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.     

垂体腺瘤患者围术期糖皮质激素替代疗法的研究进展及争议

垂体腺瘤是第二位常见的原发性中枢神经系统肿瘤,糖皮质激素常作为垂体腺瘤患者围术期常规替代治疗药物,用以预防术后肾上腺皮质功能低下或肾上腺危象发生.但近年随着临床对糖皮质激素不良反应认识程度的加深、对患者围术期激素动态变化研究的深入,并结合既往研究,使垂体腺瘤患者围术期常规给予糖皮质激素替代治疗的做法受到质疑.本文就垂体...     

垂体腺瘤患者的围手术期垂体激素监测及临床意义

为了对垂体腺瘤患者进行手术前后激素测定、分型、并监测术后垂体功能以及寻求一种判断手术切除程度及术后有无复发的方法，对50例垂体腺瘤手术患者采用放免法测定手术前、后激素水平，对测定值进行回顾性分析，判断检测结果的相互关系并分析其临床意义。结果：泌乳素(PRL)腺瘤及生长激素(GH)腺瘤全切组及末全切组术后激素水平均较术前明显降低(P＜0．05)，全切组术后激素水平与末全切组比较有明显差异(P＜0．05)。认为手术治疗可以明显改善垂体瘤患者术前高激素水平状态，手术治疗有着肯定的治疗价值。其切除程度将决定着术后内分泌的改善水平，应在不损害正常垂体前提下争取全切肿瘤。     

垂体瘤术后垂体功能评估情况的调查

目的 分析垂体瘤患者术后1周及3个月的垂体功能复查情况,为垂体瘤手术患者的后续临床治疗提供参考.方法 收集1993年1月至2010年1月期间解放军总医院内分泌科住院诊断为垂体瘤并进行完整垂体功能评估后行垂体瘤切除术的106例患者,回顾性分析其各项临床资料.结果 患者术后1周与3个月的乖体功能评估率分别为23.6％与16.0％.术前存在垂体功能减退患者,术后1周与3个月的垂体功能评估率分别为23.5％与5.9％,术前垂体功能无减退者垂体功能评估率分别为23.6％和20.8％,术前存在垂体功能减退者并未得到更多的垂体功能评价关注.术后l周因手术导致新发垂体功能减退率为48.0％,术后3个月为35.3％.结论 垂体瘤术后1周内及3个月复查率低,垂体瘤术后可能存在较多漏诊垂体功能减退的患者.     

希恩综合征合并重症急性胰腺炎1例报告

<正>希恩综合征是指产后垂体功能减退或垂体分泌的一种或多种激素减少或缺乏^[1],该疾病是由于分娩期间或分娩后大量子宫出血引起严重低血压或休克而导致垂体坏死。垂体前叶分泌的激素缺乏可导致继发性甲状腺功能减退、肾上腺功能衰竭、性腺功能减退、哺乳功能衰竭和生长激素缺乏。此类患者合并重症急性胰腺炎,救治难度极大,且相关报道较少。现对1例希恩综合征合并重症急性胰腺炎致顽固性休克患者报道如下。     

垂体危象

垂体危象是指慢性垂体前叶功能减退的患者在应激的情况下发生休克或昏迷等危象表现。这是一种较罕见的急危重症。若发生危象前，无明确患有慢性垂体前叶功能减退症者，则诊断就十分困难了，需要医生的敏锐的观察力和抓住重点地询问有关病史。此时任何激素的测定因不能及时报告对指导垂体危象抢救都没有现实意义。慢性垂体前叶功能减退症的病因主要是席汉综合征Gheehansvndrome）和垂体及垂体旁的肿瘤。席汉综合征是产后大失血所致，多见于偏僻农村、文化卫生知识较低的妇女，现在城市大医院已很少见。垂体及垂体旁的肿瘤因压迫正常的垂体组…     

垂体功能减退激素替代治疗1例

目的阐明垂体功能减退患者的临床表现，探讨垂体激素替代治疗的几点经验。方法对1例垂体腺瘤切除术后的临床特征进行分析，结合检验结果，对疗效进行观察。结果该例患者病情得到缓解，生活质量提高。结论垂体功能减退经激素替代治疗后患者相应的功能得以代偿，病情改善。积极防治并发症对保证患者体内激素水平的稳定起到了不容忽视的作用。     

鞍区肿瘤术后内分泌代谢紊乱的防治和管理

随着神经外科经鼻蝶内镜技术的进步，鞍区肿瘤手术全切率和安全性已明显提高。但由于肿瘤类型、大小、生物特性多变，且鞍区位置特殊，术中有时难免损伤下丘脑、垂体，造成垂体内分泌和水、盐代谢紊乱，导致患者术后出现记忆力下降、生长发育停滞、不孕不育、肥胖、多尿、电解质紊乱等，并且医患存在对下丘脑、垂体和靶腺激素替代认识不足，治疗不规范，随访不积极，严重时危及生命。本文从内分泌专科医生角度，详细阐述鞍区肿瘤术后垂体功能减退、下丘脑综合征及水、盐代谢紊乱的防治和管理，希望通过本文规范激素替代治疗，促进患者垂体功能重建，改善患者预后，提高远期生活质量，减少死亡率。     

老年垂体瘤患者术后垂体功能减退的高危因素

目的探讨老年垂体瘤患者术后垂体功能减退的高危因素。方法选择垂体瘤患者80例作为观察对象,观察患者术后垂体功能减退发生情况。多因素分析影响术后垂体功能减退高危因素,调查资料包括性别、术前垂体功能减退、合并高血压、合并糖尿病、合并高脂血症、吸烟史、术后肿瘤复发、肿瘤直径、手术入路。结果 80例垂体瘤患者出现垂体功能减退34例(42.5%);无垂体功能减退46例(57.5%)。经单因素分析表明,不同性别、合并高血压、合并糖尿病、合并高脂血症、吸烟史和手术入路术后垂体功能减退发生率比较差异无统计学意义(P0.05);术后垂体功能减退患者:术前垂体功能减退所占比例显著高于术后无垂体功能减退患者,术后肿瘤复发率显著高于术后无垂体功能减退患者,肿瘤直径30 mm所占比例显著高于术后无垂体功能减退患者,差异具有统计学意义(P0.05)。将上述单因素分析差异具有统计学意义的纳入多因素分析显示,术前垂体功能障碍、术后肿瘤复发和肿瘤直径30 mm为影响术后垂体功能减退高危因素。结论应对老年垂体瘤患者术后垂体功能减退的高危因素给予重点关注,早发现、早治疗。     

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.     

Can cardiac surgery cause hypopituitarism?

Apoplexy of pituitary adenomas with subsequent hypopituitarism is a rare but well recognized complication following cardiac surgery. The nature of cardiac on-pump surgery provides a risk of damage to the pituitary because the vascular supply of the pituitary is not included in the cerebral autoregulation. Thus, pituitary tissue may exhibit an increased susceptibility to hypoperfusion, ischemia or intraoperative embolism. After on-pump procedures, patients often present with physical and psychosocial impairments which resemble symptoms of hypopituitarism. Therefore, we analyzed whether on-pump cardiac surgery may cause pituitary dysfunction also in the absence of pre-existing pituitary disease. Twenty-five patients were examined 3–12 months after on-pump cardiac surgery. Basal hormone levels for all four anterior pituitary hormone axes were measured and a short synacthen test and a growth hormone releasing hormone plus arginine (GHRH-ARG)-test were performed. Quality of life (QoL), depression, subjective distress for a specific life event, sleep quality and fatigue were assessed by means of self-rating questionnaires. Hormonal alterations were only slight and no signs of anterior hypopituitarism were found except for an insufficient growth hormone rise in two overweight patients in the GHRH-ARG-test. Psychosocial impairment was pronounced, including symptoms of moderate to severe depression in 9, reduced mental QoL in 8, dysfunctional coping in 6 and pronounced sleep disturbances in 16 patients. Hormone levels did not correlate with psychosocial impairment. On-pump cardiac surgery did not cause relevant hypopituitarism in our sample of patients and does not serve to explain the psychosocial symptoms of these patients.     

Medical Management of Hypopituitarism in Patients with Pituitary Adenomas

Hypopituitarism is a disease complex with variable clinical manifestations. Recent studies have improved our understanding of its pathophysiology, particularly in patients with pituitary adenomas. In that setting, hypopituitarism was previously considered a permanent and irreversible process, requiring life-long hormone replacement therapy. While this could be true in some instances, recent data demonstrated recovery of pituitary function in a large number of patients with hypopituitarism following surgical decompression. Mechanical compression of portal vessels and the pituitary stalk, by the expanding adenoma was postulated to be the predominant mechanism causing hypopituitarism in this setting. Since portal vessels also provide blood supply to the anterior lobe, ischemic necrosis of portions of the pituitary can occur as a result of increased and prolonged compression by the expanding adenoma. Recent data demonstrated increases in intrasellar pressure (ISP) in patients with pituitary macroadenomas, particularly those with hypopituitarism. The data showed that ISP measurements correlated positively with the serum prolactin levels but not with tumor sizes. It is postulated that increased ISP has predominant role in the pathogenesis of hypopituitarism in patients with pituitary adenomas while. The increase in ISP results in decreased blood flow through the portal vessels and the pituitary stalk. The latter will result in diminished delivery of hypothalamic hormones to the anterior pituitary and may also cause ischemia and/or necrosis in some portions of the normal gland. Recovery of pituitary function can thus be anticipated after surgical decompression, in patients who have viable pituitary cells.Understanding the pathophysiology of hypopituitarism and recognizing the probability for recovery of function should be emphasized in the management of patients with this disease. An important aspect of the management is patients' education about their disease, including the use of medic alert identification. The managing physician should appreciate the variable clinical manifestations of the disease and the possible occurrence of other associated neuroendocrine, neurological and neuro-ophthalmologic signs and symptoms. Treatment of hypopituitarism should not be rigid but instead, always individualized. Management should take into consideration the patients' age, sex, education, original disease process and clinical history.     

The dominant role of increased intrasellar pressure in the pathogenesis of hypopituitarism, hyperprolactinemia, and headaches in patients with pituitary adenomas

Mild hyperprolactinemia frequently accompanies the hypopituitarism seen in patients with pituitary macroadenomas that do not secrete PRL. Recent data suggested that the hypopituitarism and mild hyperprolactinemia in this setting are largely due to compression of pituitary stalk and portal vessels. Headaches (HAs) are frequently seen in patients with large adenomas and at times in those with microadenomas. Because the walls of the sella turcica are relatively rigid, we postulate that tumor growth within the sella increases intrasellar pressure (ISP), which in turn impairs portal blood flow, resulting in mild hyperprolactinemia and hypopituitarism. We also postulate that increased mean ISP (MISP) contributes to the development of HAs. Normal MISP is not known but is unlikely to exceed normal intracranial pressure of less than 10-15 mm Hg. We determined MISP in 49 patients who had transsphenoidal surgery for pituitary adenomas. MISP was measured using a commonly available intracranial monitoring kit where a fiberoptic transducer was inserted through a 2-mm dural incision at the time of adenomectomy. Patients with deficient FSH, LH, ACTH, or TSH secretion were considered hypopituitary. Data on serum PRL levels were included for analysis only in patients whose adenomas had negative immunostaining for the hormone. MISP measurements ranged from 7-56 mm Hg, with a mean (+/-SD) of 28.8 +/- 13.5 and a median of 26 mm Hg. The pressure measurements were higher in patients with hypopituitarism than in those with normal pituitary function (P = 4.6013 x 10(-6)). Patients presenting with HAs had higher MISP than those who did not (P = 5.44 x 10(-7)), regardless of their pituitary function or tumor sizes. PRL levels correlated positively with MISP values (r = 0.715, P < 0.0001). Tumor size did not correlate with MISP or PRL levels. The findings of increased MISP in hypopituitary patients and the documented correlation with PRL levels, suggest that ISP is a major mechanism involved in the pathogenesis of hypopituitarism and hyperprolactinemia. Similarly, the increased MISP in patients with HAs, irrespective of tumor size or pituitary function, suggest that increased ISP is a major mechanism involved in the pathogenesis of this symptom. The data support the hypothesis that in patients with pituitary adenomas increased ISP is a major mechanism contributing to the development of hyperprolactinemia, hypopituitarism, and HAs. Increased ISP in these patients leads to compression of the portal vessels and the associated interruption of the delivery of hypothalamic hormones to the anterior pituitary. This would explain the reversibility of pituitary function observed in most patients after adenomectomy. However, increased ISP may also lead to decreased blood supply, resulting in ischemic necrosis in some regions of the pituitary. The latter could limit potential recovery of pituitary function after adenomectomy.     

Hypothalamic insufficiency: the cause of hypopituitarism in sarcoidosis

Ten patients with generalized sarcoidosis and hypopituitarism were studied. Six of these 10 patients presented with sarcoid involvement of the optic nerves resulting in asymmetric visual field defects. All patients had deficiencies of two or more anterior pituitary hormones and seven had abnormalities of water metabolism. Despite hypopituitarism, nine patients had a pituitary responsive to the synthetic hypothalamic releasing factors, thyrotropin releasing hormone and gonadotropin releasing hormone, and the tenth patient had a partially responsive pituitary. The demonstration of pituitary responsiveness allows us to infer hypothalamic insufficiency as the major cause for hypopituitarism in these patients. The combination of visual field defects and hypopituitarism in sarcoidosis is a medically treatable condition that simulates the clinical presentation of a pituitary tumor.     

Anterior hypopituitarism is rare and autoimmune disease is common in adults with idiopathic central diabetes insipidus

Objective Central diabetes insipidus is a rare clinical condition with a heterogenous aetiology. Up to 40% of cases are classified as idiopathic, although many of these are thought to have an autoimmune basis. Published data have suggested that anterior hypopituitarism is common in childhood‐onset idiopathic diabetes insipidus. We aimed to assess the incidence of anterior hypopituitarism in a cohort of adult patients with idiopathic diabetes insipidus. Design and patients We performed a retrospective review of the databases of two pituitary investigation units. This identified 39 patients with idiopathic diabetes insipidus. All had undergone magnetic resonance imaging scanning and dynamic pituitary testing (either insulin tolerance testing or GHRH/arginine and short synacthen testing) to assess anterior pituitary function. Results One patient had partial growth hormone deficiency; no other anterior pituitary hormonal deficits were found. Thirty‐three percent had at least one autoimmune disease in addition to central diabetes insipidus. Conclusions Our data suggest that anterior hypopituitarism is rare in adult idiopathic diabetes insipidus. Routine screening of these patients for anterior hypopituitarism may not, therefore, be indicated. The significant prevalence of autoimmune disease in this cohort supports the hypothesis that idiopathic diabetes insipidus may have an autoimmune aetiology.     

Mortality following pituitary radiotherapy

External beam radiotherapy has been used in the management of pituitary adenomas for nearly a century, preventing tumor regrowth following surgery for non-functioning pituitary adenomas and suppressing functional hypersecretion in those which are hormonally active. However, it has been linked with a number of potentially significant complications including formation of secondary intracranial tumors, cognitive impairment, hypopituitarism and cerebrovascular disease, as well as increased mortality. Radiation may cause a variety of vascular injuries and hemodynamic changes to the cerebral vasculature, and several authors have reported cerebrovascular complications and an increase in cerebrovascular mortality in patients receiving radiotherapy for pituitary and other central nervous system tumors. Ten years following pituitary radiotherapy, over 50% of patients develop deficiencies in one or more anterior pituitary hormones. A number of studies have demonstrated increased mortality in patients with hypopituitarism, predominantly due to cerebrovascular and cardiovascular disease. However, no clear answer has emerged with regards to causation, and pituitary radiotherapy has only been linked directly to mortality in one of these studies. Questions remain unanswered, and the use of conventional external beam radiotherapy in the management of pituitary disease must involve a critical risk-benefit analysis in each case.     

Subarachnoid Hemorrhage As a Cause of Hypopituitarism

Common causes of pituitary insufficiency include pathologies such as pituitary adenomas, other intra- and parasellar tumors, as well inflammatory, surgical or radiation-induced destruction of pituitary tissue. More recently, hypopituitarism has also been identified as a frequent sequel of traumatic brain injury (TBI). Despite the close anatomical relationship between pituitary gland, hypothalamic structures and the arteries of the circle of Willis and the observation that many bodily and psychosocial long-term sequelae of survivors of aneurysmal subarachnoid hemorrhage (SAH) resemble those of patients with untreated hypopituitarism, aneurysmal SAH has so far been considered a rare cause of endocrine abnormalities. However, in recent clinical investigations partial hypopituitarism has been diagnosed in 37.5–55% of SAH survivors. At present, many questions concerning the most appropriate diagnostic work-up, the clinical implications of hormone deficiencies and relative importance of hormone replacement in this patient group are not yet satisfactorily clarified. This article gives an overview of the historical and current studies on hypopituitarism as a consequence of aneurysmal SAH, which show that neuroendocrine deficiency occurs more often than has so far been appreciated. Despite the still unresolved issues, endocrine abnormalities should be considered a cause for impaired recovery and long-term morbidity in SAH-survivors. Enhanced awareness of physicians treating SAH-patients for post-traumatic hypopituitarism is called for, so that screening for hormone deficiencies and appropriate replacement therapy can be initiated.     

Recovery of hypopituitarism after neurosurgical treatment of pituitary adenomas.

Surgery is the treatment of choice for many pituitary tumors; pituitary function may suffer after operation, but relief of pressure on the normal pituitary may also favor postoperative recovery of hypopituitarism. The aim of this study was to investigate the frequency of new appearance and recovery of hypopituitarism after neurosurgery and try to identify features associated with it. Pre- and postoperative anterior pituitary functions were investigated in 234 patients with pituitary adenomas (56 nonfunctioning, 71 PRL-secreting, 66 GH-secreting, 39 ACTH-secreting, 1 LH/FSH-secreting, and 1 TSH-secreting tumors). Eighty-eight new postoperative pituitary hypofunctions appeared in 52 patients (12 NF, 14 PRL-secreting, 15 GH-secreting, 10 ACTH-secreting, and 1 LH/FSH-secreting adenomas). They corresponded to 27% ACTH deficiencies (in 29 of the 107 patients with normal preoperative ACTH in whom postoperative evaluation was complete), 14.5% (15 of 103) new GH deficiencies, 10.5% (15 of 143; P < 0.0005, significantly less than ACTH deficiency) new TSH deficiencies, 16.5% (20 of 121) new gonadotropin deficiencies, and 13% (9 of 71) new PRL deficiencies. Preoperatively, 93 were deficient in at least 1 pituitary hormone; after surgery, 45 (48%) recovered between 1 and 3 hormones. The 2 patients with LH/FSH- and TSH-secreting macroadenomas did not recover pituitary function. Factors associated with a higher probability of postoperative pituitary function recovery were: no tumor rests on postoperative pituitary imaging (P = 0.001) and no neurosurgical (P = 0.001) or pathological evidence (P = 0.049) of an invasive nature. Tumor size did not differ significantly between those who did and those who did not recover pituitary function after surgery. Even if clear hypofunction is observed at initial work-up, patients should be reassessed after surgery without substitution therapy, because practically half the preoperative pituitary hormone deficiencies recover postoperatively, eliminating the need for life-long substitution therapy.