title: Diabetic ketoacidosis and hypogonadotropic hypogonadism in association with transfusional hemochromatosis in a man with beta-thalassemia major.
abstract: We report a 23-year-old man with beta-thalassemia major and transfusional hemochromatosis, which manifested as diabetic ketoacidosis and hypogonadotropic hypogonadism. This unusual presentation of diabetic ketoacidosis in hemochromatosis has rarely been reported. Magnetic resonance imaging of the abdomen showed decreased signal intensity in the liver, spleen, and pancreas. In addition, the pituitary gland also showed heterogeneous low signal intensity, compatible with hemochromatosis. He was treated with insulin supplements and pulsatile human chorionic gonadotropin administration. Clinical improvement was noted after hormone replacement. Intensive iron chelation therapy was given to prevent cardiac complications, and to restore his gonadal function. During follow-up, the patient experienced improvement in libido and sexual potency.
authors.affiliation: Division of Endocrinology, Department of Medicine, National Taiwan University College of Medicine, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: J Y
authors.initials: JY
authors.lastname: Lu
authors.forename: C C
authors.initials: CC
authors.lastname: Chang
authors.forename: H C
authors.initials: HC
authors.lastname: Tsai
authors.forename: K S
authors.initials: KS
authors.lastname: Lin
authors.forename: Y M
authors.initials: YM
authors.lastname: Tsang
authors.forename: K M
authors.initials: KM
authors.lastname: Huang
mesh_terms: D000328:Adult; D016883:Diabetic Ketoacidosis / Q000209:etiology*; D006432:Hemochromatosis / Q000150:complications*; D006801:Humans; D007006:Hypogonadism / Q000209:etiology*; D008297:Male; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000150:complications* / Q000628:therapy