Korean J Pediatr Gastroenterol Nutr.  2002 Sep;5(2):206-212.

A Case of Minimal Change Nephrotic Syndrome Associated with D-penicillamine Therapy of Wilson's Disease

• Oh KW
• Kim SY
• Lee HS
• Choe BH
• Ko CW
• Koo JH

Abstract

Wilson's disease is a treatable autosomal recessive inherited disorder of copper metabolism due to mutation of the copper transporting gene. The basic strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering both a low copper diet and copper-chelating agents. D-penicillamine is the first choice as a copper-chelating agent. Some serious side effects could occur in 3~5% of all patients following D-penicillamine therapy. We report a 19 year-old male with Wilson's disease who developed nephrotic syndrome 6 months after the initiation of D-penicillamine therapy. Prednisolone was administered to control nephrotic syndrome and D-penicillamine was switched to trientine. Urinary remission was achieved within a week and maintained thereafter. Nephrotic syndrome was proven to be MCNS by kidney biopsy.

Keyword

Wilson's disease; D-penicillamine; Side effect; Nephrotic syndrome; Trientine

MeSH Terms

Biopsy
Copper
Diet
Hepatolenticular Degeneration*
Humans
Kidney
Liver
Male
Metabolism
Nephrosis, Lipoid*
Nephrotic Syndrome
Penicillamine*
Prednisolone
Trientine
Young Adult
Copper
Penicillamine
Prednisolone
Trientine