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

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...

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Main Authors: Susan E Natsheh, Eve A Roberts, Bo Ngan, Peter Chait, Vicky Lee Ng
Format: Article
Language:English
Published: Wiley 2001-01-01
Series:Canadian Journal of Gastroenterology
Online Access:http://dx.doi.org/10.1155/2001/651470
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author Susan E Natsheh
Eve A Roberts
Bo Ngan
Peter Chait
Vicky Lee Ng
author_facet Susan E Natsheh
Eve A Roberts
Bo Ngan
Peter Chait
Vicky Lee Ng
author_sort Susan E Natsheh
collection DOAJ
description 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.
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publishDate 2001-01-01
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series Canadian Journal of Gastroenterology
spelling doaj-art-bfc2c76f3c8842b2873712e1520d103c2025-02-03T01:29:11ZengWileyCanadian Journal of Gastroenterology0835-79002001-01-0115853754010.1155/2001/651470Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the DiagnosisSusan E Natsheh0Eve A Roberts1Bo Ngan2Peter Chait3Vicky Lee Ng4Department of Paediatrics, Division of Gastroenterology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, CanadaDepartment of Paediatrics, Division of Gastroenterology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, CanadaDepartment of Pathology, The Hospital for Sick Children, Toronto, Ontario, CanadaDepartment of Radiology, The Hospital for Sick Children, Toronto, Ontario, CanadaDepartment of Paediatrics, Division of Gastroenterology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, CanadaHemophagocytic 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.http://dx.doi.org/10.1155/2001/651470
spellingShingle Susan E Natsheh
Eve A Roberts
Bo Ngan
Peter Chait
Vicky Lee Ng
Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis
Canadian Journal of Gastroenterology
title Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis
title_full Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis
title_fullStr Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis
title_full_unstemmed Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis
title_short Liver Failure with Marked Hyperferritinemia: ‘Ironing Out’ the Diagnosis
title_sort liver failure with marked hyperferritinemia ironing out the diagnosis
url http://dx.doi.org/10.1155/2001/651470
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