyloidosis Fibrillary Glomerulonephritis
Affects the arterioles – causing Hyalinizing Arteriolar Sclerosis
Diabetic Nephropathy
Three glomerular syndromes of Diabetic Nephropathy
o Non-nephrotic proteinuria
o Nephrotic syndrome
o Chronic renal failure
Fibrillary deposits in the mesangium and capillary tufts that resembles amyloid but does not stain with congo-red
Fibrillary Glomerulonephritis
Congo red-fibrillary amyloid deposits – present in the mesangium and capillary walls
Amyloidosis
Two major groups of TUBULAR AND INTERSTITIAL DISEASES
- Acute Kidney Injury and Acute Renal Failure
2. Tubulointerstitial Nephritis
Most common cause of acute renal failure
Acute Kidney Injury and Acute Renal Failure
Symptoms of the Maintenance phase of AKI/ATI
a. Sustained decreases in urine output to 40-400 mL/day (oliguria)
b. salt and water overload
c. rising BUN
d. Hyperkalemia
e. Metabolic Acidosis
f. Other manifestations of Uremia
relatively short lengths of tubules are affected, and straight segments of proximal tubules (PST) and ascending limbs of Henle’s loop (HL) are most vulnerable
Ischemic AKI/ATI
extensive necrosis is present along the proximal convoluted tubule segments (PCT) with many toxins (e.g., mercury), but necrosis of the distal tubule, particularly ascending HL, also occurs
Toxic AKI/ATI
Diffuse interstitial, predominantly, mononuclear inflammatory infiltrate; Tubules are separated by inflammation and edema
Tubulointerstitial Nephritis
Renal disorder affecting the tubules, interstitium, and renal pelvis
Pyelonephritis
most common cause of clinical pyelonephritis
Ascending infection
Complications of Acute Pyelonephritis
o Papillary necrosis
o Pyonephrosis
o Perinephric abscess
2 forms of Chronic Pyelonephritis
o Reflux nephropathy
o Chronic obstructive pyelonephritis
Unusual and relatively rare form of chronic pyelonephritis; Accumulation of foamy macrophages intermingled with plasma cells, lymphocytes, polymorphonuclear leukocytes, and occasional giant cells
yellow-orange nodules
XANTHOGRANULOMATOUS PYELONEPHRITIS
Vascular diseases of the Kidneys
Benign Nephrosclerosis Malignant hypertension (Accelerated Nephrosclerosis) Renal Artery Stenosis Thrombotic microangiopathies Atherosclerotic Ischemic Renal Disease Atheroembolic Renal Disease Sickle Cell Disease Nephropathy Diffuse cortical necrosis Renal Infarcts
- Sclerosis of the renal arterioles and small arteries
- Reduction in functional renal mass caused by glomerulosclerosis and chronic tubulointerstitial injury
Benign Nephrosclerosis
Long-standing benign hypertension with eventual injury to the arteriolar walls or the initiating injury may spring de novo from arteritis, a coagulopathy, or some injury causing acute exacerbation of the hypertension
Malignant hypertension (Accelerated Nephrosclerosis)
- Gross: “flea-bitten” appearance
- Onion-skinning because of its concentric appearance (hyperplastic)
Malignant hypertension (Accelerated Nephrosclerosis)
Most common cause of renal artery stenosis (70% of cases) is _____________ at the origin of the renal artery
occlusion by atheromatous plaque
Congenital Anomalies of the Kidneys
o Agenesis of the kidney
o Hypoplasia
o Ectopic Kidneys
o Horseshoe Kidneys
Due to an abnormality in metanephric differentiation
-Persistence in the kidney of abnormal structures—cartilage, undifferentiated mesenchyme, and immature collecting ductules
MULTICYSTIC RENAL DYSPLASIA
term used to describe dilation of the renal pelvis and calyces associated with progressive atrophy of the kidney due to obstruction of the outflow of urine
Hydronephrosis
increased urinary concentration of the stones’ constituents, such that it exceeds their solubility (supersaturation)
UROLITHIASIS (RENAL CALCULI/STONES)
Staghorn calculi
MAGNESIUM AMMONIUM PHOSPHATE
Benign Tumors of the kidneys
Renal papillary adenoma
Renal fibroma or hamartoma (Renomedullary interstitial cell tumor)
Angiomyolipoma
Oncocytoma
Malignant Tumors of the kidneys
Renal cell carcinoma
Urothelial carcinoma of the renal pelvis
Most common Malignant Tumor of the kidneys
Renal cell carcinoma