Renal Diseases Flashcards
What parts of the kidney do cystic diseases affect?
The cilia
Cilia are structures on tubular cells which control flow into the kidneys (get cysts from too much flow)
Who does Autosomal Dominant Polycystic Kidney Disease affect?
Adults
Remember by Dominant = Adult
What gene defects are involved in autosomal dominant polycystic kidney disease?
Defects in polycystic kidney disease (PKD) genes
PKD-1: 85% of cases
PKD-2: 15% of cases
Describe the morphology of autosomal dominant polycystic kidney disease. Also, are other organs affected?
Remember adults have four decades to develop cysts so there are a ton
Gross: irregular surface
So many cysts that normal intervening parenchyma are squashed and are subjected to ischemic atrophy
Again, so many cysts that 1/3 of patients have asymptomatic cysts in liver
Autosomal Dominant polycystic kidney disease
Clinical stuff
Remember, tons of cysts!
Symptom: flank pain (tons of cysts enlarge the kidney causing this)
Disease is typically asymptomatic until fourth decade, then renal failure
Autosomal Recessive Polycystic Kidney Disease
Who does it affect?
Kids
Think recessive = less than dominant so affects kids
autosomal recessive polycystic kidney disease
gene/gene products
PKHD-1 (looks like PKID) which codes for fibrocystin.
Gene defective so expect fibrosis to be a problem in this disease
autosomal recessive polycystic kidney disease
morphology
gross: smooth surface*
cut: many small cysts in cortex/medulla
other organs: liver is affected by congenital hepatic fibrosis (cirrhosis)
*smooth surface cuz cysts develop in the cortex/medulla
autosomal recessive polycystic kidney disease
clinical:
serious from birth, many die in infancy
if survive infancy develop cirrhosis (congenital hepatic fibrosis)
What is Glomerularnephritis
Inflammation of the glomeruli (kidney filtering unit) due to deposition of immune complexes or Ab attack
Symptoms make sense given that the glomeruli are the kidney filter unit
ie patients will have nephritic syndrome (blood and protein in urine and also edema and hypertension)
Acute poststreptococcal Glomerularnephritis
causes
post-infection with the sore throat type of strep (9-14 day latency)
Then caused by deposition immune complexes
acute poststreptococcal glomerularnephritis
morphology
Think about complexes being deposited or “buried”
- deposits are mainly C3 (early may see IgG)
- complexes deposited as isolated humps in subepithelial space between outer GBM and Podocytes (humps like the humps of dirt created when dogs dig a hole)
- appearance is granular (like a backyard that a dog has been digging all over)
acute poststreptococcal glomerularnephritis
clinical
acute so short-lasting
90% of children recover without complications
Crescentic glomerularnephritis (rapid progressive glomerulonephritis)
causes
- Immune system directly attacks the glomerulus. The target is collagen IV
- Lungs also have collagen IV, so often lungs and kidney affected together
crescentic glomerulonephritis (rapid progressive glomerulonephritis)
morphology
- IgG and C3 make up the deposits. Because it targets indiscriminately, crescent is not granular but solid
- Crescents eventually destroy Bowman’s space and lead to scarring, which permanently messes with the glomerulus’ ability to filter
crescentic glomerulonephritis (rapid progressive glomerulonephritis)
clinical
- if untreated, patient will die within weeks/months
- Tx with immunosuppressants or renal transplant
Acute Tubular Necrosis
Causes
Kidneys interact with a lot of blood and whatever is in the blood.
ATN is caused by ischemia and anything toxic (antifreeze, Gentamicin, CCL4)
Acute Tubular Necrosis
morphology
If kidneys don’t get enough oxygen, the cells lining the tubule end up dying and falling off.
-As they die off, they accumulate and form the proteinaceous casts seen in ATN
Urinary Tract Infection
Cause
- Gram negative bacteria
- They infect either by ascending the urinary tract or by getting in hematogenously
urinary tract infection
predisposing conditions
Anything that helps the bacteria ascend
- females (shorter urethra)
- Outflow obstruction (urinary stones, strictures, pregnancy, tumors, BPH)
Urinary tract infection
histopathology
- The bacteria will colonize a specific area of the UT so kidneys will appear normal and the lesions are focal
- leukocyte casts in urine
- small white abscesses on the subcapsular surface made of polys, dead tissue and liquefactive necrosis
(these casts and lesions are the aftermath of the battle between body and bacteria)
urinary tract infection
clinical
Infection: body combats with inflammatory response which causes many of the symptoms (fever, chills, malaise, sweat)
Also area specific symptoms: frequency, painful urination (dysuria), bacteriuria and flank pain
Wilms Tumor
- Wilms = the kid kidney tumor
- if there’s a gene association (90% sporadic) its wilm’s tumor 1 (WT1)
- it presents early (1-3 years) and contains mixed tissue types; components are developmental tissue (normal types of fetal tissue with immature elements)
Renal Cell Carcinoma
who does it affect?
If kids have wilms, men over the age of 50 have renal cell carcinoma
Renal Cell Carcinoma
gene defect
VHL gene: regulates angiogenesis
so will be a blood related symptom
Renal Cell Carcinoma
histopathology
mass is yellow-orange
-colored by blood, hemorrhage and necrosis
Renal Cell Carcinoma
clinical
Triad of presenting features:
- hematuria (blood related symptom)
- flank pain
- palpable mass
Urothelial Carcinomas (transitional cell carcinomas)
pathogenesis:
-smoking (most bad stuff ends up in the bladder to be excreted)
histopathology:
-ranges from low grade (stay in the mucosa) to high grade (larger, solid, invasive)
Most aggressive ones will require radical surgery to remove malignant tissue
