Short answer · Medically reviewed summary · Last updated: 2023-07-13
Caroli Disease is a rare congenital disorder characterized by the presence of cystic dilatation in the intrahepatic bile ducts. It is considered a form of autosomal recessive polycystic kidney disease (ARPKD) and can affect both the liver and kidneys.
What is the prevalence of Caroli Disease?
Prevalence of Caroli Disease: how many people are affected worldwide, differences by sex and region, with sources.
Caroli Disease is a rare congenital disorder characterized by the presence of cystic dilatation in the intrahepatic bile ducts. It is considered a form of autosomal recessive polycystic kidney disease (ARPKD) and can affect both the liver and kidneys. The exact prevalence of Caroli Disease is not well-established due to its rarity and the lack of comprehensive population studies.
However, it is estimated that the prevalence of Caroli Disease ranges from 1 in 100,000 to 1 in 1,000,000 individuals worldwide. The condition is more commonly observed in certain populations, such as those with a higher rate of consanguineous marriages. Additionally, Caroli Disease can present at any age, from infancy to adulthood.
Caroli Disease can lead to various complications, including recurrent cholangitis, liver abscesses, biliary stones, and liver fibrosis. In some cases, it may progress to liver failure or require liver transplantation. Early diagnosis and appropriate management are crucial in improving outcomes for individuals with Caroli Disease.
