Polycystic Kidney Disease Flashcards
What is Polycystic Kidney Disease (PKD)?
- Congenital
- Hundreds of fluid-filled sacks
- Progressive enlargement & loss of function
What do the cysts do?
compress neighbouring structures
What does compression of the blood vessels lead to?
Hypoperfusion leads to Activation of renin-angiotensin system leads to HTN
What does compression of the collecting ducts lead to?
urine stasis leads to kidney stones
What are two types of PKD?
- austosomal dominant
- autosomal recessive
What is autosomal dominant PCKD?
- aka. Adult PKD
- PKD1 or PKD2 gene mutations
- FH needed for diagnosis
What is AD PCKD associated with?
- Cerebral artery berry aneurysms & SAH⚠️
- Mitral valve prolapse
- Benign hepatic cysts
- Heart failure
What is autosomal recessive PCKD?
- aka. Child PKD
2. PKHD1 gene
What is autosomal recessive PKD associated with?
- Oligohydraminos
- Congenital hepatic fibrosis leads to Portal HTN
- Ascending cholangitis
What types of PCKD is more common?
autosomal dominant
What is the presentation of PCKD?
- Hypertension
- Flank pain
- Haematuria
- Palpable kidneys
- Headache
- SAH (can be first presentation esp in young)
What bloods are done for PCKD?
U&Es, lipid profile
What bedside tests are done of PCKD?
- Urinalysis
- Urine culture
- BP measurement
What imaging is done for PCKD?
- Renal USS (non-invasive)
- Abdo CT or MRI
- Prenatal renal USS
What should be done with Fhx of ADPKD?
earlier HTN screening
