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
How many cysts are needed for disease confirmation?
- 15 to 39 years of age - at least 3 (unilateral or bilateral) renal cysts
- 40 to 59 years of age - at least 2 cysts in each kidney
- > 60 years of age - at least 4 cysts in each kidney
What is the conservative management of PKD?
Healthy (reno-protective) lifestyle choices
What is the medical management of PCKD if radpidly progressing?
tolvaptan
What is the medical management of HTN in PCKD?
ARB or ACEi
What is the interventional management of PCKD?
- Cyst aspiration (under CT guidance)
- Unilateral/ bilateral nephrectomy
- Renal transplant