Polycystic Kidney Dz Flashcards
1
Q
Autosomal Recessive PCKD
A
- cysts originate from renal collecting ducts
- rare 1:20,000
- most common inherited renal cystic dz in infancy and childhood
2
Q
Autosomal Dominant PCKD
A
- cysts can arise anywhere in nephron
- causes progressive kidney enlargement
- mutations in either of 2 genes lead to disease
3
Q
Clinical Features of ADPKD
A
- most are asymptomatic
- flank, back, and/or abdominal pain in 60%
- UTI and renal stones
- hematuria, HTN in 50%
- intracerebral aneurysm 16%
4
Q
Dx of ADPKD
A
- US is most used and most sensitive
- CT and MRI not routinely used
5
Q
What is the Ravine US Criteria for Dx?
A
- do not need very many cysts to cause concern
- depends on family hx
6
Q
Tx of PCKD
A
- no cure
- BP control
- slow progression of renal failure
7
Q
What extra-renal manifestations might occur?
A
- hepatic cysts are most common
- females have hepatomegaly if exposed to extra estrogen
