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Canadian Journal of Gastroenterology
Volume 15, Issue 8, Pages 537-540
http://dx.doi.org/10.1155/2001/651470
Brief Communication

Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis

Susan E Natsheh,1 Eve A Roberts,1,2 Bo Ngan,3 Peter Chait,4 and Vicky Lee Ng1,2

1Department of Paediatrics, Division of Gastroenterology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada
2Paediatric Academic Multi-Organ Transplant (PAMOT) Program, The Hospital for Sick Children, Toronto, Ontario, Canada
3Department of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada
4Department of Radiology, The Hospital for Sick Children, Toronto, Ontario, Canada

Received 28 February 2001; Accepted 28 February 2001

Copyright © 2001 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) may manifest as neonatal liver failure characterized by hepatosplenomegaly, profound coagulopathy, ascites and hyperbilirubinemia. Marked hyperferritinemia may be present in these patients, mimicking perinatal hemochromatosis. Tissue specimens are critical in distinguishing these two diseases and in directing management. Clinical recognition and diagnosis of HLH can be difficult but are crucial for appropriate therapy and genetic counselling. Liver transplantation is absolutely contraindicated for patients with HLH but may be the only life-saving treatment modality for patients with perinatal hemochromatosis.