■下丘脑-垂体轴功能障碍是术后颅咽管瘤(CP)患者的常见并发症,这极大地影响了这些患者的长期生活质量。为了更好地了解儿童CP患者术后下丘脑-垂体功能障碍和长期激素替代治疗的影响,我们评估了约200例儿童期发病的CP术后患者.
■回顾性检索2018年至2019年在北京儿童医院和北京天坛医院接受鞍区肿瘤切除术的儿童期发病CP患者的临床资料。对参与者进行随访,以评估术后长期激素替代疗法的效果并评估肿瘤复发率。
■入院年龄中位数为8.1(1.8,14.3)岁。头痛(45.5%),视力损害(39.5%),和恶心(33.0%)是最常见的临床表现。ACP占所有CP病例的95%。术后1周内中枢肾上腺功能不全和中枢甲状腺功能减退症的发生率分别为56.2%和70.3%,分别。同时,85.5%的患者需要至少一剂去氨加压素来控制尿量。总生存率和肿瘤复发率分别为98.6%和26.1%,分别,中位随访时间为29.7(19.0,40.3)个月。在后续期间,28.1%的患者符合身材矮小的诊断标准,54.4%符合肥胖标准。此外,94.4%的患者服用至少一种激素替代,74.7%服用三次或以上。左甲状腺素的患病率,糖皮质激素,去氨加压素,生长激素替代疗法为87.3%,77.5%,78.9%和31.0%,分别。接受左旋甲状腺素替代组合治疗的患者比例,氢化可的松,去氨加压素占54.9%。
■本研究是对儿童期发病CP患者的大样本系统术后内分泌功能评估。由于术后下丘脑-垂体功能障碍的患病率很高,CP患者通常需要长期的多种激素替代疗法.术后儿童期发病CP患者的个体化管理和准确的激素替代剂量具有重要意义。
Hypothalamic-pituitary axis dysfunction is a common complication in post-operative craniopharyngioma(CP) patients, and it greatly impacts the long-term quality of life of such patients. To better understand the effects of postoperative hypothalamic-pituitary dysfunction and long-term hormone replacement therapy in patients with childhood CP, we assessed approximately 200 patients with childhood-onset CP postoperatively.
Clinical details of patients with childhood-onset CP who underwent sellar tumor resection in Beijing Children\'s Hospital and Beijing Tiantan Hospital from 2018 to 2019 were retrieved retrospectively. The participants were followed up to assess the effects of post-operative long-term hormone replacement therapy and assess the tumor recurrence rate.
The median age of admission was 8.1 (1.8, 14.3) years. Headache (45.5%), visual impairment (39.5%), and nausea (33.0%) were the most common clinical manifestations. ACP accounted for 95% of all CP cases. The incidence of central adrenal insufficiency and central hypothyroidism within the first week after surgery was 56.2% and 70.3%, respectively. At the same time 85.5% of the patients required at least one dose of desmopressin to control urine output. Total survival and tumor recurrence rates were 98.6% and 26.1%, respectively, with a median follow-up time of 29.7 (19.0, 40.3) months. During the follow-up period, 28.1% patients met the diagnostic criteria for short stature, while 54.4% fit the criteria for obesity. In addition, 94.4% of the patients were taking at least one kind of hormone substitution, and 74.7% were taking three or more. The prevalence of levothyroxine, glucocorticoid, desmopressin, and growth hormone replacement therapy was 87.3%, 77.5%, 78.9% and 31.0%, respectively. The proportion of patients treated with the substitutive combination of levothyroxine, hydrocortisone, and desmopressin was 54.9%.
This study is a large-sample systematic postoperative endocrine function evaluation of patients with childhood-onset CP. Due to the high prevalence of post-operative hypothalamic-pituitary dysfunction, patients with CP usually require long-term multiple hormone substitution therapy. Individualized management and accurate hormone replacement dosage for postoperative childhood-onset CP patients are of great importance.