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Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report

机译:颅咽管瘤手术后下丘脑肥胖和非酒精性脂肪肝引起的肝肺综合征一例

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摘要

Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD) and diabetes mellitus (DM). We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection. Two years later, the craniopharyngioma recurred, and she underwent a second resection. Three years after her second operation, she was diagnosed with type 2 DM, after which she did not visit the outpatient clinic for 2 years and then suddenly reappeared with a weight loss of 25.8 kg that had occurred over 21 months. One month later, she presented to the Emergency Department with dyspnea. Laboratory findings revealed liver dysfunction and hypoxia with increased alveolar artery oxygen gradient. Liver biopsy showed portal hypertension and micronodular cirrhosis. Echocardiography and a lung perfusion scan demonstrated a right to left shunt. She was finally diagnosed with hepatopulmonary syndrome and is currently awaiting a donor for liver transplantation. Patients surviving craniopharyngioma need to be followed up carefully to detect signs of hypothalamic obesity and monitored for the development of other comorbidities such as DM, NAFLD, and hepatopulmonary syndrome.
机译:下丘脑肥胖在颅咽管瘤患者中通常很复杂,这是由于肿瘤本身引起的下丘脑损害,治疗方式以及相关的多种垂体激素缺乏症。下丘脑肥胖会引起继发性疾病,例如非酒精性脂肪肝疾病(NAFLD)和糖尿病(DM)。我们报告了一名19岁的女性,她被诊断患有颅咽管瘤,肿瘤切除后发展为下丘脑肥胖,并发展为肝肺综合征。肿瘤切除一年后,她表现出NAFLD。两年后,颅咽管瘤复发,她进行了第二次切除。第二次手术三年后,她被诊断出患有2型糖尿病,此后两年没有去门诊就诊,然后突然重新出现,体重减轻了25.8公斤,发生了21个月。一个月后,她因呼吸困难向急诊科求诊。实验室检查结果显示,肝功能障碍和缺氧与肺泡动脉氧梯度升高有关。肝活检显示门脉高压和小结节性肝硬化。超声心动图和肺灌注扫描显示右向左分流。她最终被诊断出患有肝肺综合征,目前正在等待肝移植的捐助者。存活下来的颅咽管瘤患者需要仔细随访,以发现下丘脑肥胖的迹象,并监测其他合并症的发展,例如DM,NAFLD和肝肺综合征。

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