<prompt> All of the following are true about childhood polycystic kidney disease, exceptAutosomal dominantPulmonary hypoplasiaRenal cyst present at birthHepatic fibrosis  <response>: 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. AssociationsMaternal oligohydramniosPotters syndromePulmonary hypoplasiaCongenital hepatic fibrosisHepatic cystsBiliary ductal atresiaClinical featuresMajority 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. RenalEnlarged cystic kidney at birth.Hypertension, Renal failure, Proteinuria.2. RespiratoryRespiratory distress due to pulmonary hypoplasia.3. HepaticHepatomegalyPortal hypertension Esophageal varices, hypersplenism. 