STUDIES ON COPPER METABOLISM .16. RADIOACTIVE COPPER STUDIES IN NORMAL SUBJECTS AND IN PATIENTS WITH HEPATOLENTICULAR DEGENERATION
STUDIES ON COPPER METABOLISM .16. RADIOACTIVE COPPER STUDIES IN NORMAL SUBJECTS AND IN PATIENTS WITH HEPATOLENTICULAR DEGENERATION
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DOI：
10.1172/jci103232
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发表时间：
1955-01-01
影响因子：
15.9
通讯作者：
WINTROBE, MM
中科院分区：
文献类型：
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作者：
BUSH, JA;MAHONEY, JP;WINTROBE, MM
将含有1毫居里放射性的1毫克铜口服给予4名正常受试者和4名肝豆状核变性（Wilson's disease）患者。将等量的铜 - 64静脉注射给4名正常受试者、3名肝豆状核变性患者和2名酒精性肝硬化患者。测定了经尿液和粪便排出的给予的铜 - 64的比例，并研究了同位素在血浆白蛋白和球蛋白（铜蓝蛋白）组分中的摄取情况。测定了3名正常受试者、4名肝豆状核变性患者和2名酒精性肝硬化患者在给予50微克铜 - 64后其从血浆中消失的曲线。用体表闪烁计数器测量了2名正常受试者、4名肝豆状核变性患者和2名酒精性肝硬化患者肝脏对铜 - 64的摄取情况。正常受试者口服铜 - 64后，平均有0.1%的给药剂量从尿液中回收，72.4%从粪便中回收；肝豆状核变性患者平均有2.5%从尿液中回收，52%从粪便中回收。给2名肝豆状核变性患者同时给予硫化钾（K₂S）和铜 - 64，导致从粪便中回收的放射性活度增加。正常受试者静脉注射铜 - 64后，平均有0.2%的放射性活度从尿液中回收，12.4%从粪便中回收；肝豆状核变性患者平均有5.4%从尿液中回收，2.5%从粪便中回收。酒精性肝硬化患者平均有0.8%从尿液中回收，8.5%从粪便中回收。肝豆状核变性患者铜蓝蛋白对铜 - 64的摄取受损，但酒精性肝硬化患者未受损。4名肝豆状核变性患者中有3名以及2名酒精性肝硬化患者中有1名肝脏对铜 - 64的摄取降低。肝豆状核变性患者红细胞对铜 - 64的摄取与正常个体的红细胞无差异。这些发现被解释为表明患者组织中铜的过度蓄积主要是由于胃肠道对铜的吸收增加。尽管肝豆状核变性患者静脉注射同位素后从粪便中回收的比例比正常受试者从粪便中回收的比例小，但所提供的证据支持胆汁中铜的排泄可能未受损的观点。给予硫化钾可有效抑制醋酸铜的吸收。
One mg of Cu containing 1 mc of radio-activity was given orally to 4 normal subjects and 4 patients with Wilson''s disease. The same amount of Cu64 was injected intravenously into 4 normal subjects, 3 patients with Wilson''s disease, and 2 patients with alcoholic cirrhosis of the liver. The proportion of the administered Cu64 excreted in the urine and feces was determined and the uptake of the isotope into the albumin and globulin (ceruloplasmin) fractions of plasma was studied. The curve of disappearance from the plasma of 50 [mu]g of Cu64 after the administration was determined in 3 normal subjects, 4 patients with Wilson''s disease, and 2 patients with alcoholic cirrhosis of the liver. Cu64 uptake into the liver was measured with a body-surface scintillation counter in 2 normal subjects, 4 patients with hepatolenticular degeneration, and 2 patients with alcoholic cirrhosis of the liver. Following oral administration of Cu64 to normal subjects, an average of 0.1% of the administered dose was recovered in the urine and 72.4% in the stools; in the patients with Wilson''s disease, an average of 2.5% was recovered in the urine and 52% in the stools. The administration of K2S together with Cu64 to 2 of the patients with hepatolenticular degeneration resulted in an increase in the amount of activity recovered in the stools. Following intravenous administration of Cu64 to normal subjects, an average of 0.2% of the activity was recovered in the urine and 12.4% in the stools; in the patients with Wilson''s disease, an average of 5.4% was recovered in the urine and 2.5% in the stools. In the patients with alcoholic cirrhosis of the liver, an average of 0.8% was recovered in the urine and 8.5% in the stools. The uptake of Cu64 into ceruloplasmin was impaired in the patients with hepatolenticular degeneration, but not in the patients with alcoholic cirrhosis of the liver. The uptake of Cu64 by the liver was depressed in 3 of the 4 patients with hepatolenticular degeneration and in 1 of 2 patients with alcoholic cirrhosis of the liver. The uptake of Cu64 by the erythrocytes of patients with hepatolenticular degeneration was not different from that of the red corpuscles of normal individuals. These findings are interpreted as indicating that excessive accumulation of Cu in the tissues of patients is due primarily to increased absorption of Cu from the gastro-intestinal tract. Although a smaller proportion of the isotope administered intravenously was recovered in the stools of the patients with Wilson''s disease than was recovered in the stools of normal subjects, evidence presented supports the view that the excretion of Cu in the bile is probably not impaired. The absorption of cupric acetate can be effectively inhibited by the administration of K2S.