Diabetic ketoacidosis and hypogonadotropic hypogonadism in association with transfusional hemochromatosis in a man with beta-thalassemia major

We report a 23-year-old man with P-thalassemia major and transfusional hemo chromatosis, which manifested as diabetic ketoacidosis and hypogonadotropic hypogonadism. This unusual presentation of diabetic ketoacidosis in hemoch romatosis has rarely been reported. Magnetic resonance imaging of the abdom en showed decreased signal intensity in the liver, spleen, and pancreas. In addition, the pituitary gland also showed heterogeneous low signal intensi ty, compatible with hemochromatosis. He was treated with insulin supplement s and pulsatile human chorionic gonadotropin administration. Clinical impro vement was noted after hormone replacement. Intensive iron chelation therap y was given to prevent cardiac complications, and to restore his gonadal fu nction. During follow-up, the patient experienced improvement in libido and sexual potency.