Copper-associated liver disease in childhood

A. Baker, S. Gormally, Romil Saxena, D. Baldwin, B. Drumm, J. Bonham, B. Portmann, A. P. Mowat

Research output: Contribution to journalArticle

42 Citations (Scopus)

Abstract

Background/Aims: Indian childhood cirrhosis is associated with high liver copper concentrations and progressive liver disease with a high mortality. Early treatment with penicillamine was found to reduce mortality and reverse liver damage. We aimed to define the clinical features of copper-associated liver disease outwith the Indian subcontinent and encourage the earlier consideration of the syndrome in cryptogenic liver disease. Methods: Three European children presented between 10 and 29 months of age with abdominal distension, pyrexia and hepatosplenomegaly. Over 1-5 weeks their condition deteriorated rapidly due to liver failure. Two died within 2 months of onset and one received a successful liver transplant. In two cases consideration of the diagnosis occurred only on examination of the liver after orthotopic liver transplant or death. Light microscopy was used, with haematoxylin and eosin, reticulin and orcein stains. Tissue, plasma and water copper levels were measured by flame atomic absorption spectrometry. Results: All had micronodular cirrhosis and severe hepatocellular necrosis with Mallory bodies and copious-orcein positive material. Liver copper concentrations ranged from 1100-1310 μg/g dry weight. For two patients domestic water with high copper content had been used for the preparation of feeds. No environmental source of excess copper could be identified in the third case. Conclusions: We suggest that the above condition, which is called Indian childhood cirrhosis in the Indian subcontinent and Copper Storage Disease elsewhere, would be better named 'Copper-Associated Liver Disease in Childhood', emphasising the need to consider this disorder in unexplained liver disease and to seek possible sources of excessive copper intake.

Original languageEnglish (US)
Pages (from-to)538-543
Number of pages6
JournalJournal of Hepatology
Volume23
Issue number5
DOIs
StatePublished - 1995
Externally publishedYes

Fingerprint

Liver Diseases
Copper
Liver
Mallory Bodies
Reticulin
Transplants
Hepatolenticular Degeneration
Penicillamine
Mortality
Water
Liver Failure
Hematoxylin
Eosine Yellowish-(YS)
Microscopy
Spectrum Analysis
Fibrosis
Fever
Necrosis
Coloring Agents
Light

Keywords

  • Acute liver failure
  • Copper hepatotoxicity
  • Liver transplantation.

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

Cite this

Baker, A., Gormally, S., Saxena, R., Baldwin, D., Drumm, B., Bonham, J., ... Mowat, A. P. (1995). Copper-associated liver disease in childhood. Journal of Hepatology, 23(5), 538-543. https://doi.org/10.1016/0168-8278(95)80059-X

Copper-associated liver disease in childhood. / Baker, A.; Gormally, S.; Saxena, Romil; Baldwin, D.; Drumm, B.; Bonham, J.; Portmann, B.; Mowat, A. P.

In: Journal of Hepatology, Vol. 23, No. 5, 1995, p. 538-543.

Research output: Contribution to journalArticle

Baker, A, Gormally, S, Saxena, R, Baldwin, D, Drumm, B, Bonham, J, Portmann, B & Mowat, AP 1995, 'Copper-associated liver disease in childhood', Journal of Hepatology, vol. 23, no. 5, pp. 538-543. https://doi.org/10.1016/0168-8278(95)80059-X
Baker A, Gormally S, Saxena R, Baldwin D, Drumm B, Bonham J et al. Copper-associated liver disease in childhood. Journal of Hepatology. 1995;23(5):538-543. https://doi.org/10.1016/0168-8278(95)80059-X
Baker, A. ; Gormally, S. ; Saxena, Romil ; Baldwin, D. ; Drumm, B. ; Bonham, J. ; Portmann, B. ; Mowat, A. P. / Copper-associated liver disease in childhood. In: Journal of Hepatology. 1995 ; Vol. 23, No. 5. pp. 538-543.
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AU - Portmann, B.

AU - Mowat, A. P.

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N2 - Background/Aims: Indian childhood cirrhosis is associated with high liver copper concentrations and progressive liver disease with a high mortality. Early treatment with penicillamine was found to reduce mortality and reverse liver damage. We aimed to define the clinical features of copper-associated liver disease outwith the Indian subcontinent and encourage the earlier consideration of the syndrome in cryptogenic liver disease. Methods: Three European children presented between 10 and 29 months of age with abdominal distension, pyrexia and hepatosplenomegaly. Over 1-5 weeks their condition deteriorated rapidly due to liver failure. Two died within 2 months of onset and one received a successful liver transplant. In two cases consideration of the diagnosis occurred only on examination of the liver after orthotopic liver transplant or death. Light microscopy was used, with haematoxylin and eosin, reticulin and orcein stains. Tissue, plasma and water copper levels were measured by flame atomic absorption spectrometry. Results: All had micronodular cirrhosis and severe hepatocellular necrosis with Mallory bodies and copious-orcein positive material. Liver copper concentrations ranged from 1100-1310 μg/g dry weight. For two patients domestic water with high copper content had been used for the preparation of feeds. No environmental source of excess copper could be identified in the third case. Conclusions: We suggest that the above condition, which is called Indian childhood cirrhosis in the Indian subcontinent and Copper Storage Disease elsewhere, would be better named 'Copper-Associated Liver Disease in Childhood', emphasising the need to consider this disorder in unexplained liver disease and to seek possible sources of excessive copper intake.

AB - Background/Aims: Indian childhood cirrhosis is associated with high liver copper concentrations and progressive liver disease with a high mortality. Early treatment with penicillamine was found to reduce mortality and reverse liver damage. We aimed to define the clinical features of copper-associated liver disease outwith the Indian subcontinent and encourage the earlier consideration of the syndrome in cryptogenic liver disease. Methods: Three European children presented between 10 and 29 months of age with abdominal distension, pyrexia and hepatosplenomegaly. Over 1-5 weeks their condition deteriorated rapidly due to liver failure. Two died within 2 months of onset and one received a successful liver transplant. In two cases consideration of the diagnosis occurred only on examination of the liver after orthotopic liver transplant or death. Light microscopy was used, with haematoxylin and eosin, reticulin and orcein stains. Tissue, plasma and water copper levels were measured by flame atomic absorption spectrometry. Results: All had micronodular cirrhosis and severe hepatocellular necrosis with Mallory bodies and copious-orcein positive material. Liver copper concentrations ranged from 1100-1310 μg/g dry weight. For two patients domestic water with high copper content had been used for the preparation of feeds. No environmental source of excess copper could be identified in the third case. Conclusions: We suggest that the above condition, which is called Indian childhood cirrhosis in the Indian subcontinent and Copper Storage Disease elsewhere, would be better named 'Copper-Associated Liver Disease in Childhood', emphasising the need to consider this disorder in unexplained liver disease and to seek possible sources of excessive copper intake.

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