O ZINCO NO TRATAMENTO DE DEGENERAÇÃO
HEPATOLENTICULAR
A PROPÓSITO DE TRÊS CASOS
EGBERTO R. BARBOSA * — EMMANUEL DE ALMEIDA BURDMANN *
EDUARDO R. CANÇADO * — MÔNICA 8. HADDAD **
M. SCAFF *** — HORÁCIO M. CANELAS ***
RESUMO — São descritos três casos d© degeneração hepatolenticular (DHL.) em que o
efeito nefrotóxico da D-penicilina (DP) levou à substituição dessa droga por zinco, com
resultados satisfatórios. O tempo médio de tratamento com zinco nos casos descritos foi
de 2 anos e 7 meses. Com base nos dados de literatura e na experiência com esses
pacientes, os autores consideram que os sais de zinco podem ser uma alternativa para o
tratamento da DHL quando a DP determina o aparecimento de efeitos colaterais graves que
levam à sua retirada definitiva.
PALAVRAS CHAVE: degeneração hepatolenticular, terapêutica, zinco.
Oral zinc therapy in hepatolenticular degeneration: three case reports.
SUMMARY — 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 nephro-
pathy. 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
Trabalho do Grupo de Estudo de Distúrbios do Movimento da Divisão de Clínica
Neurológica (DCN), Disciplina de Nefrologia e Disciplina de Gastroenterologia do Hospital
das Clínicas (HC) da Faculdade de Medicina da Universidade de São Paulo (FMUSP): * Mé-
dico Assistente; ** Médica Residente; *** Professor Titular.
Dr. Egberto Reis Barbosa — Climca Neurológica, Hospital das Clínicas, FMUSP - Caixa
Postal 3461 - 01060 São Paulo SP - Brasil.