What are Kayser-Fleischer rings associated with?

Kayser-Fleischer rings are specifically associated with Wilson Disease, a genetic disorder that leads to excessive accumulation of copper in the body due to impaired copper transport and metabolism. These rings are characterized by a greenish-brown deposit of copper in the corneal margin of the eye, which is visible upon examination. The presence of these rings is a significant clinical indicator of Wilson Disease and can aid in its diagnosis, alongside other signs and symptoms related to copper toxicity.

In Wilson Disease, the body's inability to eliminate copper leads to its accumulation in various tissues, including the liver and brain, ultimately causing hepatic, neurologic, and psychiatric disorders. The identification of Kayser-Fleischer rings is important because it not only indicates the diagnosis of Wilson Disease but also provides insight into the severity of copper accumulation.

Other conditions listed, such as cystic fibrosis, hemochromatosis, and Marfan syndrome, do not exhibit Kayser-Fleischer rings as a clinical feature. Cystic fibrosis primarily affects the respiratory and digestive systems, hemochromatosis involves iron overload, and Marfan syndrome is a connective tissue disorder. Thus, these conditions are not related to the pathogenic mechanisms seen in Wilson Disease that manifest with Kayser-Fleischer rings.