Ch 12 Pathoma Kidney and Urinary Tract Pathology Flashcards
What is the most common congenital renal anomaly?
Horseshoe kidney
Horseshoe kidney are ___ kidneys usually connected at the upper/lower pole. The kidney is abnormally located in the ___, since it gets caught on the ___ during its ascent/descent from the ___ to the ___.
conjoined; lower; lower abdomen; inferior mesenteric artery root; ascent; pelvis; abdomen
____ is absent kidney formation. May be __ or ___.
renal agenesis; unilateral; bilateral
Unilateral renal agenesis, leads to ___ of the existing kidney. ___ increases risk of renal failure later in life. Bilateral renal agenesis leads to ___, with these 3 developmental defects, aka ___.
hypertrophy; hyperfiltration; oligohydramnios (decrease in amniotic fluid); 1) lung hypoplasia, 2) flat face with low set ears, 3) developmental defects of the extremities; Potter sequence
Name the 3 defects of Potter sequence and what causes it.
1) lung hypoplasia; 2) flat face with low set ears; 3) developmental defects of the extremities; oligohydramnios (decrease in amniotic fluid)
Dysplastic kidney is a inherited/noninherited, ___ malformation of the ___, characterized by __ and ___ (often cartilage). Usually unilateral/bilateral. When bilateral, must be distinguished from ____.
noninherited; congenital; renal parenchyma; cysts; abnormal tissue; unilateral; inherited polycystic kidney disease (PKD)
Polycystic kidney disease is an inherited/noninherited defect leading to unilateral/bilateral enlarged/shrunken kidneys with __ in the renal __ and __. It presents in two forms, ___ and ___ forms.
inherited; bilateral; enlarged; cysts; cortex; medulla; autosomal recessive; autosomal dominant
The autosomal recessive form of PKD presents in ___, as worsening ___ and ___. Newborns may present with ___. It is associated with congenital ___ (leading to ___) and ___.
infants; renal failure; hypertension; Potter sequence; hepatic fibrosis; portal hypertension; hepatic cysts
Name 4 clinical presentations of portal hypertension
1) ascites; 2) congestive splenomegaly; 3) portosystemic shunts (esophageal varices, hemorrhoids, medusa capitae); 4) hepatorenal syndrome
The autosomal dominant form of PKD presents in ___ as ___ (due to increased __), __ and ___. It is due to mutation in the __ or __ gene. Cysts develop at birth/over time. It is associated with these 3 disorders.
young adults; hypertension; renin; hematuria; worsening renal failure; APKD1; APKD2; over time; 1) berry aneurysm; 2) hepatic cysts; 3) mitral valve prolapse (cysts in the kidney, liver, and brain)
Medullary cystic kidney disease is a inherited/noninherited defect leading to cysts in the ___. ___ results in enlarged/shrunken kidney and worsening ___.
inherited (autosomal dominant); medullary collecting ducts; parenchymal fibrosis; shrunken; renal failure
Name 5 congenital kidney diseases.
1) horseshoe kidney; 2) renal agenesis; 3) dysplastic kidney; 4) polycystic kidney disease; 5) medullary cystic kidney disease
Name the 3 congenital renal cystic diseases. Which are inherited? Which results in enlarged kidneys, which shrunken? Which is usually unilateral?
1) dysplastic kidney; 2) PKD; 3) medullary cystic kidney; PKD and MCKD; PKD; MCKD; usually unilateral: dysplastic kidney
Acute renal failure is an acute, severe decrease in ___ (develops within hours/days/weeks). The hallmark is ___ (increase in __ and __), often with ___. Can be divided into these 3 based on etiology
renal function; days; azotemia; BUN; creatinine; oliguria; prenrenal, postrenal, and intrarenal azotemia
Prerenal azotemia is due to ___ to kidneys (eg ___). The increased/decreased blood flow results in increase/decreased GFR, ___, and __. The ___ of fluid and BUN ensues (serum BUN:Cr ratio is greater/less than __). FENa is greater/less than __%, and urine osm is greater/less than ___.
decreased blood flow; cardiac failure; decreased; decreased; azotemia; oliguria; reabsorption; greater; 15; less than 1%; greater than 500mOsm/kg (tubular function remains intact so Na can be absorbed, and urine can be concentrated)
Postrenal azotemia is due to ___ of urinary tract upstream/downstream from kidney (e.g. ___). Decreased inflow/outflow results in inc/dec GFR, __, and ___.
obstruction; downstream; ureters; outflow; dec; azotemia; oliguria
During early stage of obstruction (post-renal azotemia), inc/dec tubular pressure forces BUN in to the blood (serum BUN:Cr ratio is g/l __). Tubular function remains intact, so FENa is g/l __% and urine osm is g/l __.
increased; greater than 15; less than 1%; greater than 500
During long-standing obstruction (post-renal azotemia), ___ damage ensues, resulting in inc/dec reabsorption of BUN (serum BUN:Cr ratio is g/l __). FENa is g/l __ and urine osm is g/l __.
tubular; decreased; less than 15; greater than 2% (decreased reabsorption of sodium); less than 500 (inability to concentrate urine)
What is the most common cause of acute renal failure?
Acute tubular necrosis (intra-renal azotemia)
Acute tubular necrosis is injury and necrosis of ___. __ cells plug __, and the resulting __ decreases ___. ___ are seen in the urine. Dysfunctional ___ results in inc/dec reabsorption of BUN (serum BUN:Cr ratio g/l __), inc/dec reabsorption of Na (FENa g/l __%) and urine osm g/l __.
tubular epithelial cells; necrotic; tubules; obstruction; GFR; brown, granular casts; tubular epithelium; decreased; less than 15; decreased; greater than 2%; less than 500 (inability to concentrate urine)
The etiology of acute tubular necrosis may be __ or __. Clinical features include these 3. ATN is reversible/irreversible, and often requires supportive __ since ___ can be fatal. Oliguria can persist for ___ before recovery, since tubular cells (__ cells) take time to reenter cell cycle and regenerate
1) oliguria with brown, granular casts; 2) elevated BUN and creatinine; 3) hyperkalemia (due to decreased renal excretion) with metabolic acidosis (due to dec excretion of organic acids); reversible; dialysis; electrolyte imbalances; 2-3 wks; stable
Acute tubular necrosis due to ischemia, is due to ___, which results in necrosis of tubules. It is often preceded by ___. These 2 segments of the nephron are particularly susceptible to ischemic damage
decreased blood supply; pre-renal azotemia; proximal tubule; medullary segment of thick ascending limb
Acute tubular necrosis due to nephrotoxicity, is due to ___ resulting in necrosis of tubules. The ___ is particularly susceptible. Name 6 causes and the most common one.
toxic agents; proximal tubule; 1) aminoglycosides (most common); 2) heavy metals (lead); 3) myoglobinuria (crush injury to muscle); 4) ethylene glycol (assoc with oxalate crystals in urine); 5) radio contrast dye; 6) urate (tumor lysis syndrome)
Acute tubular necrosis due to tumor lysis syndrome (chemo causes rapid death of large number of cells and nuclear material is released and turned to uric acid) can be prevented with __ and __ given prior to initiation of chemo.
hydration; allopurinol (purine analog that blocks xanthine oxidase from producing uric acid from xanthine and hypoxanthine - hypoxanthine can be recycled in salvage pathway to make more purines)
Acute interstitial nephritis is a ____ involving the interstitium and __, resulting in acute __ (___ azotemia). Causes include __, __, and __. Resolves with ___. May progress to ___.
drug-induced hypersensitivity; tubules; renal failure; intra-renal; NSAIDs, penicillin, diuretics; cessation of drug; renal papillary necrosis
Acute interstitial nephritis presents as __, __, and __ days to weeks after starting a ___. ___ may be seen in urine.
oliguria; fever; rash; drug; eosinophils
Renal papillary necrosis is necrosis of the ___. It presents with __ and __. Name 4 causes.
renal papillae; gross hematuria; flank pain; 1) chronic analgesic abuse (long term phenacetin or aspirin); 2) diabetes mellitus; 3) sickle cell trait or disease; 4) severe acute pyelonephritis
Nephrotic syndrome are ___ disorders characterized by ___, resulting in these 4 things.
glomerular; proteinuria (greater than 3.5 g/day); 1) hypoalbuminemia (pitting edema); 2) hypogammaglobulinemia (inc risk of infxn); 3) hyper coagulable state (due to loss of antithrombin III); 4) hyperlipidemia and hypercholesterolemia (fatty casts in urine)
Minimal change disease is a type of ___ syndrome. Usually due to ___, may be associated with __. There are normal/abnormal glomeruli seen on H and E stain, and __ may be seen in proximal tubule cells. MCD is the most common cause of nephrotic syndrome in ___.
nephrotic; idiopathic; Hodgkin lymphoma (Reed sternberg cells produce cytokines); normal; lipids; children
In minimal change disease, damage is mediated by __ from ___. There is a excellent/poor response to steroids. What is seen on EM? There are/are not immune complex deposits, which creates a positive/negative immunofluorescence (IF). There is selective ___ (loss of __, but not ___)
cytokines; T cells; excellent; effacement of foot processes (podocytes); not; negative; proteinuria; albumin; immunoglobulin
Focal segmental glomerulosclerosis (FSGS) is the most common cause of ____ syndrome in __ and __. Usually __, and may be associated with __, __, and __. There is focal (some __) and segmental (involving only part of the ___) ___ on HandE stain.
nephrotic; Hispanics; African Americans; idiopathic; HIV, heroin use, sickle cell disease; glomeruli; glomerulus; sclerosis
In focal segmental glomerulosclerosis what is seen on EM? There are/are not immune complex deposits, which creates a positive/negative immunofluorescence (IF). There is a excellent/poor response to steroids. Progresses to ___.
effacement of foot processes; are not; negative; poor; chronic renal failure
Membranous nephropathy is the most common cause of __ syndrome in __. Usually __, may be associated with these 4 things. It has a excellent/poor response to steroids, and progresses to ___.
nephrotic; caucasian adults; idiopathic; 1) hepatitis B or C; 2) solid tumors; 3) SLE (more likely to get nephritic syndrome, but if nephrotic, this is the type); 4) drugs (NSAIDs and penicillamine); poor; chronic renal failure
In membranous nephropathy, there is a thick ___ on H and E due to ___, which appears as a __ IF, and appears as sub epithelial deposits with ‘___’ appearance on EM.
glomerular basmenet membrane; immune complex deposition; granular; spike and dome (podocyte responds to deposit by creating additional BM creating a spike and dome appearance)
Membranoproliferative glomerulonephritis is seen as a thick ____ on H and E, often with a ‘___’ appearance. It is due to ___, and divided into 2 types based on ___. It has a excellent/poor response to steroids, and progresses to ___.
glomerular basement membrane; tram-track; immune complex deposition; location of deposits; poor; chronic renal failure
In type I of membranoproliferative glomerulonephritis, the ___ deposits are located ___. This type is associated with __ and __, and is more/less often associated with tram track appearance.
immune complex; sub endothelial; HBV and HCV; more
In type II of membranoproliferative glomerulonephritis, the ___ deposits are located ___. It is associated with ___, which is an ___ that stabilizes ___, leading to over activation of __, __, and low levels of circulating ___.
immune complex; intramembranous; C3 nephritic factor; autoantibody; C3 convertase; complement; inflammation; C3
Diabetes mellitus can lead to ____ syndrome because a high serum glucose leads to ___ of the vascular basement membrane resulting in ___.
nephrotic; nonenzymatic glycosylation (this leads to leaky vessels allowing protein to leak in leading to:) hyaline arteriolosclerosis
In DM, the glomerular ___ arteriole is more affected (has more hyaline arteriolosclerosis) than the ___ arteriole, leading to high/low glomerular filtration pressure. ___ injury leads to ___. Eventually progresses to ___ syndrome. It is characterized by ___ of the __ with formation of ___ nodules. __ slows progression of hyper filtration induced damage
efferent; afferent; high; hyperfiltration; microalbuminuria; nephrotic; sclerosis; mesangium (cells that hold together the vessels in the glomerulus); Kimmelstiel-WIlson; ACE-inhibitors (they prevent the further constriction of the efferent arteriole normally caused by angiotensin II)
___ is the most commonly involved organ in systemic amyloidosis. ___ deposits in the ___, resulting in __ syndrome. It is characterized by ___ under polarized/non-polarized light after staining with ___.
kidney; amyloid; mesangium; nephrotic; apple-green birefringence; polarized; congo red
Name 6 types of nephrotic syndrome
1) minimal change disease; 2) FSGS; 3) membranous nephropathy; 4) membranoproliferative glomerulonephritis; 5) diabetes mellitus; 6) systemic amyloidosis
Biopsy of nephritic syndrome reveals ___, inflamed ___. ___ deposition activates ___, and __ attracts neutrophils, which mediate damage.
hypercellularity (due to neutrophils attracted by C5a); glomeruli; immune complex; complement; C5a
PSGN is seen as ___, inflamed __ on H and E. It is mediated by ___ (___ IF), and is seen as ___ on EM. Treatment is ___. Children rarely (1%) progress to ___. Some adults (25%) develop ___.
hypercellular; glomeruli; immune complex deposition; granular; subepithelial ‘humps’ (deposits start endothelial than move up through BM and eventually disappear on their own); supportive; renal failure; rapidly progressive glomerulonephritis (RPGN)
Rapidly progressive glomerulonephritis is a ___ syndrome that progresses to ___ in ___ (time). It is characterized by ___ in ___ on H and E stain, which are comprised of __ and ___. Clinical picture and __ help resolve etiology
nephritic; renal failure; weeks to months; crescents; Bowman’s space (of glomeruli); fibrin; macrophages; IF
In Goodpasture syndrome, antibody against __ in __ and __ ___. It presents with these 2 symptoms, classically in ___. What is the IF pattern?
collagen; glomerular and alveolar basement membranes; hematuria and hemoptysis; young, adult males; linear (antibodies line up on the basement membrane)
IgA nephropathy (aka ___ disease), is due to __ in __ of glomeruli. Presents during ___ as episodic gross or microscopic ___ with ___, usually following ___. ___ in the mesangium is seen on IF. May slowly/rapidly progress to ___.
Berger; IgA immune complex deposition; mesangium; childhood; hematuria; RBC casts; mucosal infections (e.g. gastroenteritis); IgA immune complex deposition; slowly; renal failure
Chronic pyelonephirits: In vesicoureteral reflux, scarring at __ and ___ is characteristic. Atrophic tubules containing ___ material resemble __ (‘___’ of the kidney). ___ may be seen in urine.
upper and lower; eosinophilic proteinaceous; thyroid follicles; thyroidization
Ammonium magnesium phosphate kidney stones are most commonly caused by ___ with ___ (name 2). Acidic/alkaline urine leads to formation of stone. Classically results in __ calculi in renal __, which act as a nidus for __. Tx involves ___ and ___.
infection; urease-positive organisms (proteus vulgaris or Klebsiella); alkaline; staghorn; calyces; urinary tract infections; surgical removal (due to size); eradication of pathogen (to prevent recurrence)
Wilms tumor is a malignant/benign kidney tumor comprised of ___, primitive __ and __, and __ cells.
malignant; blastema (immature kidney mesenchyme); glomeruli; tubules; stromal
In the flat pathway of urothelial carcinoma, tumor develops as a high/low grade flat tumor and then __. Associated with ___.
high; invades; early p53 mutations
In the papillary pathway of urothelial carcinoma, tumor develops as a high/low grade papillary tumor that progresses to a high/low grade papillary tumor and then __.
low; high; invades (not associated with early p53 mutations)
