Arafah Baha M
Division of Clinical and Molecular Endocrinology, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106, USA.
Pituitary. 2002;5(2):109-17. doi: 10.1023/a:1022316631809.
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.
垂体功能减退是一种临床表现多样的疾病综合征。最近的研究增进了我们对其病理生理学的理解,尤其是在垂体腺瘤患者中。在这种情况下,垂体功能减退以前被认为是一个永久性且不可逆的过程,需要终身激素替代治疗。虽然在某些情况下可能确实如此,但最近的数据表明,大量垂体功能减退患者在手术减压后垂体功能得以恢复。推测在这种情况下,扩大的腺瘤对门静脉血管和垂体柄的机械压迫是导致垂体功能减退的主要机制。由于门静脉血管也为垂体前叶提供血液供应,扩大的腺瘤持续增加的压迫可能导致垂体部分区域发生缺血性坏死。最近的数据表明,垂体大腺瘤患者,尤其是垂体功能减退患者,其鞍内压力(ISP)升高。数据显示,ISP测量值与血清催乳素水平呈正相关,但与肿瘤大小无关。据推测,ISP升高在垂体腺瘤患者垂体功能减退的发病机制中起主要作用。ISP升高导致通过门静脉血管和垂体柄的血流量减少。后者将导致下丘脑激素输送到垂体前叶减少,也可能导致正常腺体某些部分的缺血和/或坏死。因此,对于仍有存活垂体细胞的患者,手术减压后垂体功能有望恢复。在这种疾病患者的管理中,应强调了解垂体功能减退的病理生理学并认识到功能恢复的可能性。管理的一个重要方面是对患者进行疾病教育,包括使用医疗警示标识。主治医生应了解该疾病的各种临床表现以及其他相关神经内分泌、神经和神经眼科体征和症状可能的出现情况。垂体功能减退的治疗不应僵化,而应始终个体化。管理应考虑患者的年龄、性别、教育程度、原发病过程和临床病史。