title: [Clinico-morphologic characteristics of primary hemochromatosis].
abstract: 12 autopsy cases of primary hemochromatosis were studied: 11 males (aged between 29 and 69 years, mean age 51 years) and 1 57-year-old female. Clinical course in 6 patients ran with predominant liver damage to the small nodule or was of biliary cirrhosis type, in 3 cases main manifestation was diabetes mellitus and in 3 cases cardiomyopathy. Generalized pattern of hemosiderine deposits with involvement of the liver, pancreas, heart, adrenals, pituitary, epiphysis, thyroid and other organs was established by the Pearl's test. Apart from pigment degeneration, atrophy and fibrosis, compensatory and regenerative changes were found. These changes should be taken into account when the disease is analyzed clinically and morphologically.
authors: St. Petersburg Medical Academy.
authors: A S
authors: AS
authors: Gordeladze
authors: O A
authors: OA
authors: Smirnov
authors: V G
authors: VG
authors: Radchenko
authors: G S
authors: GS
authors: Saburova
mesh_terms: D000328:Adult; D000368:Aged; D001284:Atrophy; D009202:Cardiomyopathies / Q000209:etiology / Q000473:pathology; D003920:Diabetes Mellitus / Q000209:etiology / Q000473:pathology; D005260:Female; D006432:Hemochromatosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D006485:Hemosiderin / Q000378:metabolism; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000209:etiology / Q000473:pathology; D008115:Liver Regeneration / Q000502:physiology; D008297:Male; D008875:Middle Aged
chemical_list: D006485:Hemosiderin
vernacular_title: Kliniko-morfologicheskaia kharakteristika pervichnogo gemokhromatoza.