The Effect of BAL (2,3-Dimercaptopropanol) on Hepatolenticular Degeneration (Wilson's Disease)

Studies on Copper Metabolism. XIII. Hepatolenticular Degeneration1

“…Furthermore, in this laboratory it was possible to damage the renal tubular epithelium of rats by the administration of excessive amounts of copper (44). In further support of this concept is the observation that, after the mobilization of copper from the tissues, clinical improvement occurred in some patients (6). In one of our patients (D. C.) the degree of improvement has been extremely impressive.…”

“…The amount of copper in the tissues, particularly in the liver and involved areas of the brain, is increased about ten-fold above the normal (1)(2)(3)(4). The pigmented KayserFleischer rings have been shown to contain copper (5,6). Increased excretion of copper in the urine has also been demonstrated (7)(8)(9).…”

“…(dimercaptopropanol) (6,7,9,10), glycine (10), alanine (10), "Versene" (calcium disodium salt of ethylenediamine tetra-acetic acid) (13), a high protein diet (13), and cortisone (13), the excretion of copper in the urine is enhanced. Clinical improvement has been reported in some patients following repeated courses of B.A.L.…”

Studies on Copper Metabolism. Vii. Blood Copper in Pregnancy and Various Pathologic States 1

“…Significant hypocupremia was observed only in "lipoid" nephrosis and in hepatolenticular degeneration (Wilson's disease). This is of interest in view of the reported increase in urinary excretion of copper in patients with these two diseases (36,37).…”

Studies on Copper Metabolism. Xvi. Radioactive Copper Studies in Normal Subjects and in Patients With Hepatolenticular Degeneration 1

“…Consequently, the specific activity of the copper excreted in the bile could be reduced even though the total quantity of copper excreted by this route is not decreased. That this is a plausible explanation is suggested by our earlier observation (2) in a single patient that the concentration of copper in the bile removed directly from the gall-bladder at autopsy was normal and the finding by Denny-Brown and Porter (19) (2,9) and the radiocopper balance techniques (10,13) were used. In the present study, however, the proportion of the ingested radiocopper recovered in the stools of both the normal subjects and the patients was considerably higher than the values obtained by Matthews (10) and by Bearn and Kunkel (13).…”