SURVIVAL AND PROGNOSTIC FACTORS IN 212 ITALIAN PATIENTS WITH GENETIC HEMOCHROMATOSIS
SURVIVAL AND PROGNOSTIC FACTORS IN 212 ITALIAN PATIENTS WITH GENETIC HEMOCHROMATOSIS
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DOI：
10.1002/hep.1840150417
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发表时间：
1992-04-01
期刊：
影响因子：
13.5
通讯作者：
CONTE, D
中科院分区：
文献类型：
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作者：
FARGION, S;MANDELLI, C;CONTE, D
对212例意大利遗传性血色病患者（181例男性，平均年龄50 ± 11岁; 31例女性，平均年龄49 ± 10岁）进行了中位44个月（范围= 3 ~ 218个月）的随访。31例受试者（15%）存在酒精滥用，145例受试者中分别有19例（9%）和35例（24%）慢性HBV和HCV感染。24例患者（11%）伴有β-地中海贫血性状。肝活检显示146例为肝硬化，其余66例为非肝硬化。Perls染色为III级37例，IV级171例。185例患者接受每周静脉注射，122例患者在总铁去除3至41克后实现铁耗竭。44例患者在3至198个月后死亡，其中20例死于肝细胞癌，10例死于肝功能衰竭，6例死于肝外癌，3例死于心力衰竭，5例死于与血色素沉着症无关的原因。癌症已经在其他七名仍然活着的患者中发展（五名肝细胞癌和两名肝外癌）。在非乳腺癌患者中未观察到死亡;乳腺癌患者3、5、8和10年的累积生存率分别为85%、75%、60%和47%。单因素分析显示年龄> 60岁、酗酒、心肌病、皮肤色素沉着、门脉高压、低白蛋白血症、高丙种球蛋白血症、Child分级B或C与预后有显著性负相关。在多变量分析中，只有酒精滥用、γ球蛋白大于2.0 gm/dl和Child分级B或C保持其负面预后值（分别为p < 0.01，风险比2.7; p < 0.001，风险比2.8;和p < 0.001，风险比4.3）。
Two hundred twelve Italian patients with genetic hemochromatosis (181 men, mean age 50 +/- 11 yr; and 31 women, mean age 49 +/- 10 yr) were followed for a median period of 44 mo (range = 3 to 218 mo). Alcohol abuse was present in 31 subjects (15%), and chronic HBV and HCV infection were seen in 19 (9%) and 35 (24%) of 145 cases tested, respectively. Twenty-four patients (11%) had concomitant beta-thalassemia trait. Liver biopsy revealed cirrhosis in 146 and a noncirrhotic pattern in the other 66. Perls' stain was degree III in 37 patients and IV in 171 patients. One hundred eighty-five patients underwent weekly venesection, and iron depletion was achieved in 122 cases after total iron removal of 3 to 41 gm. Death occurred in 44 patients after 3 to 198 mo and was due to hepatocellular carcinoma in 20 cases, liver failure in 10, extrahepatic cancer in six, heart failure in three and hemochromatosis unrelated causes in five. Cancer has developed in seven other patients still alive (hepatocellular in five and extrahepatic in two). No deaths were observed among noncirrhotic patients; cumulative survival rates in cirrhotic patients were 85%, 75%, 60% and 47% at 3, 5, 8 and 10 yr, respectively. Univariate analysis in the 146 cirrhotic patients showed that age greater than 60 yr, alcohol abuse, cardiomyopathy, skin pigmentation, portal hypertension, hypoalbuminemia, hypergammaglobulinemia and Child class B or C had significant negative prognostic value. At multivariate analysis, only alcohol abuse, gamma-globulins greater than 2.0 gm/dl and Child class B or C maintained their negative prognostic values (p < 0.01, hazard ratio 2.7; p < 0.001, hazard ratio 2.8; and p < 0.001, hazard ratio 4.3, respectively).