Long term results of venesection therapy in idiopathic haemochromatosis.
Long term results of venesection therapy in idiopathic haemochromatosis.
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特发性血色素沉着病的静脉切开治疗的长期结果。
DOI：
10.1093/oxfordjournals.qjmed.a067484
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发表时间：
1976
期刊：
影响因子：
--
通讯作者：
Roger Williams
中科院分区：
文献类型：
--
作者：
A. Bomford;Roger Williams
在85例治疗的特发性血色病患者中，与26例未经治疗的患者相比，观察静脉注射治疗的临床效果，结果显示，在半数出现异常检查的患者中，色素沉着和肝肿大减少，肝功能恢复正常。28%的糖尿病患者病情得到控制，尽管有些患者在观察期间进行了静脉注射，但病情仍有所发展。门脉高压、睾丸萎缩和关节病未改善。只有12名患者在静脉注射后有足够的铁再积累，值得进一步治疗。这些患者的铁蓄积率在每天1-4 mg和4-8 mg之间变化，并且在再蓄积期的早期阶段，可螯合铁水平相对于身体铁储存不适当地高。寿命表数据显示，诊断后5年和10年的存活率，治疗患者分别为66%和32%，未治疗患者分别为18%和6%，两者的统计学差异非常显著（p <0-01）。可能的临床差异，如治疗组和未治疗组之间的就诊年龄、糖尿病、肝硬化、临床肝衰竭和肝癌的存在，否则可能有利于治疗组的加权生存期，通过使用协变量分析进行校正。这使得治疗和未治疗患者的平均对数生存值分别为4-15和2-88，相当于63-4个月和17-8个月，差异非常显著（p小于0-01）。10名患者在诊断时均患有肝硬化，在完成刺络疗法后3至15年死于恶性肝癌。在其他各种部位，肿瘤死亡率也很高----在受静脉感染的人群中为22%，明显高于使用全国癌症死亡率预测的类似年龄人口的死亡率。
Observations on the clinical effects of venesection therapy in 85 treated, as compared with 26 untreated, patients with idiopathic haemochromatosis showed decreased pigmentation and hepatomegaly together with a return to normal tests of liver function in half the patients who had abnormal tests at presentation. Control improved in 28 per cent of those patients with diabetes mellitus, although some patients developed it during the period of observation, despite venesection. Portal hypertension, testicular atrophy and arthropathy were not improved. In only 12 patients was there sufficient reaccumulation of iron after the initial course of venesection to merit further treatment. Rates of iron accumulation in these patients varied between 1-4 mg and 4-8 mg per day and chelatable iron levels were noted to be inappropriately high in relation to body iron stores during the early stages of the reaccumulation period. Life table data shows that the percentage survival five and ten years after diagnosis was 66 and 32 per cent respectively for the treated patients, and 18 and 6 per cent respectively for the untreated patients, both statistically highly significant differences (p less than 0-01). Possible clinical differences such as age of presentation, the presence of diabetes mellitus, cirrhosis, clinical hepatic failure and hepatoma between the treated and untreated groups that might otherwise have weighted survival in favour of the treated group were corrected by the use of covariant analysis. This gave mean log survival values of 4-15 and 2-88 for the treated and untreated patients respectively, equivalent to 63-4 months and 17-8 months, a highly significant difference (p less than 0-01). Ten patients, all of whom had cirrhosis at the time of diagnosis, died of malignant hepatoma between three and 15 years after completing venesection therapy. There was also a high rate of death from neoplasms in a variety of other sites--22 per cent in the venesected group, strikingly higher than that rate predicted for a similarly aged population using national cancer mortality rates.