Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan.
Faculty of Education, Graduate School of Education, Chiba University, Chiba, Japan.
Front Endocrinol (Lausanne). 2024 Oct 30;15:1459451. doi: 10.3389/fendo.2024.1459451. eCollection 2024.
Childhood-onset craniopharyngiomas, though rare, are intracranial malformations that can cause obesity by disrupting the hypothalamus, a condition that often persists even after tumor resection. This severe obesity increases the risk of diabetes and fatty liver disease in childhood. Concurrently, panhypopituitarism, including growth hormone (GH) deficiency, may develop. Notably, some individuals with GH deficiency may exhibit a normal growth rate, making GH therapy unnecessary for growth purposes. However, in these cases, GH therapy may still be beneficial in preventing the progression of nonalcoholic fatty liver disease or nonalcoholic steatohepatitis. Although weight management is traditionally considered the gold standard for preventing liver cirrhosis, its effectiveness can be limited by hypothalamic dysfunction and the difficulty of achieving successful weight control. Our case study highlights a patient with normal growth despite GH deficiency, who did not receive GH replacement therapy and continued to struggle with hypothalamic obesity. Despite effective body weight control, the patient developed hepatopulmonary syndrome, indicating that relying solely on weight management may not be sufficient to prevent liver complications. This case underscores the importance of addressing GH deficiency even when growth is normal. Our findings suggest that GH replacement therapy could be beneficial for preventing liver cirrhosis in such cases.
儿童期颅咽管瘤虽然罕见，但属于颅内畸形，可通过破坏下丘脑导致肥胖，即使肿瘤切除后，这种肥胖状况往往仍会持续。这种严重的肥胖增加了儿童期糖尿病和脂肪肝疾病的风险。同时，可能会并发垂体功能减退症，包括生长激素（GH）缺乏症。值得注意的是，一些 GH 缺乏症患者可能表现出正常的生长速度，因此 GH 治疗对于生长目的而言并非必需。然而，在这些情况下，GH 治疗仍可能有益于预防非酒精性脂肪性肝病或非酒精性脂肪性肝炎的进展。尽管传统上认为体重管理是预防肝硬化的金标准，但由于下丘脑功能障碍和成功控制体重的困难，其效果可能会受到限制。我们的病例研究强调了一名尽管存在 GH 缺乏症但生长正常的患者，该患者未接受 GH 替代治疗，并继续受到下丘脑性肥胖的困扰。尽管体重得到了有效控制，但该患者仍发展为肝肺综合征，这表明仅依靠体重管理可能不足以预防肝脏并发症。该病例强调了即使生长正常也应解决 GH 缺乏症的重要性。我们的研究结果表明，在这种情况下，GH 替代治疗可能有益于预防肝硬化。
