CAJ | 학술논문

目的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.方法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0.37 μg/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(x2=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(x2=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％,x2=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％,x2=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.
목적 료해생장격소결핍증(GHD)환자발생다수체전협격소결핍여수체병조단종합정(PSIS)적관계급기림상특정,탐토PSIS영상학진단재GHD환자림상진치과정중적지도의의.방법 회고분석근십년본원생장발육중심진치적302례GHD환자각수체전협격소결핍정황.안영상학진단분위2조:66례PSIS조화236례비PSIS(대조)조,후천획득성병변(접안구종류、염증등)미납입분석.2조균안년령재분위대년령조화소년령조:남해＞13세、녀해＞12세자위대년령조,남해년령≤13세,녀해≤12세자위소년령조.대소년령조환자분석GHD결핍정도화반ACTH、촉갑상선소결핍정황,대년령조칙동시판단유무촉성선격소결핍/성발육연지.결과 생장격소격발봉치중위수PSIS조현저저우대조조,분별위0.37 μg/L화5.01μg/L,차이유통계학의의(P＜0.01).PSIS조2개년령단적다수체격소결핍구성비균현저고우대조조:대년령PSIS자위92.31％ (36/39),비PSIS위35.14％(13/37),차이유통계학의의(x2=27.10,P＜0.01)；소년령PSIS자위66.67％ (18/27),비PSIS위9.55％ (19/199),차이유통계학의의(x2=56.65,P＜0.01).PSIS조중대년령자병발신상선피질공능저하자교소년령자고(48.72％대22.22％,x2=4.76,P＜0.05),이반발갑상선공능저하재대년령자화소년령자발생솔상당(76.79％대66.67％,x2=0.85,P＞0.05).결론 여비PSIS상비,PSIS환자생장격소결핍정도교위엄중,다수체격소결핍발생솔현저위고,합병신상선피질공능저하적발생솔수년령증대이증가.대GHD환자,불론생장격소체대치료여부,균응관주안구MRI유무PSIS표현.학진위PSIS자,응상세평고화장기추종복사생장격소이외적수체전협격소수평,우기응급시발현대년령조잠재적신상선피질공능저하,이면인응격유발신상선위상.
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 (x2 =27.10,P ＜ 0.01) and 66.67％ (18/27) vs 9.55 ％ (19/199) in the young-age group (x2 =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％,x2 =4.76,P＜0.05),hypothyroidism developed in 76.79％ cases of the old-age and 66.67％ in the young-age(x2 =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.