Tadokoro Tomoko, Tani Joji, Sato Yudai, Yano Rie, Takuma Kei, Nakahara Mai, Oura Kyoko, Fujita Koji, Ono Masafumi, Tobiume Atsushi, Sato Seisuke, Inoue Takuya, Morishita Asahiro, Kobara Hideki
Department of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Japan.
Department of Cardiorenal and Cerebrovascular Medicine, Faculty of Medicine, Kagawa University, Japan.
Intern Med. 2025 Aug 1;64(15):2307-2311. doi: 10.2169/internalmedicine.4753-24. Epub 2025 Jan 3.
A 32-year-old man presented with cirrhosis. At 8 years of age, he underwent resection of a craniopharyngioma, which resulted in panhypopituitarism. He underwent self-interrupted hormone replacement therapy at 20 years of age. Computed tomography revealed severe fatty liver and cirrhosis. An endocrinological evaluation revealed panhypopituitarism. Further assessment revealed a diagnosis of hepatopulmonary syndrome. Home oxygen therapy and hormone replacement therapy were initiated. Despite these efforts, poorly controlled hypothalamic obesity led to liver failure, and the patient is currently awaiting liver transplantation. Liver cirrhosis associated with long-term panhypopituitarism may have a poor prognosis even with hormone replacement therapy.
一名32岁男性因肝硬化就诊。他8岁时接受了颅咽管瘤切除术，术后发生全垂体功能减退。20岁时他自行中断了激素替代治疗。计算机断层扫描显示严重脂肪肝和肝硬化。内分泌评估显示全垂体功能减退。进一步评估诊断为肝肺综合征。开始进行家庭氧疗和激素替代治疗。尽管采取了这些措施，但下丘脑性肥胖控制不佳导致肝功能衰竭，该患者目前正在等待肝移植。即使进行激素替代治疗，长期全垂体功能减退相关的肝硬化预后可能也较差。
