Primary liver cancer and survival in patients undergoing liver transplantation for hemochromatosis

Kowdley, Kris V.; Hassanein, Tarek; Kaur, Simrit; Farrell, Frank J.; Thiel, David H. Van; Keeffe, Emmet B.; Sorrell, Michael F.; Bacon, Bruce R.; Weber, Fredrick L.; Tavill, Anthony S.

doi:10.1002/lt.500010408

Cited by 100 publications

(67 citation statements)

References 13 publications

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“…The long-term survival in heterozygous recipients was significantly better than previous studies describing transplantation in putative hemochromatosis homozygotes (1-year survival of 58% ). 32 In summary, there was no increased prevalence of the C282Y mutation in 304 patients with end-stage liver disease. Although stainable iron is common in C282Y heterozygotes, hepatic iron concentration did not differ between heterozygotes and wild-type recipients with end-stage liver disease.…”

Section: Discussionmentioning

confidence: 90%

Prevalence of the C282Y mutation of the hemochromatosis gene in liver transplant recipients and donors

et al. 1999

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Section: Discussionmentioning

confidence: 90%

Prevalence of the C282Y mutation of the hemochromatosis gene in liver transplant recipients and donors

et al. 1999

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“…Clinical, biochemical, and pathological information on patients with severe hepatic iron overload believed to have HHC who underwent OLT were reported previously. 5 The diagnosis of iron overload was based on the following: elevated serum ferritin (Ͼ400 µg/L) and fasting transferrin saturation values (Ͼ62%), hepatic iron index (HII; hepatic iron concentration in micromoles per gram of dry weight/age in years) of 2 or greater in the absence of prior phlebotomy therapy or significant gastrointestinal bleeding, and/or pattern of significantly increased liver iron staining, predominantly in hepatocytes and bile duct cells in the absence of other causes of liver iron overload other than end-stage liver disease. Long-term follow-up information was collected by review of patient records.…”

Section: Methodsmentioning

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.…”

mentioning

confidence: 99%

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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“…[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. However, because the results have been disappointing, more research is needed to determine the optimum use of transplantation in these patients (rating, III D).…”

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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Primary liver cancer and survival in patients undergoing liver transplantation for hemochromatosis

Cited by 100 publications

References 13 publications

Prevalence of the C282Y mutation of the hemochromatosis gene in liver transplant recipients and donors

Prevalence of the C282Y mutation of the hemochromatosis gene in liver transplant recipients and donors

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

Liver transplantation

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