When to stop Penicillamine in Wilson`s disease_?
 
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Ira Shah
Medical Sciences Department, Pediatric Oncall, Mumbai, India.

Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056, India.


Clinical Problem :
A 5 years old boy was diagnosed as Wilson’s disease at the age of 4½ years in view of hepatitis with jaundice, increased copper content in liver {132 µg, gm} and elevated urine copper {643}. His ophthalmological evaluation revealed no KF ring. He was started on Penicillamine which he had received for past 6 months. He was referred for further management. On examination, he had hepatomegaly. Other systems were normal. A repeat urinary copper was 105.4 in 24 hours. There were still no KF rings.


Question :
How long this child should be continued on penicillamine_?

Expert Opinion :
The mainstay of therapy for Wilson disease is pharmacologic treatment with chelating agents along with dietary modification. Pharmacologic treatment can be divided into two phases: the initial phase, when toxic copper levels are brought under control, and maintenance therapy. Generally, penicillamine {25mg, kg, day in 2 dosages} is the first treatment used. This binds with copper and leads to excretion of copper in the urine. Zinc usually in the form of a zinc acetate, may be used to maintain stable copper levels in the body. Zinc stimulates metallothionein, a protein in gut cells that binds copper and prevents their absorption and transport to the liver. If zinc and penicillamine are given together, the interval between zinc and penicillamine should be at least 4 hrs. Zinc is the optimal drug for maintenance therapy and for the treatment for the presymptomatic patient.
Since this child now has decreasing urine copper and no KF ring, maintenance therapy may be required in this child now.


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