LONG-TERM SURVIVAL ANALYSIS IN HEREDITARY HEMOCHROMATOSIS
LONG-TERM SURVIVAL ANALYSIS IN HEREDITARY HEMOCHROMATOSIS
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DOI：
10.1016/0016-5085(91)90013-b
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发表时间：
1991-08-01
期刊：
影响因子：
29.4
通讯作者：
KERTESZ, AE
中科院分区：
文献类型：
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作者：
ADAMS, PC;SPEECHLEY, M;KERTESZ, AE
本研究调查了85例遗传性血色病患者的长期生存率。85例有记录的遗传性血色病诊断于1958年和1989年之间，并在大学医院（西安大略大学）医学中心进行了回顾性分析。通过访谈或由患者或家庭医生填写的书面问卷评估患者的当前状态。使用Kaplan-Meier寿命表和考克斯回归分析获得生存率差异的估计值。分析肝组织学、疾病的临床特征和静脉切除的数量，以确定它们与生存的关系。在平均8.1 ± 6.8年（范围：0-31年）的随访期间，85例血色病患者中有17例死亡。在诊断时患有肝硬化的患者死亡的可能性是非肝硬化患者的5.5倍。在诊断时非乳腺癌患者的估计生存率与正常人群中年龄和性别匹配的成员没有显著差异。在控制了肝硬化的数据后，糖尿病并没有增加死亡的风险。早期诊断和治疗血色素沉着症的前期阶段可以导致长期生存类似于一般人群。肝硬化的存在显著增加死亡率，是影响生存的主要临床因素。
This study investigated the long-term survival rates of 85 patients with hereditary hemochromatosis. Eighty-five patients with documented hereditary hemochromatosis diagnosed between 1958 and 1989 and followed up at the University Hospital (University of Western Ontario) medical center were retrospectively reviewed for this analysis. The current status of the patient was assessed by interview or written questionnaire completed by the patient or the family physician. Estimates of differences in survival rates were obtained using Kaplan-Meier life-table and Cox regression analysis. Liver histology, clinical features of the disease, and number of venesections were analyzed to determine their relationship to survival. In the course of a mean follow-up interval of 8.1 ± 6.8 years (range, 0–31 years), there were 17 deaths among the 85 hemochromatosis patients. Patients with cirrhosis at the time of diagnosis were 5.5 times more likely to die than noncirrhotic patients. Patients who were noncirrhotic at the time of diagnosis had an estimated survival that was not significantly different from age- and sex-matched members of the normal population. Diabetes did not increase the risk of death after data were controlled for the presence of cirrhosis. Early diagnosis and treatment of hemochromatosis in the precirrhotic stage can lead to long-term survival similar to that in the general population. The presence of cirrhosis significantly increases mortality and is the major clinical factor affecting survival.