Clinical, biochemical and histological features of primary haemochromatosis: a report of 67 cases

Autor: Dario Conte, Alberto Piperno, Clara Mandelli, Silvia Fargion, Marina Cesana, Lucia Brunelli, Luciana Ferrario, Pietro Velio, Maria Grazia Zaramella, Claudio Tiribelli, Gemino Fiorelli, Paolo A. Bianchi
Přispěvatelé: Conte, D, Piperno, A, Mandelli, C, Fargion, S, Cesana, M, Brunelli, L, Ferrario, L, Velio, P, Zaramella, Mg, Tiribelli, Claudio, Zaramella, M, Tiribelli, C
Rok vydání: 2008
Předmět:
Liver Cirrhosis
Male
HBsAg
Pathology
Cirrhosis
HLA-A3 Antigen
Gastroenterology
Liver Cirrhosis/complications
Bone Marrow
HLA Antigens
Hemochromatosis*/complications
Skin/metabolism
Medicine
HLA Antigen
Liver Neoplasms/complications
Skin
Mortality rate
Stomach
Liver Neoplasms
Hemochromatosis*/metabolism
Middle Aged
medicine.anatomical_structure
Liver Neoplasm
Hepatocellular carcinoma
Female
Hemochromatosis
Hepatocellular/complications
Human
Adult
medicine.medical_specialty
Carcinoma
Hepatocellular
HLA Antigens/analysis
Duodenum
Liver Cirrhosi
Iron
HLA-B7 Antigen
Internal medicine
Humans
Bone Marrow/metabolism
Carcinoma
Hepatocellular/complications
Duodenum/metabolism
Female
HLA Antigens/analysis
HLA-A3 Antigen
HLA-B7 Antigen
Hemochromatosis*/complications
Hemochromatosis*/metabolism
Hemochromatosis*/pathology
Humans
Iron/metabolism
Liver Cirrhosis/complications
Liver Neoplasms/complications
Male
Middle Aged
Skin/metabolism
Stomach/metabolism
Hemochromatosis*/pathology
Aged
Stomach/metabolism
Bone Marrow/metabolism
Hepatology
business.industry
Transferrin saturation
Carcinoma
Cancer
Duodenum/metabolism
medicine.disease
Gastric Mucosa
business
Iron/metabolism
Zdroj: Liver. 6:310-315
ISSN: 0106-9543
DOI: 10.1111/j.1600-0676.1986.tb00297.x
Popis: In 67 patients (mean age 51 years, range 26-79), at diagnosis of primary haemochromatosis (PH), grade III or IV liver iron overload was present in all cases, cirrhosis in 85%, transferrin saturation greater than 80% in 75%, serum ferritin greater than 1000 micrograms/l in 84%, and overt diabetes in 48%. Alcohol intake was greater than 150 g/day in 11 patients; six were chronic hepatitis B surface antigen (HBsAg) carriers. HLA-A3 and B7 antigens were present in 64% and 23% versus respectively 22% (p less than 0.01) and 9% (p less than 0.025) in controls. Iron overload was found in the stomach, duodenum, skin and bone marrow in 57, 43, 45 and 59% of the patients studied. Sixty-three patients were followed for 1-260 months (median 24); 43 received regular iron-depleting treatment and 20 did not because of liver failure, cancer or refusal. Cumulative survival was 79%, 67% and 61% at 1, 4 and 10 years, respectively. Ten patients died from hepatocellular carcinoma and two from extrahepatic cancer. The early high mortality rate was due to some cases of advanced disease or cancer. Cumulative survival in the regularly treated group was 95% at 1 year and 91% at 4 and 10 years, which was higher than in the untreated group.
Databáze: OpenAIRE
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