Lo Fu-Sung, Chang Luan-Yin, Yang Min-Hai, Van Yang-Hau, Wai Yau-Yau
Department of Pediatrics, National Taiwan University Hospital, Taiwan.
J Pediatr Endocrinol Metab. 2004 Nov;17(11):1519-26. doi: 10.1515/jpem.2004.17.11.1519.
Growth hormone deficiency (GHD) may be classified into partial isolated GHD (partial IGHD), severe IGHD or multiple pituitary hormone deficiency (MPHD) by the severity of GHD or associated with deficiency of one or more other anterior pituitary hormones during provocative tests. Morphological alterations on magnetic resonance imaging (MRI) in patients with GHD include pituitary hypoplasia, absence or interruption of pituitary stalk, and absence or ectopic posterior lobe. This study investigated the auxological, clinical severity, and anatomical characteristics of the pituitary hypothalamic region by MRI and correlated their relationships. We evaluated these parameters in 45 Taiwanese children with GHD (31 males and 14 females), aged from 3.13 to 17.91 years (10.5+/-2.5), who were divided into diagnostic subgroups of partial IGHD (18 patients), severe IGHD (13 patients), and MPHD (14 patients). We found that BA-CA, peak GH, IGF-I, IGF-I SDS, and height SDS were significantly different among these three groups. The partial IGHD group had significantly higher IGF-I than the MPHD group. There was no significant difference in prematurity, cesarean delivery, birth order, neonatal jaundice, and target height among these three groups. On MRI, patients with MPHD had significantly smaller pituitary height (PHt) SDS (p = 0.0012) and higher frequency of pituitary hypoplasia, pituitary stalk interruption, and ectopic posterior lobe (p = 0.026, 0.008, 0.005, respectively) than the other two groups. Furthermore, PHt SDS was correlated not only with peak GH (r = 0.40, p = 0.0058), but also with basal IGF-I SDS (r = 0.49, p = 0.0007) and body height SDS (r = 0.44, p = 0.025). In conclusion, morphological alterations on MRI of the hypothalamic-pituitary area are correlated with the severity of hypopituitarism. Meticulous evaluation of auxological, clinical and MRI findings can help evaluation of the severity of hypopituitarism and facilitate appropriate treatment in children with GHD.
生长激素缺乏症（GHD）可根据GHD的严重程度分为部分性孤立性GHD（部分性IGHD）、严重IGHD或多种垂体激素缺乏症（MPHD），或在激发试验期间与一种或多种其他垂体前叶激素缺乏相关。GHD患者磁共振成像（MRI）的形态学改变包括垂体发育不全、垂体柄缺如或中断以及后叶缺如或异位。本研究通过MRI调查了垂体下丘脑区域的体格学、临床严重程度和解剖学特征，并关联了它们之间的关系。我们评估了45名台湾GHD儿童（31名男性和14名女性）的这些参数，年龄为3.13至17.91岁（10.5±2.5岁），他们被分为部分性IGHD（18例患者）、严重IGHD（13例患者）和MPHD（14例患者）诊断亚组。我们发现，这三组之间的骨龄-年龄、生长激素峰值、胰岛素样生长因子-I（IGF-I）、IGF-I标准差分值（SDS）和身高SDS有显著差异。部分性IGHD组的IGF-I显著高于MPHD组。这三组在早产、剖宫产、出生顺序、新生儿黄疸和靶身高方面无显著差异。在MRI上，MPHD患者的垂体高度（PHt）SDS显著更小（p = 0.0012），垂体发育不全、垂体柄中断和后叶异位的发生率更高（分别为p = 0.026、0.008、0.005），高于其他两组。此外，PHt SDS不仅与生长激素峰值相关（r = 0.40，p = 0.0058），还与基础IGF-I SDS（r = 0.49，p = 0.0007）和身高SDS（r = 0.44，p = 0.025）相关。总之，下丘脑-垂体区域MRI的形态学改变与垂体功能减退的严重程度相关。对体格学、临床和MRI结果进行细致评估有助于评估垂体功能减退的严重程度，并促进GHD儿童的适当治疗。
