[Idiopathic hemochromatosis. Review of the literature and study of 17 patients].
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.
D000328:Adult; D000368:Aged; D001781:Blood Component Removal; D001815:Bloodletting; D003131:Combined Modality Therapy; D003676:Deferoxamine / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D006432:Hemochromatosis / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged
D003676:Deferoxamine
Hemocromatosis idiopática. Revisión de la literatura y estudio de 17 pacientes.