Path 1 Renal Cystic Flashcards Preview

Test 9 > Path 1 Renal Cystic > Flashcards

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

Simple cysts

Clinically insignificant unless hemorrhagic
-Cuboidal epithelium single layer over cysts