17: Renal Pathology III - Fang

Question 1

Diseases causing asymptomatic

isolated hematuria

Answer 1

IgA nephropathy(Berger Disease)

Alport syndrome

Thin GBM disease

Question 2

IgA nephropathy s/s and microscope findings

Answer 2

Mild hematuria
Mild proteinuria

LM: Mesangial proliferation

IF: Mesangial IgA (granular)

EM: Mesangial deposits

Question 3

defect of glomerular BM due to mutation in gene encoding for the alpha5 chian of collagen type IV

Answer 3

alport syndrome

s/s
sensorineural deafnes
xlinked dominant inheritance
affects males, females are carriers
microscopic hematuria
progresses to renal failure
proteinuria is sign of progression

Question 4

*** key lesions alport syndrome

Answer 4

GBM thickening, splitting and lamination

• basket-weave pattern

Question 5

LM: normal
IF: negative
EM: thin GBM** (glomerular basement membrane)

Answer 5

thin GBM disease

Benign familial (hereditary) hematuria

About HALF as uniformly thin as it should be

Question 6

ONLY GLOMERULAR DISEASE WITH ALL IMMUNOGLOBULIN DEPOSITION

Answer 6

Granular immune complex deposition of IgG, IgA, IgM, C3, C4

kidney is major target of SLE – lupus nephritis

Question 7

big pale waxy kidney

Answer 7

amyloidosis - amyloid gets trapped in glomeruli, blood vessels, and tubules

usually the glomerular basement membrane becomes too “leaky” to proteins, and the patient gets the “nephrotic syndrome“

Question 8

*** apple green birefringence

Answer 8

amylodosis

*when stained with congo red and seen under polarized light

Question 9

describe acute tubular necrosis

Answer 9

• • Destruction of renal TUBULAR epithelium
• • Loss of renal function
• • 50% of ACUTE renal failure

two types: ischemic and nephrotoxic

ex: aminoglycosides: ampB, gentamycin; mercury, carbon tetrachloride (dry cleaners) and rhabdomyloysis

can be associated with earthquakes

Question 10

pathologic changes with acute tubular necrosis

Answer 10

• dilated tubules with flattened epithelium

Question 11

describe acute intersitial nephritis

Answer 11

drug induced hypersensitivity rxn (note eosinophils)

• • Synthetic Penicillins
• • Rifampin
• • Ibuprofen
• • Thiazide diuretics

2 weeks later: Fever, eosinophilia, rash, and an acute renal failure (Oligouria+ increased Cr)

Question 12

describe pylonephritis

Answer 12

Pathogen: E.coli

• ASCENDING, by FAR the most common, i.e. reflux, obstruction
• HEMATOGENOUS, aka descending too (not caused by e. coli in this case – staph assoc., abscesses )
• ACUTE PYELONEPHRITIS: neutrophils*
• CHRONIC PYELONEPHRITIS: lymphocytes, scars

diagnosis - pus WBX casts, urine culture, pt must have a fever and pain in costo-vertebral angle

Question 13

acute pyelonephritis key lesions

Answer 13

• inflammatory infiltrates icnlude numerous neutrophils

- neurtrophilic infiltrates and neutrophil casts in tubules are characteristic

Question 14

pitting geographic scars and “thyroidization” ***

Answer 14

hallmarks of chroinic pyelonephritis

Question 15

describe papillary adenoma

Answer 15

• always within cortex
• small and benign

pale yellow, well-circumscribed nodules

Question 16

describe angiomyolipoma

Answer 16

• benign
• association with tuberous sclerosis (Mr, multisystem hamartomas)
• thick walled vessels, smooth muscle, and fat

Question 17

risk factors renal cell carcinoma

Answer 17

• tobacco

- chronic renal failure and acquired cystic renal disease

Question 18

renal cell carcinoma s/s

Answer 18

painless hematuria (50-60%)
Abdominal mass (25-40%)
Flank pain (35-40%)
Hypertension (20-40%)
Weight loss (30-35%)
Fever (5-15%)

Question 19

*** renal cel carcinoma strongly tend to invade _______ early

Answer 19

renal vein

*metastasizes early!

Question 20

renal cell carcinoma histotypes (3 most common)

Answer 20

– Clear cell RCC (80%)– Loss of tumor suppressor gene VHL. yellow-orange grossly.

–Papillary (chromophil) RCC (15%) – activation of pro-oncongene gene MET

– Chromophobe RCC (5%)-Loss of multiple chromosomes resulting in hypodiploidy. **Prognosis is the best among 3 histotypes

Question 21

clear cell RCC v. papilalry RCC on LM

Answer 21

Clear Cell RCC
abundant clear cytoplasm; abundant capillaries

Papillary RCC
eosinophilic (pink) cytoplasm; sparse capillaries

Question 22

Approximately _______ of patients on chronic dialysis develop acquired cystic disease in their native kidneys

Answer 22

35%

Renal cell carcinoma develops in approximately 6% of these patients

Question 23

von Hippel-Lindau syndrome (VHL syndrome)

Answer 23

Cavernous hemangiomas in cerebellum, brain stem & eye

**Remarkably increased incidence of RCC

Question 24

Urothelial (transitional cell) carcinoma of renal pelvis

Answer 24

In renal pelvis

1/10 as common as renal cell carcinomas

EXACTLY the same appearance as lower urinary tract carcinomas

MUCH more likely to obstruct the kidney than renal cell carcinomas

Associated with ureter and bladder carcinomas