This disease manifests at age 40 in men and later in women. Patients present with weakness, lethargy, arthralgia, impotence, diabetes, and dilated cardiomyopathy.Challenge: What's your diagnosis?
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4 comments:
Hemochromatosis!!
hemochromatosis
hemochromatosis
wow, nice turnout of responses :) i'm impressed...just some histology & radiology and you guys deduce it
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Bronze
This is hereditary hemochromatosis, an autosomal recessive disorder of increased intestinal iron absorption (which does not manifest until 20g iron accumulates in the body, and rate of accumulation is ~1g/year; women develop it later because of blood loss from menses). The CT shows high attenuation throughout the liver compared to the spleen (normally attenuation is similar) which is consistent with iron (or amiodarone) deposition. The first histology slide shows brown pigmentation of hepatocytes due to iron deposition (H&E stain), confirmed by the second histology image of Perls' Prussian blue stain. The title refers to the description "bronze diabetes" since many patients have skin discoloration and diabetes mellitus.
Source: UpToDate.
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