JIN-YING LUCHING-CHUNG CHANGTsai H.-C.Lin K.-S.Tsang Y.M.Huang K-.M.2020-08-122020-08-1220010929-6646https://www.scopus.com/inward/record.uri?eid=2-s2.0-0034821642&partnerID=40&md5=dc67074cdc3939c7acf01041c54ac7e2https://scholars.lib.ntu.edu.tw/handle/123456789/511454We report a 23-year-old man with β-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.diabetic ketoacidosis; Hypogonadotropic hypogonadism; Transfusional hemochromatosis[SDGs]SDG3chorionic gonadotropin; deferoxamine; glucose; gonadorelin; hemoglobin; insulin; iron; testosterone; abdomen; adult; article; beta thalassemia; case report; clinical feature; diabetes mellitus; diabetic ketoacidosis; disease association; drug pulse therapy; erythrocyte transfusion; follow up; gonad function; heart disease; hemochromatosis; hormone substitution; human; hypogonadotropic hypogonadism; hypophysis; iron chelation; libido; liver; male; nuclear magnetic resonance imaging; pancreas; sexual function; signal transduction; spleen; thalassemia major; Adult; beta-Thalassemia; Blood Transfusion; Diabetic Ketoacidosis; Hemochromatosis; Humans; Hypogonadism; Malejournal article115796172-s2.0-0034821642