This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Pediatrics
All of the following are true about childhood polycystic kidney disease, except - 
Select and explain. 
A. Autosomal dominant, B. Pulmonary hypoplasia, C. Renal cyst present at bih, D. Hepatic fibrosis
A: Autosomal dominant
Answer: A: Autosomal dominant 
Explanation: Ans. is 'a' i.e., Autosomal dominant Childhood polvcystic kidney disease o Childhood polycystic kidney disease has autosomal recessive inheritance, therefore it is also known as autosomal recessive polycystic kidney disease. o Defective gene is the PKHD1 (Polycystic Kidney and Hepatic Diseasel) which codes for a protein fibrocystin. Associations o Maternal oligohydramnios o Potter's syndrome o Pulmonary hypoplasia Cogenital hepatic fibrosis o Hepatic cysts o Biliary ductal atresia Clinical features Majority of patients present during first year of life (during infancy). However, sometimes patients may present later in life (in young adults). Presentation is variable : ? 1. Renal Enlarged cystic kidney at bih. Hypeension, Renal failure, Proteinuria. 2. Respiratory Respiratory distress due to pulmonary hypoplasia. 3. Hepatic Hepatomegaly 4. Poal hypeension Esophageal varices, hypersplenism.