Dutta Pinaki, Bhansali Anil, Singh Paramjeet, Rajput Rajesh, Khandelwal Niranjan, Bhadada Sanjay
Department of Endocrinology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
J Pediatr Endocrinol Metab. 2009 Oct;22(10):921-8. doi: 10.1515/jpem.2009.22.10.921.
Non-tumoral causes constitute a major group of childhood hypopituitarism. The structural abnormalities of the stalk and the pituitary gland correlate with number and severity of concurrent hormone deficiencies. We describe clinico-radiological correlates in patients with non-tumoral hypopituitarism.
Thirty-one children (23 boys) with congenital hypopituitarism, aged 1-17 years, with a peak GH levels of < 7 ng/ml after two pharmacological stimuli (insulin induced hypoglycemia [IIH] and clonidine) were included in the study. MRI tetrad of absent or hypoplastic pituitary, empty sella, redundant or absent stalk and absent/ectopic posterior pituitary bright spot (EPPBS) was considered as a radiological diagnostic criterion and these abnormalities were correlated with number of hormone deficiencies, severity of GH deficiency and mode of presentation at the time of delivery.
Twenty (66%) children had vertex presentation, nine breech, and two children were delivered by lower segment Cesarean section (LSCS). Seven (78%) out of nine in the breech delivery group, 14 (70%) out of 20 in the vertex group and one out of two (50%) in the LSCS group had multiple pituitary hormone deficiencies (MPHD) (p = 0.665). Hypoplastic pituitary gland and posterior pituitary abnormalities were more frequent in patients with isolated growth hormone deficiency (IGHD) as opposed to MPHD (87.5% vs 65.2%, p = 0.08, 63% vs 47%, p = 0.64), whereas empty sella and stalk abnormalities were found more frequently in MPHD than in the IGHD group (76% vs 50%, p = 0.45 and 82.6% vs 37.5%, p = 0.01). Higher frequency of MR abnormalities was found in those with a peak GH response of < 3 ng/ml irrespective of the number of other pituitary hormone deficiencies (82.6% vs 37.5%, p = 0.02). Sixteen patients had MRI tetrad and it was more prevalent in the IGHD than in the MPHD group (75% and 44%, p = 0.01) and correlated with the severity of GH deficiency (r = 0.57, p = 0.01). The imaging abnormalities were also more prevalent in children with breech as compared to vertex presentation and correlated with severity of GH deficiency (100% vs 60%, p = 0.03, r = 0.52).
Imaging abnormalities are frequent in patients with non-tumoral hypopituitarism and correlate best with severity of GH deficiency rather than number of hormone deficiencies and breech presentation.
非肿瘤性病因是儿童垂体功能减退的主要病因组。垂体柄和垂体的结构异常与并发激素缺乏的数量和严重程度相关。我们描述了非肿瘤性垂体功能减退患者的临床-放射学相关性。
本研究纳入了31例年龄在1至17岁之间的先天性垂体功能减退儿童（23例男孩），在两次药物刺激（胰岛素诱发低血糖[IIH]和可乐定）后生长激素峰值水平<7 ng/ml。垂体缺如或发育不全、空蝶鞍、垂体柄冗长或缺如以及垂体后叶亮点缺如/异位（EPPBS）的MRI四联征被视为放射学诊断标准，这些异常与激素缺乏的数量、生长激素缺乏的严重程度以及分娩时的表现方式相关。
20例（66%）儿童为头先露，9例为臀先露，2例儿童通过下段剖宫产（LSCS）分娩。臀先露组9例中的7例（78%）、头先露组20例中的14例（70%）以及LSCS组2例中的1例（50%）患有多种垂体激素缺乏（MPHD）（p = 0.665）。与MPHD患者相比，孤立性生长激素缺乏（IGHD）患者中垂体发育不全和垂体后叶异常更为常见（87.5%对65.2%，p = 0.08，63%对47%，p = 0.64），而空蝶鞍和垂体柄异常在MPHD患者中比在IGHD组中更常见（76%对50%，p = 0.45以及82.6%对37.5%，p = 0.01）。无论其他垂体激素缺乏的数量如何，生长激素峰值反应<3 ng/ml的患者中MRI异常的频率更高（82.6%对37.5%，p = 0.02）。16例患者有MRI四联征，在IGHD患者中比在MPHD组中更普遍（75%和44%，p = 0.01），并且与生长激素缺乏的严重程度相关（r = 0.57，p = 0.01）。与头先露相比，臀先露儿童的影像学异常也更普遍，并且与生长激素缺乏的严重程度相关（100%对60%，p = 0.03，r = 0.52）。
非肿瘤性垂体功能减退患者中影像学异常很常见，并且与生长激素缺乏的严重程度而非激素缺乏的数量和臀先露最相关。
