Which autosomal disorder is characterized by a paucity of bile ducts and cardiac defects?

Alagille syndrome is characterized by a triad of features, one of which is the paucity of bile ducts leading to cholestasis, or impaired bile flow. This condition is also associated with cardiac defects, particularly involving the outflow tract of the heart, such as pulmonic stenosis. Alagille syndrome is caused by mutations in the JAG1 gene and has a notable impact on multiple organ systems, including the liver, heart, eyes, and skeleton. The combination of these symptoms provides a clear clinical picture that helps in diagnosing the disorder.

Cystic fibrosis primarily affects the respiratory and digestive systems, with thick mucus causing blockages and leading to typical complications. Though it can impact the liver, it does not specifically feature the same bile duct abnormalities or cardiac defects seen in Alagille syndrome. Wilson's disease is a disorder of copper metabolism that leads to liver disease and neurological symptoms but not typically to the specific combination of bile duct paucity and cardiac anomalies. Primary sclerosing cholangitis is a liver condition associated with bile duct inflammation and scarring but does not typically present with cardiac defects. Thus, Alagille syndrome stands out as the correct answer due to its distinctive features linking liver and cardiac abnormalities.