Lewis Anthony S, Courtney C Hamish, Atkinson A Brew
Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast BT12 6BA, UK.
Pituitary. 2009;12(3):273-5. doi: 10.1007/s11102-008-0090-x.
Hypopituitarism usually occurs as the result of a pituitary tumour or as a consequence of its treatment. If, however, pituitary imaging is negative then an alternative diagnosis should be sought. Patients are often diagnosed as having idiopathic hypopituitarism when imaging is normal. Our objective is to highlight the importance of screening for hemochromatosis in patients with presumed 'idiopathic' hypopituitarism. Our patients presented initially with biochemical hypopituitarism and, after initial investigation and normal imaging, were labelled as having idiopathic disease. They subsequently developed iron overload in cardiac and hepatic tissue respectively requiring regular venesection to deplete body stores. Genetic analysis revealed homozygosity for the C282Y mutation in our first patient thus explaining his more severe iron overload whereas our second case was a heterozygote for the same mutation, with iron overload confirmed on liver biopsy. We recommend that iron studies are performed in all patients who present with hypopituitarism and normal pituitary imaging. This may lead to reversal of the hypopituitarism and avoid development of any systemic consequences of hemochromatosis.
垂体功能减退通常是由垂体肿瘤或其治疗结果引起的。然而,如果垂体成像为阴性,则应寻求其他诊断。当成像正常时,患者通常被诊断为特发性垂体功能减退。我们的目的是强调对疑似“特发性”垂体功能减退患者进行血色素沉着症筛查的重要性。我们的患者最初表现为生化性垂体功能减退,经过初步检查和正常成像后,被标记为患有特发性疾病。他们随后分别在心脏和肝脏组织中出现铁过载,需要定期放血以耗尽体内储存的铁。基因分析显示我们的首例患者存在C282Y突变纯合子,从而解释了他更严重的铁过载,而我们的第二例患者是同一突变的杂合子,肝脏活检证实存在铁过载。我们建议对所有出现垂体功能减退且垂体成像正常的患者进行铁代谢研究。这可能会使垂体功能减退得到逆转,并避免血色素沉着症的任何全身性后果的发生。