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Penicillamine is the drug of choice for the treatment of Wilson's disease, whatever the stage of the illness. Toxic manifestations may preclude the use of this life-saving drug in some patients and discontinuation of penicillamine therapy usually leads to death. We report our experience with Trientine in seven patients, aged 13 to 33 years, with Wilson's disease who developed toxic manifestations with penicillamine that required discontinuation of therapy. These include two with nephrosis, one with neutropenia, two with thrombocytopenia, and one each with a SLE-like and a Henoch-Schonlein-like syndrome. The patients were treated for periods from 6 weeks to 16 years with a dose of 0.5 to 2 g/day. Trientine proved to be an effective alternative copper chelating agent in the treatment of Wilson's disease in patients with penicillamine-induced neutropenia, thrombocytopenia, SLE, and nephrosis. No serious untoward side effects were noted.

The needs for dietary zinc are adequately met by most fully breast-fed infants despite relatively low zinc intakes in relation to estimated requirements. The objective of this study was to use stable isotope techniques to evaluate how zinc retention is achieved in normal fully breast-fed infants. Nine male infants, aged 2-5 mo, were fed expressed human milk labeled with 70Zn over a 24-h period. Complete fecal collections were obtained for 8 d. On d 4-7, a metabolic period was initiated which included test weighing and milk sampling, to measure zinc intake, and daily urine collections. Isotopic enrichment of fecal and urine samples was determined by fast atom bombardment mass spectrometry. Results included a mean (+/- SD) dietary zinc intake of 17.8 +/- 6.6 mumol/d; fractional absorption of 0.54 +/- 0.075; and total absorbed zinc of 9.5 +/- 3.5 mumol/d. Mean endogenous fecal zinc, determined on seven infants by isotope dilution, was 4.7 +/- 2.3 mumol/d, which resulted in a mean net absorption of 4.8 +/- 3.4 mumol/d. The results of the study indicated that, for fully breast-fed infants, it is the combination of a relatively high fractional absorption and efficient conservation of intestinal endogenous zinc that results in zinc retention adequate to meet the demands of growth in the face of modest intake.

1. The chromium concentration was determined in 261 samples of breast milk collected by manual expression from forty-five American women.2. Cr was measured in whole, liquid milk by graphite-furnace atomic absorption spectrophotometry, using the method of standard additions.3. The mean Cr content of the breast milk samples was 0.30 ng/ml. The range of individual values was 0.06–1.56 ng/ml and did not change significantly with duration of lactation.4. The Cr content of breast milk from these American women is similar to levels reported recently in milk from Finnish women.

The therapeutic effect of orally administered zinc was evaluated in an adult woman with acrodermatitis enteropathica. When she was off therapy and in clinical relapse the plasma zinc concentration (10 mug per 100 ml), serum alkaline phosphatase (3 1U per liter) and urine zinc excretion rate (39 mug per 24 hours) were extremely low. Di-iodohydroxyquin therapy was accompanied by a modest increase in plasma zinc concentrations. Oral zinc sulfate (220 mg three times a day or 50 mg twice a day) resulted in rapid and complete clinical remission, and in a return of plasma zinc, serum alkaline phosphatase and urinary zinc excretion to normal. These data are compatible with a severe zinc deficiency state and indicate that the inherited defect in this disease is either in or closely related to zinc metabolism. The beneficial effects of zinc therapy in this patient provide further confirmation of the efficacy of oral zinc in the treatment of acrodermatitis enteropathica.

The effect of time between collection and separation of blood samples obtained from 9 subjects was examined with respect to plasma and serum zinc concentrations. There was a linear increase of 6% in zinc concentrations for the first 2 h that was similar for plasma and serum. Plasma zinc continued to increase linearly over the next 2 h, but increase in serum zinc concentrations was less for the second 2 h. It is concluded that differences between heparinized plasma and serum zinc concentration observed previously in this laboratory and elsewhere are attributable primarily to differences in time between collection and separation.