Indian Journal of Gastroenterology

, Volume 37, Issue 1, pp 31–38 | Cite as

Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson’s disease in resource constrained setting

  • Piyush Gupta
  • Mehul Choksi
  • Ashish Goel
  • Uday Zachariah
  • Kattiparambil Gangadharan Sajith
  • Jeyamani Ramachandran
  • George Chandy
  • George Kurian
  • Grace Rebekah
  • Chundamannil Eapen Eapen
Original Article
  • 64 Downloads

Abstract

Background

Experience with zinc in treating symptomatic hepatic Wilson’s disease (WD) is limited.

Aim

To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson’s disease.

Methods

We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child’s, model for end-stage liver disease (MELD), Nazer’s, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow up.

Results

Thirty-one patients (median age 11 [5–24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child’s class A, five to Child’s B, and 17 to Child’s C. Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, and of subsequent zinc therapy was 363 (35–728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child’s, MELD, Nazer’s, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2–20) years. Fifteen of the 17 Child’s C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up.

Conclusions

Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation.

Keywords

Hepatic Wilson’s disease Penicillamine Symptomatic Wilson’s Wilson’s disease Zinc 

Notes

Acknowledgements

We acknowledge the Fluid research funds (Christian Medical College, Vellore, India) for funding this study.

Compliance with ethical standards

Conflict of interest

PG, MC, AG, UZ, KGS, JR, GC, GK, GR, and CEE declare that they have no conflict of interest.

Ethics statement

The authors declare that the study was performed in a manner conforming to the Helsinki declaration of 1975, as revised in 2000 and 2008 concerning human and animal rights, and the authors followed the policy concerning informed consent as shown on Springer.com.

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Copyright information

© Indian Society of Gastroenterology 2018

Authors and Affiliations

  • Piyush Gupta
    • 1
  • Mehul Choksi
    • 1
  • Ashish Goel
    • 1
  • Uday Zachariah
    • 1
  • Kattiparambil Gangadharan Sajith
    • 1
  • Jeyamani Ramachandran
    • 1
  • George Chandy
    • 1
  • George Kurian
    • 1
  • Grace Rebekah
    • 2
  • Chundamannil Eapen Eapen
    • 1
  1. 1.Department of HepatologyChristian Medical CollegeVelloreIndia
  2. 2.Department of BiostatisticsChristian Medical CollegeVelloreIndia

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