Suárez J, Aliaga L, Sirvent L, Nunes R, Beloqui O, Prieto J
Rev Med Univ Navarra. 1987 Jan-Mar;31(1):15-21.
Idiopathic hemochromatosis is an inherited and primary disease. Its definition requires: a) a family history of abnormalities of iron storage; b) an association with HLA; c) inadequate iron absorption in relation to iron stores in liver and other territories; d) the absence of any other known causes of iron overload. The recent evidence of its high family related frequency and the existence of an effective treatment, demand the understanding of this entity and the strongest medical effort towards the diagnosis in the pre-cirrhotic stages. We present here the results from the study of 17 patients diagnosed of idiopathic hemochromatosis over a seven year period in the University Clinic of Navarra. Our aim is to review this subject and to compare our findings with those described in the current literature.
特发性血色素沉着症是一种遗传性原发性疾病。其定义要求：a) 有铁储存异常的家族病史；b) 与人类白细胞抗原（HLA）相关联；c) 相对于肝脏及其他部位的铁储存，铁吸收不足；d) 不存在任何其他已知的铁过载原因。其家族相关频率较高以及存在有效治疗方法的最新证据，要求了解这一疾病实体，并在肝硬化前期阶段尽最大医疗努力进行诊断。我们在此展示在纳瓦拉大学诊所七年间确诊的17例特发性血色素沉着症患者的研究结果。我们的目的是回顾这一主题，并将我们的研究结果与当前文献中所描述的结果进行比较。
