病例报告:颅咽管瘤手术后严格控制体重仍引发小儿肝肺综合征。

IF 3.9 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Frontiers in Endocrinology Pub Date : 2024-10-30 eCollection Date: 2024-01-01 DOI:10.3389/fendo.2024.1459451
Satoko Yoshikawa, Tomozumi Takatani, Rieko Takatani, Ayano Inui, Tomoo Fujisawa, Hiromichi Hamada
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引用次数: 0

摘要

儿童期发病的颅咽管瘤虽然罕见,但却是一种颅内畸形,可通过破坏下丘脑而导致肥胖,这种情况即使在肿瘤切除后也常常持续存在。这种严重的肥胖会增加儿童时期患糖尿病和脂肪肝的风险。与此同时,还可能出现泛垂体功能障碍,包括生长激素(GH)缺乏症。值得注意的是,一些生长激素缺乏症患者可能表现出正常的生长速度,因此无需为生长目的而进行生长激素治疗。然而,在这些情况下,GH疗法可能仍有益于预防非酒精性脂肪肝或非酒精性脂肪性肝炎的恶化。虽然体重管理历来被认为是预防肝硬化的金标准,但其有效性可能会受到下丘脑功能障碍和难以成功控制体重的限制。我们的病例研究重点介绍了一位虽然缺乏促生长激素但生长发育正常的患者,他没有接受促生长激素替代治疗,并继续与下丘脑肥胖症作斗争。尽管体重得到了有效控制,但患者还是患上了肝肺综合征,这表明仅仅依靠控制体重可能不足以预防肝脏并发症。这个病例强调了即使生长发育正常,也要解决GH缺乏问题的重要性。我们的研究结果表明,在这种情况下,GH替代疗法对预防肝硬化是有益的。
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Case report: Pediatric hepatopulmonary syndrome despite strict weight control after craniopharyngioma surgery.

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.

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来源期刊
Frontiers in Endocrinology
Frontiers in Endocrinology Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
5.70
自引率
9.60%
发文量
3023
审稿时长
14 weeks
期刊介绍: Frontiers in Endocrinology is a field journal of the "Frontiers in" journal series. In today’s world, endocrinology is becoming increasingly important as it underlies many of the challenges societies face - from obesity and diabetes to reproduction, population control and aging. Endocrinology covers a broad field from basic molecular and cellular communication through to clinical care and some of the most crucial public health issues. The journal, thus, welcomes outstanding contributions in any domain of endocrinology. Frontiers in Endocrinology publishes articles on the most outstanding discoveries across a wide research spectrum of Endocrinology. The mission of Frontiers in Endocrinology is to bring all relevant Endocrinology areas together on a single platform.
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