垂体柄阻断综合征MRI诊断对生长激素缺乏症患者临床处理的意义

Significance of MRI diagnosis in pituitary stalk interruption syndrome and management of patients with growth hormone deficiency

了解生长激素缺乏症（GHD）患者发生多垂体前叶激素缺乏与垂体柄阻断综合征（PSIS）的关系及其临床特征，探讨PSIS影像学诊断在GHD患者临床诊治过程中的指导意义。回顾分析近十年本院生长发育中心诊治的302例GHD患者各垂体前叶激素缺乏情况。按影像学诊断分为2组：66例PSIS组和236例非PSIS（对照）组，后天获得性病变（蝶鞍区肿瘤、炎症等）未纳入分析。2组均按年龄再分为大年龄组和小年龄组：男孩〉13岁、女孩〉12岁者为大年龄组，男孩年龄≤13岁，女孩≤12岁者为小年龄组。对小年龄组患者分析GHD缺乏程度和伴ACTH、促甲状腺素缺乏情况，大年龄组则同时判断有无促性腺激素缺乏/性发育延迟。 结果 生长激素激发峰值中位数PSIS组显著低于对照组，分别为0.37μg/L和5.01μg/L，差异有统计学意义（P＜0.01）。PSIS组2个年龄段的多垂体激素缺乏构成比均显著高于对照组：大年龄PSIS者为92.31%（36/39），非PSIS为35.14%（13/37），差异有统计学意义（χ2=27.10, P＜0.01）；小年龄PSIS者为66.67%（18/27），非PSIS为9.55%（19/199），差异有统计学意义（χ2=56.65, P＜0.01）。PSIS组中大年龄者并发肾上腺皮质功能低下者较小年龄者高（48.72%对22.22%, χ2=4.76, P＜0.05），而伴发甲状腺功能低下在大年龄者和小年龄者发生率相当（76.79%对66.67%, χ2=0.85, P〉0.05）。结论 与非PSIS相比，PSIS患者生长激素缺乏程度较为严重，多垂体激素缺乏发生率显著为高，合并肾上腺皮质功能低下的发生率随年龄增大而增加。对GHD患者，不论生长激素替代治疗与否，均应关注鞍区MRI有无PSIS表现。确诊为PSIS者，应详细评估和长期追踪复查生长激素以外的垂体前叶激素水平，尤其应及时发现大年龄组潜在的肾上腺皮质功能低下,以免因应激诱发肾上腺危象。

Objective To emphasize the significance of diagnosing pituitary stalk interruption syndrome（PSIS）by means of MRI in the management of patients with growth hormone deficiency（GHD）. Methods By reviewing the data of 66 GHD children with PSIS and 236 GHD children without PSIS in our hospital within the recent ten years, the function and secretion of anterior pituitary hormones in of the two groups were compared. Those with acquired pituitary diseases were not included in the study. Subjects were divided into two subgroups according to their age. Boys who were older than 13 years and girls older than 12 years were defined as old-age group, thyrotropin, ACTH, FSH, and LH were determined in this group. Those boys who were younger than 13 years and girls younger than 12 years were defined as youngage group, thyrotropin and ACTH were evaluated in this group. Results There was statistically significant difference between the medians of the growth hormone peak in the group with PSIS and the group without PSIS（ 0.37 vs 5.01 μg/L, P〈0.01）. The ratio of multiple pituitary hormone deficiency in GHD children with PSIS was significantly higher than that in GHD children without PSIS, being 92.31%（36/39） vs 35.14%（13/37）in the old-age group（χ2=27.10, P〈0.01）and 66.67%（18/27） vs 9.55%（19/199）in the young age group（χ2=56.65, P〈0.01）. In the PSIS group, 48.72% old age patients developed adrenal insufficiency, being much higher than that in the youngage（22.22%, χ2=4.76, P〈0.05）, hypothyroidism developed in 76.79% cases of the oldage and 66.67% in the youngage（χ2=0.85, P〉 0.05）. Conclusions The manifestation of GHD was much more severe in the patients with PSIS than those without PSIS. Other accompanied anterior pituitary hormone deficiencies were more prevalent in PSIS group than in nonPSIS group. The result suggested that all GHD patients with PSIS should be evaluated for the anterior pituitary functions and further following up is clinically important for the early diagnosis of the other anterior pituitary hormone deficiencies, especially for the investigation of adrenal insufficiency.