[Clinic and therapy of idiopathic hemochromatosis. Report of 51 cases (author's transl)].
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.
D000328:Adult; D000368:Aged; D001815:Bloodletting; D002614:Chelating Agents / Q000627:therapeutic use; D005260:Female; D006432:Hemochromatosis / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007501:Iron / Q000032:analysis / Q000097:blood; D010535:Laparoscopy; D008099:Liver / Q000032:analysis / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012880:Skin Pigmentation
D002614:Chelating Agents; D007501:Iron
Klinik und Therapie der idiopathischen Hämochromatose. Erfahrungen bei 51 eigenen Fällen