中华内分泌代谢杂志
中華內分泌代謝雜誌
중화내분비대사잡지
CHINESE JOURNAL OF ENDOCRINOLOGY AND METABOLISM
2008年
5期
483-485
,共3页
叶红英%李庆华%吴晞%杨叶虹%闻杰%鹿斌%周丽诺%李益明%周一呜%姚振威%朱禧星%胡仁明
葉紅英%李慶華%吳晞%楊葉虹%聞傑%鹿斌%週麗諾%李益明%週一嗚%姚振威%硃禧星%鬍仁明
협홍영%리경화%오희%양협홍%문걸%록빈%주려낙%리익명%주일오%요진위%주희성%호인명
垂体柄中断综合征%生长发育迟缓%垂体功能减退
垂體柄中斷綜閤徵%生長髮育遲緩%垂體功能減退
수체병중단종합정%생장발육지완%수체공능감퇴
Pituitary stalk interruption syndrome%Growth hormone deficiency%Hypopituitarism
目的 提高对垂体柄中断综合征(PSIS)的认识和诊断水平.方法 回顾分析5例PSIS患者的临床表现、垂体前叶及各靶腺轴和垂体后叶功能、影像学表现.结果 PSIS患者5例,其中男性4例,年龄17～21岁,3例有难产史,1例有头颅外伤史,均表现为不同程度的生长发育障碍,1例有畏寒、反应迟钝等甲状腺功能减退症状,均无多饮和低比重尿.所有患者存在生长激素缺乏、低促性腺激素性性腺功能减退和肾上腺皮质储备功能不足,4例患者同时存在中枢性甲状腺功能减退,3例临床并无相应表现.磁共振检查显示所有患者垂体柄和垂体后叶正常高信号消失,而在视交叉下(4例)、蝶窦内(1例)见到点状异常高信号,伴部分空蝶鞍.结论 PSIS以生长发育迟缓为主要临床表现,部分性或完全性垂体前叶功能减退而垂体后叶功能正常,磁共振检查呈现的特征性改变为重要诊断依据.
目的 提高對垂體柄中斷綜閤徵(PSIS)的認識和診斷水平.方法 迴顧分析5例PSIS患者的臨床錶現、垂體前葉及各靶腺軸和垂體後葉功能、影像學錶現.結果 PSIS患者5例,其中男性4例,年齡17～21歲,3例有難產史,1例有頭顱外傷史,均錶現為不同程度的生長髮育障礙,1例有畏寒、反應遲鈍等甲狀腺功能減退癥狀,均無多飲和低比重尿.所有患者存在生長激素缺乏、低促性腺激素性性腺功能減退和腎上腺皮質儲備功能不足,4例患者同時存在中樞性甲狀腺功能減退,3例臨床併無相應錶現.磁共振檢查顯示所有患者垂體柄和垂體後葉正常高信號消失,而在視交扠下(4例)、蝶竇內(1例)見到點狀異常高信號,伴部分空蝶鞍.結論 PSIS以生長髮育遲緩為主要臨床錶現,部分性或完全性垂體前葉功能減退而垂體後葉功能正常,磁共振檢查呈現的特徵性改變為重要診斷依據.
목적 제고대수체병중단종합정(PSIS)적인식화진단수평.방법 회고분석5례PSIS환자적림상표현、수체전협급각파선축화수체후협공능、영상학표현.결과 PSIS환자5례,기중남성4례,년령17～21세,3례유난산사,1례유두로외상사,균표현위불동정도적생장발육장애,1례유외한、반응지둔등갑상선공능감퇴증상,균무다음화저비중뇨.소유환자존재생장격소결핍、저촉성선격소성성선공능감퇴화신상선피질저비공능불족,4례환자동시존재중추성갑상선공능감퇴,3례림상병무상응표현.자공진검사현시소유환자수체병화수체후협정상고신호소실,이재시교차하(4례)、접두내(1례)견도점상이상고신호,반부분공접안.결론 PSIS이생장발육지완위주요림상표현,부분성혹완전성수체전협공능감퇴이수체후협공능정상,자공진검사정현적특정성개변위중요진단의거.
Objective To raise the level of clinical diagnosis for the patients with pituitary stalk interruption syndrome (PSIS). Methods Five patients (4 males) with PSIS were retrospectively analyzed with respects to the clinical features, endocrine status and image characteristics. Magnetic resonance imaging (MRI) scan was performed at hypothalamic-pituitary region. Results The clinical manifestations of all patients consisted of growth retardation and delayed puberty without polyuria. Deficiency of multiple anterior pituitary hormones was revealed in all patients by the evaluation of endocrine status. The features of MRI included a lack of visible pituitary stalk, absence of posterior lobe hypersignal in the sella turcica and a hyperintense spot in the region of the thalamus opticus. Conclusion The clinical characteristics of patients with PSIS are growth retardation and delayed puberty. The evaluation of anterior pituitary function is necessary. The detection of an anatomical abnormality around hypothalamic-pituitary region by MRI is important diagnostic evidence.
