Hepatolenticular degeneration (HLD), also known as Wilson’s disease (WD), is an autosomal recessive disease of copper metabolism, mainly manifested by liver cirrhosis and degeneration of the basal ganglia. Disease causes mild to severe four stages: liver steatosis, hepatitis, liver fibrosis, and cirrhosis. Clinical manifestations are classified into nerve injury, liver injury, and other organ injury. Kayser-Fleischer ring (K-F ring) is one of the important signs and basis for the diagnosis of WD. Serum ceruloplasmin: The decrease of serum ceruloplasmin is one of the important bases for the diagnosis of this disease.

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Hepatolenticular Degeneration

  • Hongjun Li,
  • Ruili Li,
  • Chaohong Fu,
  • Lu Wang,
  • Ying Zhou

摘要

Hepatolenticular degeneration (HLD), also known as Wilson’s disease (WD), is an autosomal recessive disease of copper metabolism, mainly manifested by liver cirrhosis and degeneration of the basal ganglia. Disease causes mild to severe four stages: liver steatosis, hepatitis, liver fibrosis, and cirrhosis. Clinical manifestations are classified into nerve injury, liver injury, and other organ injury. Kayser-Fleischer ring (K-F ring) is one of the important signs and basis for the diagnosis of WD. Serum ceruloplasmin: The decrease of serum ceruloplasmin is one of the important bases for the diagnosis of this disease.