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.