Marked Iron in Liver Explants in the Absence of Major Hereditary Hemochromatosis Gene Defects: A Risk Factor for Cardiac Failure
| Autor: | Matthew M. Yeh, Linda D. Ferrell, Perumal Vivekanandan, Hubert Fenton, John Hart, Michael Torbenson |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
Liver Cirrhosis
Male Pathology medicine.medical_specialty Cirrhosis Heart disease Iron medicine.medical_treatment Hepatitis C virus Liver transplantation medicine.disease_cause Liver Cirrhosis Alcoholic Risk Factors Humans Medicine Risk factor Hemochromatosis Protein Retrospective Studies Heart Failure Transplantation business.industry Histocompatibility Antigens Class I Membrane Proteins Middle Aged medicine.disease Hepatitis C Liver Transplantation Amino Acid Substitution Hereditary hemochromatosis Heart failure Female Hemochromatosis business |
| Zdroj: | Transplantation. 87:1256-1260 |
| ISSN: | 0041-1337 |
| Popis: | Background. Patients with hereditary hemochromatosis are known to have an increased risk for morbidity and mortality after orthotopic liver transplantation. Methods. The clinical, histological, and genetic findings were examined in a series of seven adult patients with marked iron accumulation in their liver explants and cardiac failure despite the absence of HFE mutations. Results. Causes for cirrhosis were alcohol and hepatitis C virus (HCV) (n=2), HCV (n=1), alcohol (n=1), and cryptogenic cirrhosis (n=3). Ages at transplantation ranged from 46 to 62 years. Genetic studies confirmed all seven cases were negative for HFE mutations C282Y and H63D. The liver explants showed marked iron accumulation that predominately involved hepatocytes, with more than 90% of the iron in hepatocytes. Two patients required cardiac transplantation and four died of cardiac failure. Cardiac tissues obtained from autopsies (n=3), endomyocardial biopsy (n=1), or cardiac transplants (n=2) showed marked myocyte hypertrophy and iron deposits with or without interstitial fibrosis. Conclusions. This study highlights a unique set of liver transplant patients with marked iron deposition in their cirrhotic liver who developed severe cardiac failure and have iron deposits in the heart, despite the absence of major HFE gene mutations. The cause of the systemic iron overload remains to be discovered. |
| Databáze: | OpenAIRE |
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