**Core Concept**
Caroli's syndrome is a rare congenital disorder characterized by non-obstructive dilatation of the intrahepatic bile ducts. It is a form of congenital hepatic fibrocystic disease, which also includes other conditions such as autosomal recessive polycystic kidney disease (ARPKD) and autosomal dominant polycystic kidney disease (ADPKD).
**Why the Correct Answer is Right**
The correct inheritance pattern of Caroli's syndrome is autosomal recessive. This means that a person needs to inherit two copies of the mutated gene (one from each parent) to develop the condition. The mutated gene responsible for Caroli's syndrome affects the PKHD1 gene, which encodes the fibrocystin protein. This protein is essential for the proper functioning of the bile ducts and the kidneys.
**Why Each Wrong Option is Incorrect**
* **Option A:** Caroli's syndrome is not inherited in an autosomal dominant pattern, as a single copy of the mutated gene would not be enough to cause the condition.
* **Option B:** While Caroli's syndrome is associated with other congenital anomalies, it is not typically associated with an X-linked inheritance pattern.
* **Option D:** Caroli's syndrome is not a multifactorial condition, as it is primarily caused by a specific genetic mutation rather than a combination of genetic and environmental factors.
**Clinical Pearl / High-Yield Fact**
Caroli's syndrome often presents with recurrent cholangitis, abdominal pain, and liver abscesses. It is essential to consider this condition in patients with these symptoms, especially if they have a family history of similar conditions.
**Correct Answer: C. Autosomal recessive.**
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