Erfurth Eva Marie, Müller Hermann L
Department of Endocrinology, Skåne University Hospital, Institution of Clinical Sciences Lund University, Lund 22685, Sweden.
Department of Pediatrics and Pediatric Hematology/Oncology, University Children's Hospital, Carl von Ossietzky Universität, Klinikum Oldenburg AöR, Oldenburg 26133, Germany.
Best Pract Res Clin Endocrinol Metab. 2025 Apr 17:101999. doi: 10.1016/j.beem.2025.101999.
After diagnosis of craniopharyngioma, patients frequently develop a rapid weight gain leading to morbid hypothalamic obesity due to disease- and/or treatment-associated hypothalamic lesions. Hypothalamic obesity should be diagnosed and treated in the context of hypothalamic syndrome. Hypothalamic syndrome includes neuroendocrine deficiencies, disruption of circadian rhythm, disturbed hunger-satiety and thirst feelings, temperature dysregulation, and neurocognitive, sleep and psychosocial behavioral problems. Long-term prognosis is frequently impaired by increased risk for metabolic syndrome, cardiovascular problems, severe impairments of health-related quality of life, and premature mortality. Treatment of hypothalamic syndrome is challenging. Recently, an algorithm for personalized, risk-specific treatment of hypothalamic syndrome has been published. Dextro-amphetamines and other central stimulating agents as well as glucagon-like peptide-1 receptor (GLP-1R) agonists may cause weight loss. Bariatric surgery is effective. However, non-reversible procedures are controversial due to ethical and legal considerations in minors. Hypothalamus-sparing treatment strategies and research on novel therapeutic agents for hypothalamic syndrome are warranted.
颅咽管瘤确诊后,由于疾病和/或治疗相关的下丘脑病变,患者常迅速体重增加,导致病态下丘脑肥胖。下丘脑肥胖应在下丘脑综合征的背景下进行诊断和治疗。下丘脑综合征包括神经内分泌缺陷、昼夜节律紊乱、饥饿饱腹感和口渴感紊乱、体温调节异常以及神经认知、睡眠和社会心理行为问题。代谢综合征、心血管问题、健康相关生活质量严重受损以及过早死亡的风险增加,常常会影响长期预后。下丘脑综合征的治疗具有挑战性。最近,已发表了一种针对下丘脑综合征的个性化、风险特异性治疗算法。右旋苯丙胺和其他中枢兴奋剂以及胰高血糖素样肽-1受体(GLP-1R)激动剂可能会导致体重减轻。减肥手术是有效的。然而,由于未成年人的伦理和法律考量,不可逆手术存在争议。有必要采取保留下丘脑的治疗策略并开展针对下丘脑综合征的新型治疗药物研究。