Polycystic Kidney Disease

Question 1

What is autosomal recessive polycystic kidney disease

Answer 1

Present in neonates and is usually picked up on antenatal scans

Question 2

Features of PKD

Answer 2

Cystic enlargement of renal collecting ducts, oligohydramnios, pulmonary hypoplasia, Potter syndrome, congenital liver fibrosis

Question 3

Problems associated with PKD

Answer 3

Liver failure due to liver fibrosis, portal HTN leading to oesophageal varices, progressive renal failure, HTN due to renal failure, chronic lung disease

Question 4

Outcome of baby with PKD

Answer 4

Poor prognosis.
Survival depends on extensive interventions in neonatal period thoughtout life. 1/3 die as neonate, 1/3 survive to adulthood

Question 5

What is multicystic dysplastic kidney

Answer 5

One of baby’s kidneys is made up of many cysts while other kidney is normal.

Question 6

How is multicystic dysplastic kidney diagnosied

Answer 6

Antenatal US scans

Question 7

Rare cases of multicystic dysplastic kidney

Answer 7

Can be bilateral which can lead to death in infancy