XIV - The Kidneys and Its Collecting System Flashcards
Mastery
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A glomerular syndrome dominated by the acute onset of usually grossly visible hematuria, mildly moderate proteinuria, azotemia, edema and hypertension.
Acute nephritic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 542
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A glomerular syndrome characterized by heavy proteinuria, hypoalbunemia, severe edema, hyperlipidemia and lipiduria.
Nephrotic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 542
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Characterized by bacteriuria and pyuria, which may be asymptomatic.
Urinary tract infection(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 542
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Most common type of collagen found in glomerular basement membrane.
Type IV(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 543
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Most frequent cause nephrotic syndrome in children. Under light microscope, the glomerular basement membrane appears normal, but on electrin microscopy, GBM shows uniform and diffuse effacement of foot processes of the podocytes. Good response to corticosteroid therapy.
Minimal change disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 550
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A lesion characterized histologically by sclerosis affecting some but not all glomeruli and involving only segments of each affected glomerulus.
Focal segmental glomerulosclerosis (FSGS)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 550
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Slowly progressive disease characterized by the presence of subepithelial immunoglobulin-containing deposits along the glomerular basement membrane. Histologically, there is diffuse thickening of the BM. (+) spike and dome pattern on electron microscopy. Often resistant to steroid therapy.
Membranous glomerulonephritis (Membranous Nephropathy)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 551
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Characterized by discrete SUBENDOTHELIAL electron-dense deposits in irregular granular pattern.
Type I MPGN(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 553
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GBM is thickened, showing a “tram-track” appearance on PAS stains. There is proliferation of mesangial and endothelial cells as well as infiltrating leukocytes.
MPGN(Membranoproliferative Glomerulonephritis)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 553
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MPGN wherein the lamina densa and the SUBENDOTHELIAL space of the GBM are transformed into an irregular, ribbon-like extremely electron dense structure.
Type II MPGN(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 554
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Caused by glomerular deposition of immune complexes resulting in diffuse proliferation and swelling of resident glomerular cells. There is uniformly increased cellularity of glomerular tufts. Electron microscopy show subepithelial “humps” against the GBM, with granular deposits of IgG and complement.
Acute postinfectious (poststreptococcal) Glomerulonephritis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 555
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Most common cause of recurrent microscopic or gross hematuria and is the most common glomerular disease revealed by renal biopsies.
IgA Nephropathy (Berger disease)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 555
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Pathologic hallmark of this disease is the deposition of IgA in the mesangium.
IgA nephropathy (Berger Disease)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 556
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Nephritis accompanied by nerve deafness, lens dislocation, posterior cataracts and corneal dystrophy.
Alport syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 556
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Caused by mutations in genes encoding GBM collagen, manifest as hematuria and slowly progressing proteinuria and declining renal function. GBM has “basket-weave” appearance due to splitting and lamination of the lamina densa.
Hereditay nephritis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 557
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A clinical syndrome characterized by rapid and progressive lossof renal function with features of nephritic syndrome,often with severe oliguria. Histologic feature is the presence of crescents.
Rapidly Progressive Glomerulonephritis (Crescentic GN)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 557
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CrGN characterized by linear deposits of IgG and C3 on the GBM. anti-GBM Ab also bind to pulmonary alveolar capillary basement membranes to produce pulmonary hemorrhages associated with renal failure.
Anti-Glomerular Basement Membrance Antibody (Type I) CrGN(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 557
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CrGN which are immune complex-mediated disorders, as a complication of any of the IC nephritides including PSGN, IgA Nephropathy and HSP. There is segmental necrosis and characteristic granular pattern of the underlying immune complex disease.
Immune Complex-Mediated (Type II) CrGN(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 558
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CrGN defined by the lack of anti-GBM Ab and immune complex deposition. Glomeruli show segmental necrosis, negative immunofluorescence.
Pauci-immune (Type III) CrGN(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 558
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An important cause of end-stage renal disease presenting as chronic renal failure. Kidneys are symmetrically contracted, surfaces are red-brown and diffusely granular. Glomeruli are obliterated with marked interstitial fibrosis.
Chronic glomerulonephritis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 559
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A common suppurative inflammation of the kidney and renal pelvis caused by bacterial infection, either by hematogenous spread or through ascending infection.
Pyelonephritis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 560
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Hallmark of this disease is scarring involving the pelvis or calyces, or both, leading to papillary blunting and marked calyceal deformities.
Chronic pyelonephritis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 562
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Nephritis with prominent eosinophilic and mononuclear inflitrate, with pronounced edema.
Acute Drug-Induced Interstitial Nephritis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 563
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A clinicopatholigic entity characterized by damaged tubular epithelial cells and clinically by acute suppression of renal function.Most common cause of acute renal failure.
Acute tubular necrosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 564
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Characterized by necrosis of short segments of the tubules, seen in the straight portions of the PCT and TALOH. There is a variety of tubular injuries, associated with proteinaceous casts in the distal tubules and collecting ducts.
Ischemic ATN(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 566
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ATN characterized by necrosis and injury of the PCT with sparing of the tubular membranes.
Toxic ATN(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 566
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Phase of ATN lasting about 36 hrs, dominated by the inciting medical, surgical or obstetric event. Slight decline in renal output and a rise in serum creatinine.
Initiation phase(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 566
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Phase of ATN where urine output falls markedly, between 50-400mL/day. Dominated by signs and symptoms of uremia and fluid overload.
Maintenance phase(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 566
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Phase of ATN characterized by a steady increase in urine volume, electrolyte imbalance and increased vulnerability to infection.
Recovery(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 566
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Chronic interstitial nephritis associated with renal papillary necrosis. The necrotic papillae appear yellowish-brown, showing coagulative necrosis associated with loss of cellular detail with preservation of tubular outlines.
Analgesic nephropathy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 564
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T-cell mediated immune reaction of the kidneys to an offending agent, characterized by interstitial inflammation, with abundant eosinophils and edema.
Drug-induced interstitial nephritis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 564
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Renal changes in benign hypertension, associated with hyaline arteriolosclerosis, appearing as homogenous, pink hyaline thickening of arterial walls. Larger blood vessels show fibroelastc hyperplasia.
Benign nephrosclerosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 566
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Acute renal injury assiciated with malignant hypertension. The kidneys show small, pinpoint petechial hemorrhages, “flea-bitten” appearance. Concentric arrangement of cells, described as “onion-skin” lesions cause marked narrowing of arterioles and small arteries.
Malignant nephrosclerosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 567
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Characterized by widespread thrombosis and presence of fibrin thrombi in glomeruli and small vessels resulting in acute renal failure. Consequence of childhood HUS and TTP.
Thrombotic microangiopathies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 568
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One of the main causes of acute renal failure in children.
Hemolytic Uremic Syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 568
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Innocuous lesions of the kidney, 1-5cm in diameter, translucent, lined by a gray, glistening, smooth membrane, filled with clear fluid. Composed of a single layer of cuboidal or flattened cuboidal epithelium, usually confined to the cortex.
Simple renal cyst(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 569
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Autosomal dominant. Kidneys are enlarged, composed solely of cysts without intervening parenchyma. Cysts are filled with clear or turbid fluid. Cysts may arise at any level of the nephron, with variable, often atrophic lining.
Adult polycystic kidney disease (APKD)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 569
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What is the pathology behind APKD?
Defective gene PKD1,which codes for polycystin-1.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 569
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Function of policystin-1?
Protein that is involved in cell-cell or cell-matrix adhesion.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 569
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Autosomal recessive. There are numerous small cysts in the cortex and medulla, giving the kidney a “sponge-like” appearance. Cysts have uniform cuboidal epithelium. Associated with multiple cysts in the liver.
Childhood Polycystic Kidney Disease (CPKD)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 570
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An under-recognized vause of chronic kidney disease in children and young adults, associated with mutations in several genes that encode neohrocystins that may be involved in ciliary function. Kidneys are contracted and contain multiple cysts.
Medullary cystic disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 571
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Calculus formation at any level of the urinary collecting system.
Urolithiasis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 571
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Most common composition of kidney stones. Precipitates in the presence of alkaline urine.
Calcium oxalate and/or calcium phosphate(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 571
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Most important cause of kidney stone formation.
Supersaturation(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Kidney stones occuring in patients with alkaline urine due to UTI, particularly Proteus vulgaris and Staphylococci.
Struvite stones(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Component of struvite stones.
Magnesium ammonium phosphate(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Kidney stones seen in patients with gout and leukemias. Urine pH is decreased.
Uric acid stones(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Kidney stones associated with a defect in the renal transportation of certain amino acids. Forms in acidic urine.
Cystine stones(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Branching structures which create a cast of the renal pelvis and calyceal system.
Stagnorn calculi(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Most common composition of staghorn calculi.
Magnesium ammonium phosphate (Struvite)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Dilation of the renal pelvis and calyces, accompanied by atrophy of the renal parenchyma, caused by obstruction to urine outflow.
Hydronephrosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 572
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Dilation of the ureters secondary to obstruction.
Hydroureter(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 573
