Endocrine Abstracts (2006) 11 P292

Glucose metabolism, insulin secretion and insulin sensitivity in juvenile hemochromatosis

N Angelopoulos1, M Noutsou2, A Goula1 & G Tolis1


1Hippocrateio Hospital,Department of Endocrinology, Athens, Greece; 2Hippocrateio Hospital,Department of Diabetes, Athens, Greece.


Among specific diabetes subtypes secondary to pancreatopathies, hereditary hemochromatosis is an inherited disorder of iron metabolism, resulting to excessive iron overload and tissue damage in various organs. We report here the case of a man with the young-onset form of the disease and we describe his glycaemic status, before and during venesection therapy. A 25-year-old man attended our clinic in Athens, Greece, with hypogonadotropic hypogonadism due to hereditary hemochromatosis. Genetic analysis revealed that he was suffered from juvenile aggressive form, and treatment initiated with frequent phlebotomies, among with androgen substitution. Within 18 months of therapy ferritin level was normalized and hypogonadism was fully restored. Despite severe iron overload glucose tolerance remained normal during the various stages of the disease, although alterations in both insulin secretion and sensitivity were detected. In conclusion, glucose metabolism in juvenile hemochromatosis is a unique clinical entity and mainly depends on the efficacy of chelation therapy and the duration that patient remains under the iron-overload stage.

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