[The clinical characteristics of the course of hemochromatosis]

Terapevticheskii Arkhiv
|January 1, 1991
PubMed

Eight patients with hemochromatosis (HC) were followed up. For a long time HC ran a latent course. Throughout many years, the symptoms occurred at varying succession. In many cases, the first manifest symptoms included skin itch, arthropathy, and diabetes mellitus. They may be dominant in the clinical picture for a long time, masking the genuine cause of the disease. The correct diagnosis was established, as a rule, at the pronounced stage of HC. Before that event the patients were followed up and treated by the endocrinologist (3 persons), by the infectionist (2 persons), dermatologist and traumatologist (2 persons), and by the internist (1 person). Appearance of one of the symptoms of the classical triad can be regarded as the onset of HC. The cardinal symptoms that determine the clinical picture progressed for the most part: liver cirrhosis, diabetes mellitus, and melanoderma. The first two require appropriate correction. Melanoderma presents a cosmetic and social and psychological problem. In HC patients, disorders occurring by the type of liver porphyria are recordable. They are of secondary nature, being an unfavourable prognostic sign. Investigation of iron metabolism in patients suffering from chronic liver diseases should be carried out by all means, since it can be regarded a specific enough test in the diagnosis of HC. Emphasis is laid on the importance of early diagnosis of HC.

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