Wang Fengxue, Han Jinyan, Wang Zengmin, Shang Xiaohong, Li Guimei
Shandong Provincial Hospital affiliated to Shandong University, Department of Pediatrics, Shandong, China
J Clin Res Pediatr Endocrinol. 2020 Mar 19;12(1):71-78. doi: 10.4274/jcrpe.galenos.2019.2019.0086. Epub 2019 Sep 2.
The aim was to assess growth velocity (GV) during human recombinant growth hormone (hGH) treatment of children with multiple pituitary hormone deficiency (MPHD) caused by pituitary stalk interruption syndrome (PSIS) and to analyze the characteristics of patients that attained normal adult heights.
Data from 74 (16 female) children with MPHD caused by PSIS with GH, thyroid stimulating hormone, gonadotropin and adrenocorticotropic hormone deficiencies were collected. Subjects were divided into groups: 12 pre-pubescent females (Female-Group) and 36 pre-pubescent males (Male-Group 1). The remaining 22 males were further sub-divided into two groups (Male-Group 2 and Male-Group 3) according to the initiation of gonadotropin replacement treatment, based on bone age and height.
No differences in change in height standard deviation score (△HtSDS) and GV were observed at different time points of hGH treatment between the Female-Group and Male-Group 1 (p>0.05). GV was significantly greater in the first year of hGH therapy than in subsequent years: Female-Group p=0.011; Male-Group 1 p<0.001; Male-Group 2 p=0.005; and Male-Group 3 p=0.046. Adult height was achieved by 23 (19 males and 4 females) patients. The total gain in height positively correlated with the GV during the first year (r=0.626, p<0.001).
GV during hGH treatment were similar amongst pre-pubescent males and females with MPHD caused by PSIS. GV during the first year of hGH treatment appears to be an effective predictor of final height in patients with MPHD caused by PSIS.
评估垂体柄中断综合征（PSIS）所致多发性垂体激素缺乏症（MPHD）患儿在接受重组人生长激素（hGH）治疗期间的生长速度（GV），并分析达到正常成人身高的患者特征。
收集74例（16例女性）因PSIS导致MPHD且伴有生长激素、促甲状腺激素、促性腺激素和促肾上腺皮质激素缺乏的患儿数据。研究对象分为几组：12例青春期前女性（女性组）和36例青春期前男性（男性组1）。其余22例男性根据促性腺激素替代治疗开始时间，基于骨龄和身高进一步分为两组（男性组2和男性组3）。
女性组和男性组1在hGH治疗的不同时间点，身高标准差评分变化（△HtSDS）和GV无差异（p>0.05）。hGH治疗第一年的GV显著高于随后几年：女性组p=0.011；男性组1 p<0.001；男性组2 p=0.005；男性组3 p=0.046。23例（19例男性和4例女性）患者达到成人身高。身高总增长与第一年的GV呈正相关（r=0.626，p<0.001）。
PSIS所致MPHD的青春期前男性和女性在hGH治疗期间的GV相似。hGH治疗第一年的GV似乎是PSIS所致MPHD患者最终身高的有效预测指标。
