Why KF ring is also seen in primary biliary cholangitis and neonatal hepatitis?

Why KF ring is also seen in primary biliary cholangitis and neonatal hepatitis?

The Kayser-Fleischer (KF) ring is a golden-brown discoloration of the cornea that is caused by the deposition of copper in the Descemet membrane of the eye. It is most commonly associated with Wilson’s disease, a rare genetic disorder that causes the accumulation of copper in the liver, brain, and other organs.

However, the KF ring can also be seen in other liver diseases, including primary biliary cholangitis (PBC) and neonatal hepatitis. In PBC, the KF ring is thought to be caused by the accumulation of copper in the liver, which can occur as a secondary effect of the autoimmune destruction of the bile ducts. In neonatal hepatitis, the KF ring is thought to be caused by the accumulation of copper in the liver as a result of a metabolic defect that impairs the ability of the liver to process copper.

Although the KF ring is a hallmark of Wilson’s disease, its presence alone is not sufficient for a diagnosis of Wilson’s disease, and additional diagnostic tests, such as serum copper and ceruloplasmin levels and genetic testing, may be needed to confirm the diagnosis. In cases where the KF ring is present in the absence of other signs of Wilson’s disease, it may indicate the need for further investigation to identify the underlying cause of the copper deposition.