ISSN - 0973-0958

Pediatric Oncall Journal

When to stop Penicillamine in Wilson`s disease_?

When to stop Penicillamine in Wilson`s disease_?

Dr Ira Shah.
Medical Sciences Department, Pediatric Oncall, Mumbai, India.

Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056
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.

How long this child should be continued on penicillamine_?
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.
Compliance with ethical standards
Funding:  None  
Conflict of Interest:  None
Cite this article as:
Shah I. When to stop Penicillamine in Wilson`s disease_?. Pediatr Oncall J. 2016;13: 29. doi: 10.7199/ped.oncall.2016.6
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License
Disclaimer: The information given by is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.
0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0