Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease: Authors’ Reply

Excerpt

To the Editor: We thank Dr Kumar for taking interest in our article [1]. We agree with the author that use of modalities like plasmapheresis should be considered in Wilson’s disease- Acute liver failure (WD-ALF); as seen in our case and in other cases mentioned by the author, plasmapheresis can either provide a bridge to liver transplant or rarely prevent the transplant. The commonly used prognostic scoring system- the revised Wilson’s Prognostic Index was devised after retrospective review of 74 Wilsons disease (WD) children at the King’s College Hospital, London between 1967 and 2000 [2]. The revised score was tested prospectively in 14 WD children between 2001 and 2003 [2]. The validity of this score in the present era with availability of newer hepatic assist systems remains to be established in a larger number of patients. Over-reliance on the prognostic scoring systems may result in unnecessary transplants and misallocation of a rare resource [3]. …