CAJ | 학술논문

目的探讨垂体柄中断综合征(PSIS)的临床特点,以期提高对该病的认识.方法收集广州军区总医院内分泌科自2006年1月至2009年1月收治的9例PSIS患者,对其临床表现、影像学改变及内分泌系统异常进行分析总结.结果 (1)几乎全部患者MRI垂体柄缺如,垂体高度不超过3 mm,仅有一例外伤性病因患者为4.5 mm;(2)2例成年起病,有明显外伤史并以垂体危象入院;7例5～12岁发病,以生长发育异常为主因,诊断年龄17～28岁;(3)9例均有生长激素(GH)和垂体促性腺激素(GnH)显著缺乏,7例早年发病患者有6例出现继发性甲状腺和肾上腺皮质功能低下;(4)早年发病者均无垂体危象、无家族史和中膈视发育不良.结论 PSIS表现为垂体柄缺乏和腺垂体发育不良,以GH和GnH缺乏为主,多合并促肾上腺皮质激素和促甲状腺激素不同程度缺乏.
목적 탐토수체병중단종합정(PSIS)적림상특점,이기제고대해병적인식.방법 수집엄주군구총의원내분비과자2006년1월지2009년1월수치적9례PSIS환자,대기림상표현、영상학개변급내분비계통이상진행분석총결.결과 (1)궤호전부환자MRI수체병결여,수체고도불초과3 mm,부유일예외상성병인환자위4.5 mm;(2)2례성년기병,유명현외상사병이수체위상입원;7례5～12세발병,이생장발육이상위주인,진단년령17～28세;(3)9례균유생장격소(GH)화수체촉성선격소(GnH)현저결핍,7례조년발병환자유6례출현계발성갑상선화신상선피질공능저하;(4)조년발병자균무수체위상、무가족사화중격시발육불량.결론 PSIS표현위수체병결핍화선수체발육불량,이GH화GnH결핍위주,다합병촉신상선피질격소화촉갑상선격소불동정도결핍.
Objective To explore the clinical characteristics of pituitary stalk interruption syndrome (PSIS) to raise our awareness of this disease. Methods The clinical data, including clinical manifestations, MR image changes and disorders of endocrine system, of 9 patients admitted to our hospital were collected and analyzed. Results Eight of 9 patients showed absence of pituitary stalk under MRI with height of the pituitary no more than 3 mm; only one exceptional patient with traumatic etiology showed 4.5 mm of the pituitary. Two patients were adult-onset and clearly induced by head trauma, and both of them were hospitalized due to pituitary crisis; the other 7 patients, having the disease at the age of 5 to 12, were complained of growth and development retardation at the age of 17 to 28. All the patients were totally deficient in growth hormone (GH) and pituitary gonadotropin (GnH) secretion; in addition, secondary hypothyroidism and hypocortisolism occurred in 6 of the 7 young-onset patients. No consanguinity, sign of pituitary crisis, and septooptic dysplasia were noted in those young-onset patients.Conclusion PSIS is characterized by absence of pituitary stalk and pituitary hypoplasia, by GH and GnH deficiency, and mostly combined with ACTH and TSH deficiency of different extent.