Outcome of liver transplantation in patients with hemochromatosis

Farrell, Frank J.; Nguyen, Minh; Woodley, Susan; Imperial, Joanne C.; Garcia-Kennedy, Richard; Man, Kwun Nok Mimi; Esquivel, Carlos O.; Keeffe, Emmet B.

doi:10.1002/hep.1840200221

Cited by 115 publications

(40 citation statements)

References 19 publications

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“…[6][7][8] We previously reported short-term results after OLT in a cohort of 37 patients who were clinically diagnosed with HHC based on phenotypic criteria. 5 This report presents long-term follow-up data in this cohort, showing that 5-year survival after OLT is also reduced in this group compared with the OLT population as a whole.…”

Section: Discussionmentioning

confidence: 99%

“…6 In addition, ongoing cardiac iron loading could potentially be a cause of late post-OLT morbidity in patients with abnormally elevated intestinal iron absorption and would be expected to respond to post-OLT iron reduction therapy. The optimal post-OLT management of patients with hepatic iron overload is far from clear.…”

Section: Discussionmentioning

confidence: 99%

“…4 We previously reported the 1-year survival rate after OLT among patients with marked hepatic iron overload in the native liver is only 58%, less than that for other indications. 5 Other studies have shown 1-year survival rates of 53% to 83% in patients with hepatic iron overload, [6][7][8] although long-term survival data have not previously been reported. The goals of this study are to examine long-term survival after OLT in patients with severe hepatic iron overload in the native liver and determine the causes of death in this population.…”

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confidence: 99%

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Long-term follow-up after liver transplantation in patients with hepatic iron overload

et al. 1999

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Patients with hepatic iron overload who undergo orthotopic liver transplantation (OLT) have a worse 1-year survival than those who undergo transplantation for other indications; the long-term outcome in this population is unknown. The purpose of this study is to report long-term follow-up after OLT in a cohort of patients with hepatic iron overload. Five liver transplant centers in the United States reported follow-up data on 37 patients receiving a first liver transplant who had severe hepatic iron overload in their native livers. KaplanMeier 5-year survival among these patients was compared with survival data from all age-matched liver transplantations reported to the United Network for Organ Sharing (UNOS) over the same time period (1987 to 1993). The 5-year survival rate after OLT was 40% in the hepatic iron overload group compared with an overall survival rate of 62% for all patient groups from the UNOS registry (P ‫؍‬ .0009). Although sepsis was the cause of 53% of all deaths occurring within the first year after OLT, cardiac complications accounted for 50% of the late mortality in patients with hepatic iron overload. In conclusion, longterm survival after OLT is significantly decreased in patients with hepatic iron overload. Infectious and cardiac complications are the most common causes of death in these patients. Further studies are needed to define the relationship between hepatic iron overload and mortality and to examine the effect of iron depletion on outcome after OLT in this patient population.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Long-term follow-up after liver transplantation in patients with hepatic iron overload

et al. 1999

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“…Postoperative survival is significantly less than that for patients who undergo transplantation for other conditions. [66][67][68] Although not well characterized, these suboptimal results appear to result from a high rate of postoperative infection and occasional deaths from cardiomyopathy. 69,70 Recommendations Liver transplantation is indicated for carefully selected patients with hereditary hemochromatosis.…”

Section: Metabolic Diseasesmentioning

confidence: 99%

Liver transplantation

Carithers

2000

Liver Transpl

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Liver transplantation has revolutionized the care of patients with end-stage liver disease. Liver transplantation is indicated for acute or chronic liver failure from any cause. Because there are no randomized controlled trials of liver transplantation versus no therapy, the efficacy of this surgery is best assessed by carefully comparing postoperative survival with the known natural history of the disease in question. The best examples of this are in primary biliary cirrhosis and primary sclerosing cholangitis, for which well-validated disease-specific models of natural history are available. There are currently relatively few absolute contraindications to liver transplantation. These include severe cardiopulmonary disease, uncontrolled systemic infection, extrahepatic malignancy, severe psychiatric or neurological disorders, and absence of a viable splanchnic venous inflow system. One of the most frequently encountered contraindications to transplantation is ongoing destructive behavior caused by drug and alcohol addiction. The timing of the surgery can have a profound impact on the mortality and morbidity of patients undergoing liver transplantation. Because of the long waiting lists for donor organs, the need to project far in advance when transplantation might be required has proven to be one of the greatest challenges to those treating patients with end-stage liver disease. Three important questions must be addressed in a patient being considered for liver transplantation: (1) when should the patient be referred for possible transplantation? (2) when should the patient be listed for transplantation? and (3) when is the patient too sick to have a reasonable chance of surviving the perioperative period?

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“…Despite the apparent heterogeneity among patients who have undergone transplantation with cirrhosis thought secondary to HHC, these patients have an increased risk of posttransplantation morbidity and mortality compared to transplant recipients with other causes of end-stage liver disease. [5][6][7][8][9] Recently, a gene strongly associated with HHC was discovered and named HFE. 10 Within this gene, 2-point mutations were discovered.…”

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confidence: 99%

Iron overload in cirrhosis?HFE genotypes and outcome after liver transplantation

et al. 2000

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Previously, we found appreciable hepatic iron deposition in one third of our patients undergoing liver transplantation (LTx) with approximately 10% of cases having quantifiable iron in the range of that seen in hereditary hemochromatosis (HHC). The aim of this study was to compare clinical outcome in liver transplant patients with and without iron overload. We also sought to determine the prevalence of HFE mutations in liver transplant patients with iron overload. Of 456 consecutive liver transplants, 41 explants had an hepatic iron index (HII) greater than 1.9, and these cases were compared to 41 matched liver transplant recipients without increased hepatic iron. Posttransplantation complications, along with patient and graft survival were monitored. HFE gene testing was performed using DNA-based techniques. Kaplan-Meier 5-year patient survival after LTx was significantly lower in cases with hepatic iron overload compared to matched controls without iron excess (48% vs.

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Outcome of liver transplantation in patients with hemochromatosis

Cited by 115 publications

References 19 publications

Long-term follow-up after liver transplantation in patients with hepatic iron overload

Long-term follow-up after liver transplantation in patients with hepatic iron overload

Liver transplantation

Iron overload in cirrhosis?HFE genotypes and outcome after liver transplantation

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