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• W2052346411 abstract "Summary: The distribution of 64Cu is reported in whole blood, plasma fractions, urine, and stool in an adult volunteer and two patients with Menkes' kinky hair disease (trichopoliodystrophy) after in-tragastric and iv administration. In the adult, after oral circulating 64Cu, showed an initial peak at 40–60 min which was 4% of the total dose (or 5% of the amount calculated to be absorbed), a small secondary peak at 3.5% by 4 hr, then a gradual rise to 32 hr. Approximately 55% of the whole blood radioactivity was in the plasma from 8–48 hr. In the two patients, after ingestion or injection, there was no early blood peak and about 10% of both the absorbed or injected copper persisted in the circulation from the 4th hr to beyond 24 hr in a pattern similar to that in the adult, but less than 40% of the 64 64Cu, was in the plasma fraction after 4 hr. In all subjects, the albumin/globulin ratio of radioactivity fell progressively, suggesting prompt ceruloplasmin synthesis by the liver. No radioactivity was detected in cerebrospinal fluid 10 hr after 64Cu, injection in patient 2. Urinary excretion of 64Cu, in the adult appeared to be linear after 6 hr (approximately 0.08% of the total dose per 24 hr). In patient 1, it was half as great (0.04%/24 hr), but if calculated on the assumption that only 6% of ingested copper was absorbed in patient 1, his urinary excretion rate, 0.67%/24 hr would be eight times that of the control, strongly suggestive of renal inability to conserve copper. In the adult, stool radioactivity totaled 24% of the ingested dose by 48 hr. In patient 1, stool radioactivity was already 94% by 48 hr and in patient 2, after iv 64Cu, stool radioactivity was only 2.8% by 96 hr. This low stool radioactivity after injected copper indicated minimal biliary loss of radiocopper, and ruled out a rapid enterohepatic recycling of copper in this disease. Because 94% of a physiologic dose of 64Cu, was in the 48-hr stool in patient 1, <8% of his ingested copper could have been absorbed, compared with over 80% in the adult control. This confirms that there is an absorption defect for copper in Menkes' disease, resulting in approximately 10% of normal copper uptake. Speculation: The data are interpreted as confirming that there is a mucosl block to copper absorption in Menkes' disease, but that this block is incomplete. Despite depleted circulating copper, erythrocyte uptake appeared normal or high in these patients, and hepatic incorporation into ceruloplasmin seemed to be unimpaired. There may be renal transport abnormalities in Menkes' disease as well, because of the unexpectedly high urinary 64Cu, excretion in the presence of hypocupremia. Perhaps an abnormality of blood-brain copper distribution is responsible for the severe neurologic features of this disease." @default.
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• W2052346411 date "1979-11-01" @default.
• W2052346411 modified "2023-10-17" @default.
• W2052346411 title "Distribution of Ingested and Injected Radiocopper in Two Patients with Menkes' Kinky Hair Disease" @default.
• W2052346411 doi "https://doi.org/10.1203/00006450-197911000-00015" @default.
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