The drug of choice for the initial treatment of "decoppering" in Wilson's disease, an inherited disorder of copper metabolism, is the chelating agent D-penicillamine. In the case of harmful side-effects an alternative drug is triethylenetetramine dihydrocholoride (trien or trientine). Using the 24-h-urine excretion of copper and the oral copper loading test with copper-64, a double function for trien was found: trien increases the urine copper excretion and decreases the intestinal copper absorption respectively.
Stable isotopes, such as 13C, should be substituted for the long-lived radionuclide 14C in the 14C breath test when doing liver function tests in children and pregnant women. For comparison 13C, 15N-methacetin and 14C-methacetin were synthesized as suitable diagnostic agents. Methods are described for the measurement of 14C and 13C in the breath. After oral administration of labelled methacetin to healthy subjects and patients with liver diseases a good correspondence between 13C- and 14C-measurements in the same subject on the one hand, and a good discrimination between controls and patients on the other hand were shown. Findings with regard to 14C measurements in urine are discussed supporting the supposed advantage of 13C-methacetin over 14C-methacetin application.
The hepatic uptake of 64Cu in the body was studied by whole-body counting in normal subjects, homozygotes, and heterozygotes of Wilson’s disease. Special attention was paid to the copper kinetics during the first hour after injection of 64Cu. Two different measuring techniques were used simultaneously: one collimated Nal(Tl) crystal was in a fixed position over the liver, the counts being recorded by a single channel analyser connected to a multi-channel analyser in a multi-channel scaling mode; and a second collimated crystal makes scanning movements along the body axis, the counts being recorded by a multi-channel analyser in a multi-spectrum scaling mode. With this procedure it is possible to use a double nuclide labelling technique by means of which both the 64Cu values for an organ can be corrected for the 64Cu blood content in the region of interest, and information can be obtained on the actual dynamic 64Cu movement in the body.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.