Cystic renal disease Flashcards Preview

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Flashcards in Cystic renal disease Deck (15):
1

Is the presence of a few renal cortical cysts in adults a cause for concern?

No

2

What is the factor that elevates cause for concern with cysts?

Elevated creatinine

3

What features characterize AD (adult) polycystic disease?

Slowly expanding cysts that compress normal tissue causing ischemic atrophy

4

What are the causes of AD polycystic kidney disease?

1.Mutations in Polycystin-1
2.Mutations in Polycystin-2

5

What are the results of Polycystin mutations

1.Ciliopathy
2.Defective mechanosensing of urine flow
3.Dysregulation of cell adhesion

6

At what level of the nephron do cysts form?

Any level

7

What is the theory behind the mutations of PKD

Two hit hypothesis.

8

At what age do symptoms usually start?

30's

9

What are the symptoms?





Flank pain or a dragging sensation in the abdomen.
2.Frequent gross Hematuria
3.Hypertension develops in 75%
4.Cyst formation in the Liver
5.Cerebral vascular aneurysms
-risk for sudden death due to rupture and subarachnoid hemorrhage

10

What is the cause of AR PKD?

Mutations in Fibrocystin

11

What is fibrosystin?

A protein of uncharacterized function in the cilia of tubular epithelial cells

12

What are the consequences of AR PKD?

Immediate untreatable respiratory failure at birth due to pulmonary hypoplasia

13

What is Nephronopthisis?

Medullary cystic disease complex AR cystic renal disease

14

Where are cysts located in Nephronopthisis?

numerous small cysts at the corticomedullary junction

15

What are other associated findings in Nephronopthisis?

Chronic tubulointerstitial nephritis and fibrosis