Path 1 Renal Cystic Flashcards Preview

Test 9 > Path 1 Renal Cystic > Flashcards

Flashcards in Path 1 Renal Cystic Deck (18)
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1
Q

Autosomal Dominant Polycystic Kidney Disease

A
  • Bilateral kidneys

- Functioning nephrons compressed by cysts

2
Q

PKD1

A

Polycystin-1 integral membrane protein

  • Localized to tubular epithelial cells of distal nephron
  • 85% of cases
  • Death by 50 years
3
Q

PKD2

A

Polycystin-2 (Ca+ channel)

  • All segments of renal tubules as well as extra-renal tissues
  • Manifests at around 70 years
4
Q

ADPKD progression accelerators

A
  • African American (esp. sickle cell)
  • Male
  • HTN
5
Q

ADPKD most common cause of death

A

Coronary or hypertensive heart disease

6
Q

ADPKD associated pathologies

A

-Berry aneurysms, mitral valve prolapse, polycystic liver disease

7
Q

Autosomal Recessive Polycystic Kidney Disease

A

-PKHD1 gene mutation (fibrocystin)

8
Q

ARPKD most common cause of death

A

Respiratory failure due to failed lung development (oligohydramnios)

9
Q

ARPKD associated pathologies

A

Congenital hepatic fibrosis

  • Portal hypertension
  • Splenomegaly
10
Q

ADPKD v ARPKD gross appearance

A

Bumpy distorted v. enlarged smooth

11
Q

ADPKD v ARPKD histologic appearance

A
  • Large cysts compressing normal tubules

- Radial cysts or dilated collecting tubules

12
Q

Congenital hepatic fibrosis histology

A

Bile duct proliferation

-Portal fibrosis

13
Q

Medullary sponge kidney

A

Cystic dilations of medullary collecting ducts

  • Renal function normal (found in adults)
  • Small kidneys with granular surfaces
14
Q

Medullary cystic disease

A

Cysts at corticomedullary junction

  • Cortical atrophy, interstitial fibrosis, thickened BM, preserved glomeruli
  • Chronic tubulointerstitial nephritis & renal failure
15
Q

Congenital medullary cystic disease

A

Nephronophthisis

16
Q

Multicystic renal dysplasia

A

Abnormality in metanephric differentiation

  • Teratorma
  • Associated with ureteral agenesis or atresia
17
Q

Acquired cystic disease

A

Dialysis-associated

  • Cortical and medullary cysts
  • Cysts contain calcium oxalate crystals
  • Renal cell carcinoma in 7% of patients
18
Q

Simple cysts

A

Clinically insignificant unless hemorrhagic

-Cuboidal epithelium single layer over cysts