Nagel B H, Palmbach M, Petersen D, Ranke M B
Department of Paediatrics, University of Tübingen, Germany.
Eur J Pediatr. 1997 Oct;156(10):758-63. doi: 10.1007/s004310050707.
In order to validate an association between pituitary size and severity of growth hormone deficiency (GHD) we evaluated the magnetic resonance images (MRI) of 107 children with different causes of short stature. Ninety-one MRIs were evaluable (64 male, 27 female; age: 9.1 +/- 3.9 years). The levels of insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3), and tests of GH stimulation and spontaneous secretion, led to the following subgroups: severe isolated GHD (SIGHD) (GH < 7 ng/ml) (n = 21); partial, isolated GHD (GH 7-10 ng/ml) (n = 22); multiple pituitary hormone deficiency (MPHD) (n = 13); neurosecretory dysfunction (n = 10); non-classifiable diagnosis (NC) (n = 13); idiopathic short stature (n = 9); and intra-uterine growth retardation (n = 3). Pituitary height (PHT) was measured and hypoplasia was assumed when PHT was < -2 SDS. An ectopic posterior pituitary with missing stalk and a hypoplastic anterior pituitary was present in 12 (57%) SIGHD cases, 12 (92%) MPHD cases and 1 patient from the NC group. An isolated hypoplastic anterior pituitary was observed in 15%-33% of the other groups. PHT (mm; mean, SD) in MPHD (1.7 +/- 0.5) was lower than in SIGHD (2.7 +/- 1.0, P < 0.05), with PHT of both groups being lower than in all the other groups (3.8 +/- 0.9, P < 0.0001). PHT SDS correlates with IGF-I SDS (r = 0.48, P < 0.0001), IGFBP-3 SDS (r = 0.46, P < 0.0001) and the highest peaks in tests of GH stimulation and GH spontaneous secretion (r = 0.36, P < 0.0001). In contrast to all the other groups, no correlation with age was observed in MPHD and SIGHD. Breech delivery was recorded in up to 26% of patients in all seven groups. Surprisingly, only 1 out of 23 patients with an ectopic posterior pituitary was born by breech delivery, suggesting that ectopia of the posterior lobe is not necessarily related to breech delivery.
PHT is significantly correlated with GH secretion in several types of short stature. Patients with ectopic posterior pituitary, missing stalk and hypoplastic anterior pituitary either suffer from SIGHD or MPHD, and this anatomical defect is not necessarily related to breech delivery.
为验证垂体大小与生长激素缺乏症(GHD)严重程度之间的关联,我们评估了107例不同病因导致身材矮小儿童的磁共振成像(MRI)。91例MRI可评估(64例男性,27例女性;年龄:9.1±3.9岁)。胰岛素样生长因子-1(IGF-1)和胰岛素样生长因子结合蛋白-3(IGFBP-3)水平,以及生长激素刺激试验和自发分泌试验,产生了以下亚组:严重孤立性生长激素缺乏症(SIGHD)(生长激素<7 ng/ml)(n = 21);部分孤立性生长激素缺乏症(生长激素7-10 ng/ml)(n = 22);多种垂体激素缺乏症(MPHD)(n = 13);神经分泌功能障碍(n = 10);不可分类诊断(NC)(n = 13);特发性身材矮小(n = 9);以及宫内生长迟缓(n = 3)。测量垂体高度(PHT),当PHT<-2 SDS时假定为垂体发育不全。12例(57%)SIGHD病例、12例(92%)MPHD病例和1例NC组患者存在异位后叶且垂体柄缺失以及垂体前叶发育不全。在其他组中,15%-33%观察到孤立性垂体前叶发育不全。MPHD组的PHT(mm;均值,标准差)(1.7±0.5)低于SIGHD组(2.7±1.0,P<0.05),两组的PHT均低于所有其他组(3.8±0.9,P<0.0001)。PHT SDS与IGF-I SDS(r = 0.48,P<0.0001)、IGFBP-3 SDS(r = 0.46,P<0.0001)以及生长激素刺激试验和生长激素自发分泌试验中的最高峰值相关(r = 0.36,P<0.0001)。与所有其他组不同,MPHD和SIGHD组未观察到与年龄的相关性。所有七个组中高达26%的患者记录为臀位分娩。令人惊讶的是,23例异位后叶患者中只有1例为臀位分娩,提示后叶异位不一定与臀位分娩有关。
在几种类型的身材矮小中,PHT与生长激素分泌显著相关。存在异位后叶、垂体柄缺失和垂体前叶发育不全的患者患有SIGHD或MPHD,且这种解剖学缺陷不一定与臀位分娩有关。