Absorption of Copper in Homozygotes and Heterozygotes for Wilson's Disease and Controls: Isotope Tracer Studies with 67Cu and 64Cu

1972 ◽  
Vol 43 (5) ◽  
pp. 617-625 ◽  
Author(s):  
G. T. Strickland ◽  
W. M. Beckner ◽  
Mei-Ling Leu

1. Absorption of copper was determined by the simultaneous administration of 64Cu orally and 67Cu intravenously to six patients with Wilson's disease (WD), eighteen of their parents and siblings, four normal subjects and three subjects with cirrhosis of the liver. Absorption was calculated by three methods: (1) the mean ratio of 64Cu to 67Cu body retention at 3 and 4 days as determined by whole-body counting; (2) the mean ratio of 64Cu to 67Cu at 3 and 4 days as determined by faecal excretion; and (3) the mean ratio of 64Cu to 67Cu plasma radioactivity 6–24 h after administration. 2. The total-body counting and faecal methods for determining copper absorption agreed with each other, demonstrating that the normal absorption of copper is 40–70% (mean 56%) of the dose and that absorption is not influenced by cirrhosis of the liver, age or sex; but it appears to be inversely related to the amount of carrier copper. The absorption of copper in both homozygotes and heterozygotes for WD did not differ significantly from that of the control subjects. Therefore, the increased body burden of copper in WD does not appear to be due to over absorption, but rather to decreased biliary excretion of copper.

1972 ◽  
Vol 43 (5) ◽  
pp. 605-615 ◽  
Author(s):  
G. T. Strickland ◽  
W. M. Beckner ◽  
Mei-Ling Leu ◽  
S. O'Reilly

1. Studies with 67Cu were carried out on thirteen patients with Wilson's disease (WD), twenty-nine of their parents and siblings, thirteen normal subjects and seven subjects with cirrhosis of the liver. Control subjects and five siblings of patients with WD generally excreted more than 15% of the dose of 67Cu in their 5-day stool collections, had normal liver/thigh radioactivity ratio patterns, and had whole-body biological 67Cu half-times of less than 40 days. The remaining twenty-four family members, including all twelve parents, and the patients with WD generally excreted less than 15% of the dose of 67Cu in their 5-day stool collections, had abnormal liver/thigh radioactivity ratio patterns, and had whole-body biological 67Cu half-times greater than 40 days. 2. The data demonstrate a normal 67Cu biological half-time of 28 days and faecal excretion of 22% in 5 days, and suggest a decreased biliary excretion of copper in both homozygous and heterozygous individuals with WD.


1973 ◽  
Vol 29 (2) ◽  
pp. 269-278 ◽  
Author(s):  
Ann Ashworth ◽  
P. F. Milner ◽  
J. C. Waterlow ◽  
R. B. Walker

1. The absorption of iron from59Fe-labelled maize and soya-bean preparations was measured by whole-body counting in forty-two apparently healthy Jamaican infants and compared with the absorption of ferrous ascorbate.2. The mean absorption of Fe from maize was 4·3% and from soya beans baked at 300°, 9·4%, compared with 28·5% for ferrous ascorbate. In a group of children given boiled soya beans the mean absorption of Fe was 2·8%, and of ferrous ascorbate 16·7%.3. There was much variability between replicate tests made on the same child at intervals of 1–2 weeks.4. The absorption of food Fe was not increased in children who were considered to be anaemic (haemoglobin less than 100 g/l) or Fe-deficient (serum Fe less than 500 μg/l and saturation of total Fe-binding capacity less than 15%).5. The poor availability of Fe in maize meal, which is a staple food of children in Jamaica, is probably an important cause of the high prevalence of Fe-deficiency anaemia.


1980 ◽  
Vol 58 (1) ◽  
pp. 101-103 ◽  
Author(s):  
S. Amin ◽  
T. Spinks ◽  
A. Ranicar ◽  
M. D. Short ◽  
A. V. Hoffbrand

1. Whole-body counting has been used to monitor the clearance of [57Co]cyanocobalamin in normal subjects, vegans and patients with pernicious anaemia. After oral administration of 57Colabelled cyanocobalamin (1 μg/l μCi), subjects were counted for radioactivity monthly for a maximum period of 1 year. 2. The results obtained were consistent with a monoexponential clearance model and a least-squares fit showed that there was no significant difference between the mean clearance rates for the vegans and normal subjects. 3. The patients with pernicious anaemia cleared the vitamin significantly more quickly than the normal control subjects. 4. This may be due to failure to reabsorb biliary vitamin B12 in pernicious anaemia because of the absence of intrinsic factor.


1954 ◽  
Vol 17 (2) ◽  
pp. 205-213 ◽  
Author(s):  
C.J. Earl ◽  
Mildred Jewett Moulton ◽  
Bertram Selverstone

2019 ◽  
Vol 160 (51) ◽  
pp. 2021-2025
Author(s):  
Dániel Németh ◽  
Anikó Folhoffer ◽  
Szilvia Bianka László ◽  
László Kóbori ◽  
Dénes Görög ◽  
...  

Abstract: Introduction: Wilson’s disease is a lethal-without-treatment inherited disorder of copper metabolism. Despite the increased focus on the diagnosis and treatment, liver transplantation is needed in a number of cases even nowadays. Aim: To collect and analyze the data of the Hungarian Wilson’s disease patients who underwent liver transplantation. Method: Data of 24 Wilson’s disease patients who underwent liver transplantation at the Semmelweis University have been analyzed retrospectively. The diagnosis of Wilson’s disease was based on the international score system. The diagnosis of acute liver failure corresponded to the King’s College criteria. All liver transplantations had been performed at the Department of Transplantation and Surgery of Semmelweis University, in 1996 for the first time. Results: The mean age was 26 years, F/M = 13/11. Twelve patients needed urgent liver transplantation for acute liver failure, and 12 underwent transplantation for decompensated liver cirrhosis. One patient had been retransplanted because of chronic rejection. Three patients with acute on chronic liver failure were transplanted via the Eurotransplant program. The mean time on the waiting list was 3 vs 320 days in acute liver failure and chronic liver disease groups, respectively. The overall 5-year survival was 66%, but it was 80% after 2002 indicating both the learning curve effect and the improvement of vigilance in Hungary. Despite difficulties of the diagnostic process, Wilson’s disease was identified in 21/24 patients prior to the transplantation. Conclusion: Liver transplantation is needed in a number of cases of Wilson’s disease. The ideal indication and timing of transplantation may improve the survival of the patients. Orv Hetil. 2019; 160(51): 2021–2025.


1992 ◽  
Vol 82 (2) ◽  
pp. 237-244 ◽  
Author(s):  
A. M. Peters ◽  
P. Allsop ◽  
A. W. J. Stuttle ◽  
R. N. Arnot ◽  
M. Gwilliam ◽  
...  

1. It is widely believed that the lung is an important site of granulocyte margination and releases most of the granulocytes of the peripheral neutrophilia of exercise. 2. We measured granulocyte margination in the lung in terms of the lung total blood granulocyte pool and the lung circulating granulocyte pool in eight patients without inflammatory disease or evidence of lung pathology by comparing the regional γ-camera lung count rate of 111In-labelled granulocytes with that of 111In-labelled erythrocytes. According to the respective 111In activities in peripheral blood samples taken between 5 and 40 min after granulocyte injection, the lung marginating granulocyte pool was 0.78 (sem 0.045) of the lung total blood granulocyte pool or 4.6 (0.92) of the lung circulating granulocyte pool 5 min after injection, decreasing to plateau values of 0.57 (0.053) and 1.53 (0.28) from 20 min after injection. This compared with corresponding whole-body ratios of about 0.6 and 1.5, respectively. 3. After 4 min of maximal exercise in four normal subjects given 111In-labelled granulocytes 60 min before exercise, the 111In-labelled granulocyte count rate over the lung increased to 1.23 (0.05) of the pre-exercise value with a time course that was essentially identical with the time course of the peripheral native neutrophilia. The spleen 111In signal decreased with the same time course, reaching a minimum of 0.63 (0.05) of the pre-exercise level at 5–10 min after the end of exercise. 4. In a further four normal subjects given 99mTc-labelled erythrocytes, exercise resulted in an increase in the lung 99mTc count rate to 1.11 (0.05) of the pre-exercise value. This increase was maximal immediately after the end of exercise. Based on changes in peripheral haemoglobin, total blood volume decreased in the eight normal subjects to 0.89 (0.01) of the pre-exercise value. 5. Using (a) the mean increases seen in lung 111In and 99mTc count rates in the normal subjects, (b) the mean haemoconcentration recorded in the normal subjects and (c) the mean baseline ratio of lung marginating granulocyte pool/lung total blood granulocyte pool measured in the patients, we calculated that the lung marginating granulocyte pool after exercise declined to only 0.94 of the pre-exercise value in the four normal subjects given 111In-labelled granulocytes. 6. We conclude (a) that although granulocytes marginate in the lung, they do not do so to an extent greater than the average for the whole body, and (b) that there is no significant release of granulocytes from the lungs as a result of exercise.


1994 ◽  
Vol 71 (1) ◽  
pp. 95-101 ◽  
Author(s):  
Thomas E. Fox ◽  
Susan J. Fairweather-Tait ◽  
John Eagles ◽  
S. Gabrielle Wharf

Absorption from wheat intrinsically and extrinsically labelled with 67Zn and extrinsically labelled with 65Zn was measured from 67Zn faecal excretion and 6sZn whole-body retention in rats. There were significant differences between the extrinsically- and intrinsically-labelled 67Zn (P < 0·001), but not between the extrinsically-labelled 65Zn and intrinsically-labelled 67Zn. The effect of chicken meat on the absorption of Zn from intrinsically-labelled wheat was also studied in the rat. Mean Zn absorption from wheat and chicken meat fed separately was 18·5 and 68·2% respectively, and from a mixture of the two containing the same level of Zn was 50·1%. The apparent absorption of Zn from the composite meal was significantly higher than predicted from the results of the foods on their own (P < 0·001).


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