Three patients with symptoms and signs of hepatolenticular degeneration (HLD) who developed serious renal side effects of D-penicillamine (DP) had their therapeutic schedule changed to zinc. Patient 1, a 55 year-old man had been well until 12 years old, when skeletal changes (osteomalacia) due to tubular renal disturbance began. His diagnosis of HLD had first been established at age of 32 when he presented with «wing-beat» tremor. He was then begun on DP and his neurological symptons resolved within one year of initiating therapy but skeletal abnormalities remained unchanged as a sequel. During the next 22 years the patient was continued on DP therapy but with poor compliance. Then the reapperance of his neurological manifestations occurred several times. By the age of 53, after one year without therapy, his neurological status has worse. DP was reinstituted but some weeks later his renal laboratorial parameters became serevely affected. DP was discontinued and zinc sulfate (220mg three times daily) was introduced. On this therapeutic regimen his renal laboratorial parameters returned to previous level after one month. Within one year on this therapeutic regimen neurological manifestations were resolved. After 31 months on zinc treatment he remains neurologically asymptomatic and his renal function is satisfactory. Patient 2, a 41 years old woman had been her diagnosis of HLD at age of 20, when following the diagnosis of the disease in her old brother, she was found to have the laboratorial features of HLD and bilateral Kayser-Fleischer rings. DP treatment was recommended at that time but she quitted the follow-up. When she. was 23, an esophageal variceal bleeding occurred. At the age of 30 she started DP treatment. During 9 years the patient, under regular use of the drug remained assymptomatic. At the age of 39 she developed acute renal failure due to penicillamine-induced immune complex nephropathy. DP was then stopped and prednisone introduced. After 4 months, as renal function returned to normal, prednisone was discontinued and zinc sulphate (220mg three times daily) introduced. She remained asymptomatic for 38 months. By the age of 41 a relapse of renal manifestations occurred, and she died months later. Patient 3, a 20 year-old man developed hemolytic anemia as the single manifestation of HLD at 16. DP was introduced with excellent results. Neverthless, 4 months later, with the appearance of severe renal side--effects, this drug was replaced by zinc sulphate (220mg three times daily). After 11 months under this therapeutic schedule he was hematologically asymptomatic but an epigastric distress appeared. Zinc sulphate was discontinued and zinc acetate (170mg three times daily) was introduced. Since that time, after 30 months of treatment with zinc, he remains asymptomatic and Kayser-Fleisher rings disappeared. A review of zinc salts therapy of HLD is presented and discussed.
hepatolenticular degeneration; therapeutics; zinc