Survival and causes of death in cirrhotic and in noncirrhotic patients with primary hemochromatosis.

作者: Claus Niederau , Rudolf Fischer , Amnon Sonnenberg , Wolfgang Stremmel , Hans J. Trampisch

DOI: 10.1056/NEJM198511143132004

关键词: Life expectancyCardiomyopathySurgeryGastroenterologyCirrhosisHemochromatosisBloodlettingDiabetes mellitusMortality rateInternal medicineLiver cancerMedicine

摘要: We analyzed survival and causes of death among 163 patients with primary hemochromatosis diagnosed between 1959 1983. The mean follow-up period was 10.5 +/- 5.6 years (+/- S.D.). Cumulative 92 per cent at 5 years, 76 10 59 15 49 20 years. Life expectancy reduced in cirrhosis the liver as compared those without (P less than or equal to 0.05), diabetes mellitus 0.002), who could not be depleted iron during first 18 months venesection therapy 0.001). Prognosis influenced by sex 0.5). Patients had a life that different from expected an age- sex-matched normal population. Analysis 53 patients, population, showed cancer 219 times more frequent (16 patients), cardiomyopathy 306 (3 13 (10 7 patients). Death rates for other causes, including extrahepatic carcinomas (seven were expected. conclude precirrhotic stage treated have expectancy, whereas cirrhotic shortened high risk even when complete depletion has been achieved.

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