Studies on Copper Metabolism. XXIII. Portal (Laennec's) Cirrhosis of the Liver1

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Abstract
Alterations in copper metabolism were studied in a group of patients with Laennec''s cirrhosis of the liver. The mean value ([plus or minus] standard deviation) for the total serum copper was 141 [plus or minus] 38.6 [mu]g/100 ml; for ceruloplasmin, 39 [plus or minus] 10.3 mg/100 ml; for "non-ceruloplasmin" serum copper, 18 [plus or minus] 14.3 [mu]g/100 ml; for spinal fluid copper, 15 [plus or minus] 11.2 [mu]g/100 ml; for urine copper, 45 [plus or minus] 38.2 [mu]g/24 hours. Values in normal subjects were: total serum copper, 108 [plus or minus] 9 [mu]g/100 ml; ceruloplasmin, 34 [plus or minus] 4 mg/100 ml; "non-ceruloplasmin" serum copper, 2.5 [plus or minus] 3.7 [mu]g/100 ml; spinal fluid copper, 6.2 [plus or minus] 2.4 [mu]g/100 ml; urine copper, 10.5 [plus or minus] 6.6 [mu]g/24 hours. Following the administration of BAL (2.3-dimercaptopropanol) the patients excreted an average of 87 [mu]g of Cu in the urine daily; normal subjects excreted 40 [mu]g. Thioctic acid had no influence on the amount of Cu excreted by either group. The mean value for the total amount of Cu in the liver of 12 patients with cirrhosis was 19 mg as compared with 8.5 mg in five "normal" subjects. In 6 of the patients there was a normal amount of Cu in the liver. In 3 patients there was an increased amount of Cu in this organ. The amount of Cu in the heart, kidneys, muscle and spleen was not increased significantly from the normal. The mean value for the total amount of Cu in the brain of 9 patients with cirrhosis was 6.5 mg as compared with 6.0 mg in five "normal" subjects. It seems unlikely that the alterations in Cu metabolism are of any significance in the pathogenesis of the neuropsychiatric manifestations of Laennec''s cirrhosis of the liver. It is suggested that in patients with cirrhosis and an associated cholangiolitis the excretion of Cu into the bile is impaired and this results in retention of the element in the liver.