Renal pathology III Flashcards
what 3 diseases are associated with asymptomatic isolated hematuria?
- IgA nephropathy (Berger disease)
- alport syndrome
- thin GBM disease
what is the most common type of primary glomerulonephropathy worldwide?
IgA nephropathy
what are the symptoms of IgA nephropathy?
- mild hematuria
- mild proteinuria
what are the LM, IF, and EM findings in IgA nephropathy?
- LM: mesangial proliferation / hypercellularity
- IF: mesangial IgA
- EM: mesangial deposits
what is alport syndrome?
defect of the GBM due to mutation in the gene encoding the alpha-5 chain of type IV collagen
what is the inheritance pattern of alport syndrome?
X-linked dominant (incomplete - women can get it)
what are the symptoms of alport syndrome?
- sensorineural hearing loss
- renal failure / microscopic hematuria
- proteinuria (indicates progression)
what are the key lesions of alport syndrome?
- GBM thickening / splitting / LAMINATION
- “basket-weave” pattern
which disease is associated with a basket weave pattern on histology?
alport syndrome
what are the LM, IF, and EM findings in thin GBM disease?
- LM: normal
- IF: negative
- EM: thin GBM
what are the secondary glomerulonephropathies?
- SLE
- diabetes
- amyloidosis
in SLE there is granular immune complex deposition of what factors?
- IgG
- IgA
- IgM
- C3
- C4
what is the only glomerular disease with all Ig deposition?
SLE
where does amyloid get trapped in amyloidosis?
- glomeruli
- blood vessels
- tubules
what is the pathogenesis of kidney amyloidosis?
- GBM becomes leaky to proteins and the patient gets nephritic syndrome
what stain is used to visualize amyloid? how does it appear?
- Congo Red
- apple green biorefringence
what is the pathogenesis of acute tubular necrosis?
destruction of renal tubular epithelium
what are the two types of acute tubular necrosis?
- ischemic
- nephrotoxic
what drugs are nephrotoxic?
- aminoglycosides
- amphotericin B
- gentamycin
what heavy metal is nephrotoxic?
mercury
what organic solvents are nephrotoxic?
carbon tetrachloride
how is myoglobin nephrotoxic?
causes rhabdomyolysis (in heat stroke)
- dilated tubules with flattened epithelium
- necrotic debris in tubules
diagnosis?
acute tubular necrosis (ATN)
what is the cause of acute interstitial nephritis?
drug induced hypersensitivity
what drugs cause acute interstitial nephritis? what is seen clinically after ingestion?
- synthetic penicillins
- rifampin
- ibuprofen
- thiazide diuretics
2 weeks later:
- fever
- eosinophilia
- rash
- acute renal failure
what is the main pathogen associated with pyelonephritis?
e. coli
what is the cause of acute pyelonephritis?
infections of the pelicalyceal system and renal parenchyma
how does acute pyelonephritis present? how is the diagnosis made?
- presentation: pain in the costo-vertebral angle along with other signs of infection
- diagnosis: pus (WBC) casts, urine culture
polymorphonuclear casts in tubules - diagnosis?
acute pyelonephritis
what are the key lesions in acute pyelonephritis?
- neutrophilic infiltrates in tubules
- neutrophilic casts in tubules
how does acute pyelonephritis differ from acute interstitial nephritis?
in acute pyelonephritis the inflammatory infiltrates include neutrophils
what is the main etiology of chronic pyelonephritis?
obstruction (congenital or acquired)
is chronic pyelonephritis more common in males or females?
females
what are the hallmark features of chronic pyelonephritis?
- pitting geographic scars
- thyroidization
- inflammatory infiltrate
- fibrosis
- pitting geopraphic scars
diagnosis?
chronic pyelonephritis
atrophic tubules that resemble thyroid gland is associated with what renal disease?
chronic pyelonephritis
- pitting geographic scars
- thyroidization
these are hallmark features of what disease?
chronic pyelonephritis
papillary adenomas are always located in which part of the kidney?
cortex
angiomyolipomas are associated with what condition?
tuberous sclerosis
what are the signs and symptoms of tuberous sclerosis?
- mental retardation
- multisystem hamartomas
- angiomyolipomas
angiomyolipomas consist of what 3 structures?
- thick walled vessels
- smooth muscle
- fat
what are the main risk factors for renal cell carcinoma?
- tobacco
- chronic renal failure
- acquired cystic renal disease
what are the primary symptoms of renal cell carcinoma?
- hematuria
- abdominal mass
renal cell carcinoma strongly tend to invade what vessel early in the disease?
renal VEIN
what are the renal cell carcinoma histotypes? which is most common?
- clear cell (most common)
- papillary
- chromophobe
what is the gross appearance of renal cell carcinoma?
- yellow orange
- sharp borders
- gray-white necrosis and foci of hemorrhagic discoloration
what is the histological appearance of renal cell carcinoma?
- rounded or polygonal cells
- clear or granular cytoplasm
- abundant capillaries
- rounded or polygonal cells
- clear or granular cytoplasm
- abundant capillaries
diagnosis?
renal cell carcinoma
what is the gross appearance of chromophil (papillary) renal cell carcinoma?
thick capsule with red / brown reactive changes and hemorrhage
what is the histological appearance of chromophil (papillary) renal cell carcinoma?
- papillary structures, many of which enclose clusters of foamy macrophages
- abundant eosinophilic cytoplasm and mildly atypical nuclei
- sparse capillaries
- papillary structures, many of which enclose clusters of foamy macrophages
- abundant eosinophilic cytoplasm and mildly atypical nuclei
- sparse capillaries
diagnosis?
chromophil (papillary) renal cell carcinoma
von hippel lindau syndrome is characterized by what types of lesions? where?
cavernous hemangiomas in:
- cerebellum
- brain stem
- eye
on hippel lindau carries a very high incidence of what type of cancer?
renal cell carcinoma
what is the histological appearance of urothelial carcinoma of renal pelvis?
papillary growth lined by urothelial cells mild nuclear atypia and pleomorphism
what is the most common renal malignancy of early childhood?
wilms tumor
wilms tumor is caused by what type of genetic defect? which genes? which chromosome?
- loss of function mutation of tumor suppressor genes WT1 or WT2
- chromosome 11
wilms tumor histology
epithelial component surrounded by metanephric blastema and tumor immature spindle cell stroma
epithelial component surrounded by metanephric blastema and tumor immature spindle cell stroma
diagnosis?
wilms tumor
what is the difference between clear cell RCC and papillary RCC?
- clear cell RCC has clear cytoplasm and abundant capillaries
- papillary RCC has eosinophilic cytoplasm and sparse capillaries
