Growth and Adult Height during Human Growth Hormone Treatment in Chinese Children with Multiple Pituitary Hormone Deficiency Caused by Pituitary Stalk Interruption Syndrome: A Single Centre Study

Abstract

Objective: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. Methods: 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. Results: 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). Conclusion: 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. Abstract 71 J Clin Res Pediatr Endocrinol 2020;12(1): [71][72][73][74][75][76][77][78] What is already known on this topic? What this study adds?Pituitary stalk interruption syndrome (PSIS) is a kind of congenital disease associated with multiple pituitary hormone deficiency (MPHD). Human recombinant growth hormone (hGH) treatment is the optimal therapy for short stature in children with isolated GH deficiency and can effectively increase growth velocity (GV) to attain adult heights within the target range.GVs during hGH treatment were similar amongst pre-pubescent males and females with MPHD caused by PSIS. The GV during the first year of hGH treatment is an effective predictor of future height outcomes in patients with MPHD caused by PSIS.

Short Stature: Think About the Pituitary Stalk Interruption Syndrome

“…Numerous reports have shown that there are many perinatal adverse events in PSIS patients, such as breech delivery, dystocia, and hypoxia [ 8 , 9 ]. Wang et al studied 59 cases of children with PSIS, of which 54 cases had breech deliveries [ 10 ]. Fukuta et al have supported the idea that breech presentation is a risk factor for pituitary stalk transection syndrome [ 11 ].…”

“…Better prognosis and quality of life are linked to fast identification of hormonal deficiency with quick treatment initiation [ 14 ]. Wang et al described in the literature the utility of increased first-year growth velocity as an effective predictor of final height in patients with PSIS [ 10 ].…”