A Late Presentation of a Fatal Disease: Juvenile Hemochromatosis

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A Late Presentation of a Fatal Disease: Juvenile Hemochromatosis

Juvenile hemochromatosis is a rare and severe form of hereditary hemochromatosis. We report the case of a 39-year-old female who presented with heart failure and cirrhosis from previously unrecognized juvenile hemochromatosis. This is the latest presentation described in the literature. An important clue to the diagnosis was a history of amenorrhea since the age of 20 that had never been invest...

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A mouse model of juvenile hemochromatosis.

Hereditary hemochromatosis is an iron-overload disorder resulting from mutations in proteins presumed to be involved in the maintenance of iron homeostasis. Mutations in hemojuvelin (HJV) cause severe, early-onset juvenile hemochromatosis. The normal function of HJV is unknown. Juvenile hemochromatosis patients have decreased urinary levels of hepcidin, a peptide hormone that binds to the cellu...

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ژورنال

عنوان ژورنال: Case Reports in Medicine

سال: 2013

ISSN: 1687-9627,1687-9635

DOI: 10.1155/2013/875093