Fischer R
Leber Magen Darm. 1976 Dec;6(6):316-29.
Idiopathic hemochromatosis is a genetical defect leading to continuous accumulation of iron. Symptomatology is caused by iron deposition in different organs. Phlebotomy is by far the most efficient therapy. Case reports are given of 51 cases in different stages of the disease, all of which were proved by biopsy. Taking into account data of these cases some aspects of heredity, history and clinical symptomatology of this metabolic disease are discussed; histological and peritoneoscopic findings during the course of the disease under management by phlebotomy are demonstrated. On 46 patients treated in this was 25 were freed of iron deposits and 5 improved. In most cases there was a reduction of collagenous tissue in the liver. In some, and even in far advanced cases, almost complete anatomical and clinical normalization could be reached. Prognosis depends upon early diagnosis and consequent long-term treatment by phlebotomy.
特发性血色素沉着症是一种导致铁持续蓄积的基因缺陷疾病。症状是由铁在不同器官沉积引起的。放血疗法是目前最有效的治疗方法。本文报告了51例处于疾病不同阶段的病例，所有病例均经活检证实。考虑到这些病例的数据，讨论了这种代谢性疾病的遗传、病史和临床症状的一些方面；展示了在放血治疗过程中疾病的组织学和腹腔镜检查结果。在接受这种治疗的46例患者中，25例铁沉积消失，5例病情改善。大多数病例肝脏中的胶原组织减少。在一些病例中，甚至在病情严重的晚期病例中，几乎可以实现完全的解剖学和临床正常化。预后取决于早期诊断以及随后长期的放血治疗。
