RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164088185 T1 Wilson Disease T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164088185 RD 2024/10/11 AB It is an inherited disease of copper metabolism dysfunction that is characterized by cirrhosis and central nervous system (CNS) findings. The neurological clinical features include tremor, dysarthria, rigid dystonia, seizures, and psychiatric disorders. Other clinical features include acute liver failure, chronic hepatitis, cirrhosis, renal tubular acidosis, renal failure, and cardiomyopathy. It is fatal if not recognized and treated promptly. The pathognomonic sign is the presence of a Kayser-Fleischer ring, a brownish-yellow ring surrounding the corneo-scleral junction (limbus). This ring is visible especially when neurological symptoms are present. Medications used include chelating agents such as trientine and D-penicillamine, which may present significant implications with anesthesia. Zinc sulphate supplements are also frequently used. Complications include liver failure and/or cancer, as well as kidney problems.