Pathoma - Kidney, Urinary tract pathology

Question 1

autosomal recessive polycystic kidney disease associated with…

Answer 1

–> congenital hepatic fibrosis. consider this diagnosis in an infant with portal hypertension. also can see hepatic cysts.

–> newborns may present with potter sequence.

cysts in renal cortex and medulla

Question 2

autosomal dominant polycystic kidney disease associated with…

Answer 2

berry aneurysm, hepatic cysts, mitral valve prolapse

cysts in the renal cortex and medulla

Question 3

autosomal dominant polycystic kidney disease mutations

Answer 3

APKD1, APKD2

Question 4

dysplastic kidney

Answer 4

NON-INHERITED. malformation of renal parenchyma with cysts and abnormal tissue.

Question 5

medullary cystic kidney disease

Answer 5

cysts in the medullary collecting duct.

shrunken kidneys due to fibrosis.

Question 6

causes of acute tubular necrosis

Answer 6

aminoglycosides 
heavy metals
myoglobinuria 
ethylene glycol
radiocontrast dye 
urate (from tumor lysis syndrome)

Question 7

ATN clinical features

Answer 7

• muddy brown casts
• elevated BUN creatinine
• hyperkalemia
• metabolic acidosis (decreased excretion of organic acids).

Question 8

Acute Interstitial Nephritis causes

Answer 8

drug induced hypersensitivity reaction
NASIDs
Penicillin
Diuretics

Question 9

Acute Interstitial Nephritis clinical findings

Answer 9

oliguria, fever, rash in days to weeks after starting a drug.
–> eosinophils in the urine

• usually resolves with cessation of the drug but can continue on to papillary necrosis.

Question 10

nephrotic syndrome

Answer 10

protein loss greater than 3.5 g per day

• hypoalbuminemia - edema
• hypogammaglobulinemia - increased risk of infection
• hypercoaguable state - loss of antithrombin 3
• hyperlipidemia, hypercholesterolemia - fatty casts in the urine

Question 11

minimal change disease

Answer 11

• usually idiopathic, but can be associated with hodgkins lymphoma.
• most common cause of nephrotic disease in children

Question 12

Focal Segmental Glomerulosclerosis

Answer 12

most common cause of nephrotic syndrome in patients with HIV, IVDA, and sickle cell disease

–> will also see foot process effacement in this disorder.

Question 13

minimal change disease treatment

Answer 13

steroids, excellent response

Question 14

Focal Segmental Glomerulosclerosis treatment

Answer 14

steroids, poor response. patients often progress to chronic renal failure.

Question 15

Membranous nephropahty

Answer 15

most common cause of nephrotic syndrome in caucasian adults. can be associated with Hep B/C, SLE, some drugs.

• note that patients with SLE presenting with nephrotic syndrome will have this.

Question 16

Membranous nephropahty pathophysiology

Answer 16

thickening of basement membrane due to immune complex deposition just beneath the podocyte. can detect with IF, will appear granular.

Question 17

Angiomyolipoma

associated with…

Answer 17

hamartoma composed of blood vessel, smooth muscle, and adipose

–> increased frequency in patients with tuberous sclerosis