All of the following are true about childhood polycystic kidney disease, except –
Childhood polycystic kidney disease 

Childhood polycystic kidney disease has autosomal recessive inheritance, therefore it is also known as autosomal recessive polycystic kidney disease.
Defective gene is the PKHD1 (Polycystic Kidney and Hepatic Di8easel) which codes for a protein fibrocystin. Associations
Maternal oligohydramnios
Potter's syndrome
Pulmonary hypoplasia
Congenital hepatic fibrosis
Hepatic cysts
Biliary ductal atresia

Clinical features

Majority of patients present during the first year of life (during infancy). However, sometimes patients may present later in life (in young adults).
The presentation is variable : -

1. Renal

Enlarged cystic kidney at birth.
Hypertension, Renal failure, Proteinuria.

2. Respiratory

Respiratory distress due to pulmonary hypoplasia.

3. Hepatic

Hepatomegaly
Portal hypertension → Esophageal varices, hypersplenism.
Autosomal dominant
Pulmonary hypoplasia
Renal cyst present at birth
Hepatic fibrosis