Flashcards in Path 1 Renal Cystic Deck (18):
1
Autosomal Dominant Polycystic Kidney Disease
-Bilateral kidneys
-Functioning nephrons compressed by cysts
2
PKD1
Polycystin-1 integral membrane protein
-Localized to tubular epithelial cells of distal nephron
-85% of cases
-Death by 50 years
3
PKD2
Polycystin-2 (Ca+ channel)
-All segments of renal tubules as well as extra-renal tissues
-Manifests at around 70 years
4
ADPKD progression accelerators
-African American (esp. sickle cell)
-Male
-HTN
5
ADPKD most common cause of death
Coronary or hypertensive heart disease
6
ADPKD associated pathologies
-Berry aneurysms, mitral valve prolapse, polycystic liver disease
7
Autosomal Recessive Polycystic Kidney Disease
-PKHD1 gene mutation (fibrocystin)
8
ARPKD most common cause of death
Respiratory failure due to failed lung development (oligohydramnios)
9
ARPKD associated pathologies
Congenital hepatic fibrosis
-Portal hypertension
-Splenomegaly
10
ADPKD v ARPKD gross appearance
Bumpy distorted v. enlarged smooth
11
ADPKD v ARPKD histologic appearance
-Large cysts compressing normal tubules
-Radial cysts or dilated collecting tubules
12
Congenital hepatic fibrosis histology
Bile duct proliferation
-Portal fibrosis
13
Medullary sponge kidney
Cystic dilations of medullary collecting ducts
-Renal function normal (found in adults)
-Small kidneys with granular surfaces
14
Medullary cystic disease
Cysts at corticomedullary junction
-Cortical atrophy, interstitial fibrosis, thickened BM, preserved glomeruli
-Chronic tubulointerstitial nephritis & renal failure
15
Congenital medullary cystic disease
Nephronophthisis
16
Multicystic renal dysplasia
Abnormality in metanephric differentiation
-Teratorma
-Associated with ureteral agenesis or atresia
17
Acquired cystic disease
Dialysis-associated
-Cortical and medullary cysts
-Cysts contain calcium oxalate crystals
-Renal cell carcinoma in 7% of patients
18