无功能性垂体腺瘤围手术期内分泌水平的变化及诊断进展

无功能性垂体腺瘤起源于垂体前叶上皮细胞,约占垂体腺瘤的1/3,大多数患者临床表现为头痛、视物模糊。但是部分患者术前及术后出现垂体功能减退。肿瘤对门静脉及垂体柄的压迫导致垂体前叶缺血坏死是引起垂体功能低下的主要机制,术中操作、肿瘤大小及残留都可导致新发垂体功能低下。术前主要以生长激素缺乏为主,而术后出现各个激素轴的改变。垂体功能减退容易被忽视,仅靠激素基础值的检测诊断远远不够,必要时需行刺激试验确诊。一旦明确垂体功能减退,充足的激素替代治疗能够提高患者的生活质量。     

原发性高钠血症的临床病例分析

为探讨原发性高钠血症的治疗方案,对4例原发性高钠血症患者分别采用两种不同方案治疗.2例先给予垂体前叶激素的治疗,再给予垂体后叶激素补充治疗,另2例同时给予垂体前叶和后叶激素治疗,结果发现:4例患者治疗后临床症状及血渗透压均正常,使用垂体前叶和后叶激素同时治疗的患者治疗时间缩短,而且治疗方案安全.     

垂体柄中断综合征五例分析并文献复习

目的 分析垂体柄中断综合征(pituitary stalk interruption syndrome,PSIS)患者的临床特点提高对该类疾病的临床认识.方法 采用回顾性分析的方法,对我院近7年来收治的5例PSIS患者的临床表现、实验室检查和影像学特点进行分析.结果 5例PSIS患者中男性4例,女性1例.临床特征是:(1)生长发育迟缓;(2)出生时臀位分娩率高;(3)身高159～165 cm,下部量大于上部量,指间距小于身长;(4)骨龄落后3～8岁不等;(5)智力基本正常,但学习成绩逐渐下降;(6)有垂体前叶功能减退的临床表现 和实验室检查;(7)垂体后叶功能正常;(8)垂体MRI增强扫描:垂体柄未见,垂体小,后叶异位.PSIS的发病机制不详.垂体前叶激素替代治疗是唯一有效的疗方式,但要把握治疗时机.结论 PSIS发病率低临床特征不同于单纯性垂体侏儒和低促性腺激素性性腺功能减退,临床应提高认识,把握治疗时机.     

五例垂体柄中断综合征的临床诊断

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.     

垂体腺瘤与女性生育功能

育龄期女性垂体腺瘤的发生率越来越高,由于垂体的重要位置和功能,垂体腺瘤与育龄期女性的生育功能有密切关系.性腺功能的破坏、高催乳素血症及腺体本身的高分泌状态是各类型垂体腺瘤影响生育功能的共同机制.另外,垂体腺瘤本身或是手术治疗后引起垂体前叶激素缺乏也可影响生育功能.对有生育要求的育龄期垂体腺瘤女性,选择合适的治疗方法以及对垂体前叶激素缺乏患者早期正确替代治疗,对恢复生育功能有重要意义.     

垂体柄阻断综合征合并肝硬化1例报告

正垂体柄阻断综合征(pituitary stalk interruption syndrome,PSIS)是垂体柄缺如或变细并出现神经垂体异位,下丘脑分泌的激素无法通过垂体柄输送至垂体后叶所致的临床系列综合征[1]。PSIS临床少见,主要临床表现为生长激素缺乏导致的生长迟缓。目前为止尚未见PSIS引起肝硬化的病例报道。现报道1例以"呕血、黑便"入院,查体见发育迟缓、肝脾肿大,结合血液检验、影像学、肝穿病理等检查及既往史诊断为PSIS合     

老年垂体前叶功能减退症促性腺激素的变化及意义

目的检测老年垂体前叶功能减退症患者促性腺激素的变化,探讨促性腺激素水平在老年垂体前叶功能减退症患者的变化及诊断意义。方法对2005年6月至2010年2月解放军总医院内分泌科确诊的29例60岁以上无明确诱因首次诊断为垂体前叶功能减退症患者的临床表现、垂体相关激素指标进行回顾性分析。结果 (1)促性腺激素:黄体生成素(LH)低于正常值28例,占96.6%,卵泡刺激素(FSH)低于正常值27例,占93.1%;(2)促肾上腺皮质激素(ACTH)低于正常值27例,占93.1%;(3)促甲状腺激素(TSH)低于正常值18例,占62.1%。结论老年垂体前叶功能减退症患者促性腺激素水平降低多见,较其他垂体激素降低出现早,发生频率高,可作为老年垂体前叶功能减退症早期断的依据。     

生长激素缺乏但持续增高:垂体柄中断合并垂体多激素缺乏一例报告及文献复习

报道一例垂体柄中断合并垂体多激素缺乏、包括经低血糖刺激试验发现生长激素缺乏、但表现为持续增高的病例.结合文献复习发现,此种非生长激素依赖的身高生长一般有垂体其他激素尤其是促性腺激素缺乏、骨骺未闭合为基础条件.     

中华医学会第六次全国内分泌学术会议纪要(第二部分)

(续第 18卷第 1期第 83页 )四、下丘脑及垂体1.基础研究 :第四军医大学神经科学研究所鞠躬教授作了关于“垂体前叶的直接神经调节作用”的专题报告。近 10年的研究发现 ,哺乳动物垂体前叶激素的分泌除了受下丘脑激素经门脉系统的循环调节以外 ,在垂体前叶存在神经系统的直接调节作用。其主要依据 :(1)在垂体前叶发现有神经纤维 (人、猴、狗及大鼠 ) ;(2 )在神经纤维与垂体腺细胞之间形成突触 ;(3)血浆激素水平的改变可以诱导神经活性的改变 ;(4)刺激神经纤维可以调节垂体前叶激素的释放。北京协和医院的“垂体特异性转录因子Ｐｉｔ 1与人Ｂ…     

丘脑下部对内分泌的作用

丘脑下部是目前激素研究最活跃的领域之一。丘脑下部具有神经分泌作用,所分泌的丘脑下部激素控制垂体,转而控制全身内分泌。丘脑下部对垂体前叶的控制是神经化学性的。丘脑下部神经分泌细胞能分泌若干种激素物质—释放因子或抑制因子,经短程血运运载到前叶,控制前叶激素的合成和分泌。垂体后叶自身没有制造激素的能力,它所分泌的激素     

Spontaneous regression of anterior pituitary deficits in a woman harboring a persistent hypothalamic mass

Sellar or parasellar lesions can cause anterior pituitary dysfunction either by direct damage to the anterior pituitary gland or by compression of the pituitary stalk and mediobasal hypothalamus which contain the hypophysiotropic hormones. Without treatment, the pituitary deficits in such cases are not likely to improve. We describe a case in which deficits in anterior pituitary hormones spontaneously remitted in a woman who had a persistent hypothalamic lesion that was not amenable to surgery or radiotherapy. The factors that may predict spontaneous recovery of anterior pituitary function in such cases are discussed.     

Dynamic MRI in the congenital agenesis of the neural pituitary stalk syndrome: the role of the vascular pituitary stalk in predicting residual anterior pituitary function

OBJECTIVE Magnetic resonance imaging (MRI) without contrast medium is unable to give detailed information on the hypothalamic-pituitary structures. MRI using gadopentetate dimeglumine (Gd-DTPA), and dynamic MRI, were performed in patients with hypopituitarism previously diagnosed as having anterior pituitary hypoplasia, ectopic posterior pituitary and unidentified pituitary stalk (1) to determine whether Gd-DTPA improves the delineation of hypothalamic-pituitary structures; (2) to verify whether, if so, such improvement can be correlated with residual pituitary function in patients subjected to long-term follow-up; and (3) to identify the hypothalamic-pituitary vascular network in such cases. PATIENTS Eighteen patients (13 males, 5 females) aged 10–26.4 years with unidentified pituitary stalk at first MRI study were evaluated. Eight had isolated GH deficiency (IGHD), and 10 had multiple pituitary hormone defect (MPHD) with the progression to complete anterior pituitary deficits seen by the age of 15 years in 8 patients (1 had GH and FSH–LH deficiency and 1 had GH, TSH and FSH–LH deficiency). RESULTS The MRI revealed a very thin pituitary stalk in 7 patients (38.8%), 6 with IGHD (75%) and 1 (10%) with MPHD (GH and FSH-LH deficiency), after Gd-DTPA administration. Reassessment of anterior pituitary function showed that the thyroid, adrenal and gonadal functions were intact in the 6 patients with IGHD and pituitary stalk identified by Gd-DTPA as well as in one IGHD patient with no evidence of pituitary stalk. In one 10-year-old with IGHD at the time of presentation (6 years) and no pituitary stalk seen after Gd-DTPA, subclinical hypothalamic hypothyroidism and suspected hypogonadotropic hypogonadism were documented. Partial ACTH deficiency was recorded In the patient with TSH and FSH–LH deficiency with no pituitary stalk. After Gd-DTPA, patients with absent pituitary stalk had a risk of developing MPHD 27 times greater than had those with an identified pituitary stalk (relative risk r=27, 95% confidence interval 1.9–368.4, Fisher’s exact test P=0.009). Dynamic MR images obtained every 4.6 s revealed rapid enhancement of hypothalamic-pituitary structures and allowed the determination of the times to initial enhancement of ectopic posterior pituitary and hypoplastic anterior pituitary which ranged between 9.2 and 18.4 s, and that of complete anterior pituitary (32.2–41.4 s). The time to maximum enhancement of anterior pituitary was significantly longer than in controls (35.5±3.8 s vs 25.2±1.6 s, P<0.0001). CONCLUSIONS MRI with Gd-DTPA proved more sensitive in identifying the vascular component of pituitary stalk and added new information about the partial preservation of hypothalamo-hypophyseal portal vessels. The vascular pituitary stalk is easily recognized after Gd-DTPA in most IGHD patients, but exceptionally in MPHD; this sheds light on the possible normal course of affected patients. The neural component of the pituitary stalk is lacking regardless of whether patients have IGHD or MPHD, indicating that the term congenital agenesis of the neural pituitary stalk is more appropriate than pituitary stalk interruption. The times to enhancement of ectopic posterior pituitary and residual anterior pituitary obtained by the fast-framing MRI technique disclose dynamic changes in regional blood supply which appear direct, arterial and mainly independent of the portal system     

Growth hormone deficiency with ectopic neurohypophysis: anatomical variations and relationship between the visibility of the pituitary stalk asserted by magnetic resonance imaging and anterior pituitary function.

In GH-deficient children showing ectopic posterior pituitary hyperintense signal (EPP), the anatomical details of the pituitary-hypothalamic region and the relationship between the visibility of the pituitary stalk and anterior pituitary function were studied by magnetic resonance imaging (MRI). The absence or presence of the pituitary stalk was recorded by MRI before and after the injection of gadolinium in 25 children with GH deficiency and EPP at the age of 8.7+/-5.0 yr (16 males and 9 females). Patients were classified into 2 groups according to the presence (group 1; n = 14), or the absence (group 2; n = 11) of pituitary stalk visibility after gadolinium injection. Most patients in group 1 (12 of 14) demonstrated isolated GH deficiency, whereas all but 1 patient in group 2 showed multiple anterior pituitary hormone deficiency. The prevalence of a normally sized adenohypophysis was higher in group 1 than in group 2 (50% vs. 9%; P<0.05). Although the EPP was found at the median eminence in all group 2 patients, it was visualized in group 1 at different levels of the pituitary stalk in 60% of cases (8 of 14; at the proximal end of the pituitary stalk, n = 4; in the middle of the pituitary stalk, n = 2; at the distal end of the pituitary stalk, n = 2). This demonstrates that the ectopic posterior pituitary migration abnormality may be complete or partial. In conclusion, although the pathogenesis of GH deficiency with EPP remains unclear, these results suggest that in cases of GH deficiency associated with ectopic posterior pituitary hyperintense signal, patients with no visible pituitary stalk on MRI after gadolinium injection present a more severe form of the disease in childhood associated with multiple anterior pituitary hormone deficiency, whereas visibility of the pituitary stalk is related to isolated GH deficiency. Nevertheless, careful follow-up of these latter patients is necessary, as the natural history of the disease is not established until adulthood.     

The relation between pituitary magnetic resonance imaging findings and GH, TSH, PRL dynamics in patients with idiopathic GH deficiency

The relation between pituitary magnetic resonance imaging (MRI) findings and anterior pituitary function was studied in 36 patients with classic idiopathic GH deficiency. These patients were divided into three groups based on MRI findings which were compared with those of 14 normal short children; i.e. normal stalk (N = 6), narrowed stalk (N = 20), and transected stalk (N = 10). The transected and narrowed stalk groups showed significantly delayed TSH responses to TRH compared with the normal stalk group and with the normal short children. Further, the mean maximal TSH increment in the narrowed and transected stalk group was slightly greater than that in normal short children. In contrast, there were no differences in basal plasma GH and PRL levels and their responses to GHRH and TRH among the three groups. When the patients were divided into normal anterior pituitary and atrophic pituitary groups regardless of stalk changes or when they were divided into groups of stalk changes (narrowing and transection) with and without pituitary atrophy, no differences in GH, TSH and PRL dynamics between the groups were observed. These results indicate that pituitary thyrotrope functions, but not somatotrope and lactotrope functions, in patients with idiopathic GH deficiency are more closely correlated to stalk changes than to anterior pituitary changes observed on MRI.     

Dopa accumulates in the hypothalamic-hypophysial portal vessels and is taken into the anterior pituitary of NSD-1015-treated rodents.

DOPA was measured in the anterior pituitary and hypothalamic-hypophysial portal blood after treatment with NSD-1015, a DOPA decarboxylase inhibitor. NSD-1015 caused DOPA to accumulate in the anterior pituitary of mice and rats, and increased DOPA in the hypothalamic-hypophysial portal blood of rat. Serum prolactin was also increased. Interruption of the anterior pituitary blood supply from the hypothalamic-hypophysial system by cannulation of the entire pituitary stalk eliminated the NSD-1015-induced DOPA accumulation in the rat pituitary. We conclude that DOPA can be taken into the anterior pituitary from the portal blood of NSD-1015-treated rodents and that the anterior pituitary lacks tyrosine hydroxylase activity in both mice and rats.     

Localization of growth hormone-releasing hormone in the human hypothalamus and pituitary stalk

The localization of the recently identified GH-releasing hormone (GHRH) in the human hypothalamus and pituitary stalk was determined by microdissection techniques and a specific RIA for GHRH. The highest concentrations of GHRH immunoreactivity (IR-GHRH) in the hypothalamus were found in the area of the infundibular nucleus (83 +/- 4 ng/mg protein; average +/- range). Lower quantities were found in other hypothalamic regions. Very high concentrations of IR-GHRH were present in the upper portion of the pituitary stalk (1454 +/- 48 ng/mg protein), and they decreased gradually toward the distal end of the stalk (21 +/- 3 ng/mg). This concentration gradient suggests that the peptide reaches the anterior pituitary mainly by way of the long portal vessels. Somatostatin, the second neuropeptide involved in the regulation of GH secretion from the anterior pituitary, had a pattern of distribution along the pituitary stalk very similar to that of IR-GHRH.     

Congenital hypopituitarism in a 48-year old adult. Natural course, hormonal study and MRI evidence

A case of Congenital Hypopituitarism (CH) in an untreated 48 yr-old-man is reported. The hormonal studies demonstrated a panhypopituitarism and MR imaging revealed absence of pituitary stalk, small anterior pituitary remnant on the sella floor and ectopic neurohypophysis at the tuber cinereum. The pattern of hormonal responsiveness suggests that CH encompasses findings typical of primary anterior pituitary disease and those of hypothalamic dysfunction.     

A case of lymphocytic infundibuloneurohypophysitis showing diabetes insipidus followed by anterior hypopituitarism associated with thrombasthenia

We report a case of a 42-year old male patient with diabetes insipidus followed by anterior hypopituitarism associated with thrombasthenia. The patient had been diagnosed with thrombasthenia since the age of 19. He was admitted and diagnosed as diabetes insipidus in 1995. Although T1-weighted image of magnetic resonance imaging (MRI) showed empty sella and partial pituitary stalk hypertrophy, the anterior pituitary functions were normal at that time. Three years later, he was re-admitted after an episode of general malaise and impotence in 1998. Endocrinological studies revealed adrenal insufficiency, hypothyroidism and hypogonadism. T1-weighted image of MRI demonstrated the thickening of pituitary stalk and neurohypophysis. Analysis of anti-pituitary antibodies by immunoblotting identified a major band at 61.5 kDa. The diabetes insipidus was controlled by desmopressin acetate and the shrinkage of pituitary stalk was seen after hormonal replacement therapy including glucocorticoid and thyroid hormone. We suggested that this case represented lymphocytic infundibuloneurohypophysitis, in which a chronic inflammatory process occurred in infundibulum and/or neurohypophysis and that hypopituitarism developed possibly due to damage to the pituitary portal vessels caused by a thickened pituitary stalk, although a pituitary biopsy was not done because of the risk of bleeding in thrombasthenia. The pituitary autoantibodies in sera from patients with hypopituitarism may be helpful to characterize the patient with lymphocytic hypophysitis.     

Sequential contrast-enhanced magnetic resonance imaging in the diagnosis of growth hormone deficiencies

The purpose of the present study was to assess the presence and the time-course of contrast-enhancement in the pituitary gland and pituitary stalk of 24 patients with isolated growth hormone (GH) deficiency and multiple pituitary hormone deficiency. The patients were evaluated clinically (auxological measurements), endocrinologically (spontaneous GH secretion and GH stimulation tests) and with conventional MRI scans. In addition, fast-framing dynamic magnetic resonance imaging (MRI) with Gd-DTPA enhancement was used to quantitate the time course of contrast enhancement within the neurohypophysis, pituitary stalk, postero-superior adenohypophysis and antero-inferior adenohypophysis. In 3 patients without evidence of abnormalities at normal conventional MRI scans (normal anterior lobe and pituitary stalk, normal posterior lobe) and a high response to the GRF provocation test, sequential time-resolved Gd-enhanced MRI demonstrates reduced contrast enhancement in the pituitary stalk. These findings are consistent with impairment in stalk vasculature, presumably located at the level of the portal venous system, and could play a role in the pathogenesis of pituitary hormonal deficiency.     

伴有泪腺炎的淋巴细胞性垂体炎

淋巴细胞性垂体炎是一种少见的自身免疫性疾病，本例的临床症状包括眼睑肿胀，垂体前叶功能低下和中枢性尿崩症，MRI显示双侧眼睑肿胀，双泪腺增大，各眼外肌未见异常，垂体柄明显增粗，垂体后叶高信号消失，垂体前叶未见异常。泪腺活检显示淋巴细胞浸润。经大剂量甲基强的松龙冲击治疗后，垂体病变和眼睑肿胀均在短期内显著减轻，临床诊断为伴有泪腺炎的淋巴细胞性垂体炎。