Norman P. Goldstein

Copper content of saliva of normal subjects and treated Wilson's disease patients

Abstract Saliva from 6 male and 2 female patients with Wilsons disease was analyzed for copper by a modified spectrophotometric procedure employing oxalyldihydrazide as a color reagent. The values fell within the range obtained for 89 male control subjects, i.e., 1 to 75 μg./100 ml., with the frequency distribution being skewed toward the lower quantities. All 8 patients had been under therapy for several years with a low copper diet and daily administration of D-Penicillamine. Thus, although it is possible that untreated patients might yield different data, it appears that in Wilsons disease there is no abnormal accumulation of copper in saliva. Although a similar situation exists with nails and hair, this is in contrast to most other tissues and fluids which accumulate excessive copper in this disease.

Effect of adrenocortical steroids on the hypercalciuria of Wilson's disease (hepatolenticular degeneration)☆

Abstract The effect of adrenocortical steroids on the urinary and fecal excretion of calcium was studied in 2 cases of Wilsons disease: one patient had hypercalciuria; the other did not. Both patients had an increase in urinary excretion and a decrease in fecal excretion of calcium while receiving orally 45 mg. of prednisone a day. The patient without hypercalciuria later exhibited similar changes when given orally 45 mg. of cortisone acetate a day. Although these findings shed no light on the cause of hypercalciuria seen in some cases of Wilsons disease, they do show that the urinary excretion of calcium by patients with Wilsons disease can be altered by the administration of adrenocortical steroids.

Intake—Excretion studies of fat and nitrogen in 7 patients with hepatolenticular degeneration (Wilson's disease)

Summary and conclusionsIntake-excretion studies of fat and nitrogen were performed in 7 patients with Wilsons disease. Standard technics were used.Fecal fat excretion was slightly to moderately increased in 2 patients who had Wilsons disease with clinically overt cirrhosis (average daily fecal fat excretion up to 16.5 gm. and 9.5 gm., respectively). Fecal fat values reverted to normal in these 2 patients as clinical improvement occurred.Fecal nitrogen excretion was normal for all 7 patients with Wilsons disease.The pathogenetic basis for steatorrhea in Wilsons disease has not been established.