Asunto(s)
Fístula Pancreática/complicaciones , Pancreatitis Crónica/complicaciones , Pleuresia/etiología , Colangiopancreatografia Retrógrada Endoscópica , Estudios de Seguimiento , Humanos , Laparotomía/métodos , Masculino , Persona de Mediana Edad , Pancreatectomía/métodos , Fístula Pancreática/diagnóstico , Fístula Pancreática/cirugía , Pancreatitis Crónica/diagnóstico , Pancreatitis Crónica/cirugía , Enfermedades Pleurales/diagnóstico , Enfermedades Pleurales/etiología , Enfermedades Pleurales/cirugía , Pleuresia/diagnóstico , Pleuresia/cirugía , RecurrenciaRESUMEN
Liver pathology was studied in 3 patients with primary chemochromatosis. In two cases so-called iron free foci with signs of hepatocytes with feature of dysplasia were found. Many siderosomes were found ultrastructurally in the cytoplasma of hepatocytes. Histological markers of virus infection were absent in a patient with positive serum HbsAg and HCV-Ab. Alcohol did not produce typical histological changes. In this case grave liver reticuloendothelial hemosiderosis typical for secondary hemochromatosis and overloading with iron of spleen pulp according to MR imaging were observed.
Asunto(s)
Hemocromatosis/patología , Antígenos de Histocompatibilidad Clase I/genética , Hígado/patología , Proteínas de la Membrana/genética , Mutación , Marcadores Genéticos/genética , Hemocromatosis/genética , Proteína de la Hemocromatosis , Homocigoto , Humanos , Inmunohistoquímica , Hígado/ultraestructura , Imagen por Resonancia Magnética , Microscopía Electrónica , Persona de Mediana EdadAsunto(s)
Citaféresis , Hemocromatosis/diagnóstico , Hemocromatosis/terapia , Hígado/patología , Imagen por Resonancia Magnética , Biopsia con Aguja , Estudios de Evaluación como Asunto , Ferritinas/sangre , Estudios de Seguimiento , Hemocromatosis/patología , Humanos , Hierro/sangre , Masculino , Persona de Mediana Edad , Factores de TiempoRESUMEN
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.