摘要
目的观察儿童颅咽管瘤(CP)术后致身材矮小者使用重组人生长激素(rhGH)治疗的疗效及安全性。方法纳入CP术后在复旦大学附属儿科医院内分泌遗传代谢科定期随访的患儿。分为rhGH治疗组和rhGH未治疗组。CP术后1～3个月病情稳定后首次随访患儿垂体功能,之后每3个月随访身高、体重、甲状腺功能和生长因子(IGF-1、IGF-BP3),比较两组治疗前后身高变化。每6～12个月随访头颅MRI,观察两组患儿CP复发及继发肿瘤发生情况。结果 CP术后患儿共18例,男、女各9例,均存在生长激素缺乏症(GHD)。rhGH治疗组和rhGH未治疗组分别为6和12例,平均手术年龄分别为(10.1±4.2)和(10.1±4.0)岁。16/18例(88.9%)存在垂体功能减低,其中12例(75.0%)伴甲状腺功能减低,9例(56.2%)伴中枢性尿崩症,4例(25.0%)伴性发育延迟,11例(68.8%)伴促肾上腺皮质激素下降。rhGH治疗组中2例单用rhGH治疗,4例同时使用左旋甲状腺素、醋酸去氨加压素和氢化可的松治疗,开始给予rhGH治疗的时间为术后(3.5±2.4)年,平均治疗时间为(2.6±2.2)年,治疗前身高增长速度(HV)为每年(3.1±1.0)cm,身高标准差(HTSDS)为(-2.63±0.93),至本文观察时点HV为每年(12.0±1.10)cm,HTSDS为(-0.21±1.39),生长因子水平较治疗前明显上升。rhGH未治疗组治疗前HV为每年(3.2±0.9)cm,HTSDS为(-2.44±0.62),至本文观察时点HV为每年(3.8±1.0)cm,HTSDS为(-3.76±0.97),生长因子水平治疗前后差异无统计学意义。两组随访头颅MRI均未见异常。结论儿童CP术后可出现多种内分泌激素异常,GH替代治疗可明显改善患儿身高,治疗期间未见原肿瘤复发及继发肿瘤发生。
Objective To observe the safety and effectiveness of recombinant human growth hormone （rhGH） replacement in postoperative craniopharyngioma （CP） children with short stature. Methods Children with pre-diagnosed CP and attending followed-up after surgery were recruited in Children＇s Hospital of Fudan University. A retrospective case-control study was conducted. Children were divided into rhGH treatment group and control group. They received first follow - up with 1 to 3 months after surgery and the pituitary hormone functions were measured. Body height, body weight, IGF-1, IGF-BP3 and thyroid function were measured every 3 months and the change in body height after treatment was compared between two groups. Head magnetic resonance（MR） was carried out every 6 to 12 months to observe the difference of the tumor recurrence and second malignant neoplasm between two groups. Results Eighteen postoperative CP children （9 girls and 9 boys） were diagnosed as growth hormone deficiency （GHD） and recruited. There were 6 patients and 12 patients in rhGH treatment group and the control group, respectively. The average age at surgery was （10.1 ± 4.2） years and （10.1 ± 4.0） years in treatment group and the control group, respectively. 16 out of 18 patients （88.9%） were with multiple pituitary hormone deficiency. Among the 16 patients, 12 of them （75%） also had hypothyroidism, 9 of them （56.2%） had central diabetes insipidus, 4 of them （25%） were with delayed puberty and 11 of them （68.8%） had adrenal insufficiency. In rhGH treatment group, 2 patients were treated by rhGH only, 4 patients were treated by rhGH combined with L-thyroxine, cortisone and Minirin. The average time of 6 patients starting rhGH replacement was （3.5 ± 2.4） years after surgery and the average duration of treatment was （ 2.6±2.2） years. Before treatment, the height velocity （HV） was （3.1 ±1.0） cm per year, body height standard deviation （HTSDS） was （ 2.63 ±0.93）, at the end ofobservation of this study, the HV was （ 12.0±1. 1 ） cm per year, HTSDS was （ 0.21 ± 1.39） , serum IGF-1 and IGF-BP3 levels were significantly elevated, lo control group, before treatment, the HV was （3.2 ± 0.9） cm per year, HTSDS was （ 2.44 ±0. 62）. At tile end of observation of this study, the ltV was 3.8± 1.0 cm per year, HTSDS was （ 3.76 ±0.97）, serum IGF-1 and IGF-BP3 levels were not significantly changed. Recurrent tumor or second malignant neoplasm was not found by head MR in both groups. Conclusions Multiple pituitary hormone deficiency could occur in postoperative craniopharyngioma children and rhGH replacement could improve body height. No recurrent tumor or second malignant neoplasm was found during treatment.
出处
《中国循证儿科杂志》
CSCD
2013年第3期201-204,共4页
Chinese Journal of Evidence Based Pediatrics
关键词
颅咽管瘤
重组人生长激素
安全性
Craniopharyngioma
Recombinant human growth hormone
Safety
