autosomal recessive polycystic kidney disease associated with…
–> 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
autosomal dominant polycystic kidney disease associated with…
berry aneurysm, hepatic cysts, mitral valve prolapse
cysts in the renal cortex and medulla
autosomal dominant polycystic kidney disease mutations
NON-INHERITED. malformation of renal parenchyma with cysts and abnormal tissue.
medullary cystic kidney disease
cysts in the medullary collecting duct.
shrunken kidneys due to fibrosis.
causes of acute tubular necrosis
aminoglycosides heavy metals myoglobinuria ethylene glycol radiocontrast dye urate (from tumor lysis syndrome)
ATN clinical features
- muddy brown casts
- elevated BUN creatinine
- metabolic acidosis (decreased excretion of organic acids).
Acute Interstitial Nephritis causes
drug induced hypersensitivity reaction
Acute Interstitial Nephritis clinical findings
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.
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
minimal change disease
- usually idiopathic, but can be associated with hodgkins lymphoma.
- most common cause of nephrotic disease in children
Focal Segmental Glomerulosclerosis
most common cause of nephrotic syndrome in patients with HIV, IVDA, and sickle cell disease
–> will also see foot process effacement in this disorder.
minimal change disease treatment
steroids, excellent response
Focal Segmental Glomerulosclerosis treatment
steroids, poor response. patients often progress to chronic renal failure.
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.
Membranous nephropahty pathophysiology
thickening of basement membrane due to immune complex deposition just beneath the podocyte. can detect with IF, will appear granular.
hamartoma composed of blood vessel, smooth muscle, and adipose
–> increased frequency in patients with tuberous sclerosis