PKD

Question 1

What is PKD?

Answer 1

This is a heterogenous group of disorders characterised by renal cysts and numerous systemic and extra renal manifestations.

Question 2

How many types of PKD are there?

Answer 2

Two types- ADPKD and ARPKD.

There are 2 genes in ADPKD- PKD1 (85%) and ADPKD2 (15%)

Question 3

Pathophysiology of PKD

Answer 3

Renal cysts develop and grow over time, leading to compression of the normal renal architecture and infrarenal vasculature, increased renal size, interstitial fibrosis and tubular atrophy and progressive renal impairment.

Cysts develop from cells in the tubular portion of the nephron and collecting ducts.

Question 4

Signs and symptoms of PKD

Answer 4

Renal cysts 
HTN 
Abdominal and flank pain 
Haematuria 
Palpable kidneys/abdominal mass 
Headaches 
Dysuria, urgency, suprapubic pain and fever.

Question 5

What are the risk factors for PKD?

Answer 5

FHx of ADPKD

FHx of cerebrovascular event

Question 6

Investigations for PKD

Answer 6

Renal ultrasound (cysts may be found) 
CT scan of abdomen and pelvis 
Urinalysis and culture 
U&Es 
Fasting lipid profile 
ECG 
CT scan of brain 
Genetic testing

Question 7

Differentials of PKD

Answer 7

Acquired cystic kidney disease 
Simple cyst 
Tuberous sclerosis complex 
Von Hippel-Lindau syndrome
Medullary cystic kidney disease

Question 8

Management of PKD

Answer 8

ACEi for HTN
Antibiotics for UTIs such as ciprofloxacin
Analgesics for nephrolithiasis
Avoidance of oestrogen And Limit caffeine intake for polycystic liver disease
RRT for end stage renal disease
Nephrectomy for severe pain and poor function