New developments in the management of acromegaly. Should we achieve absolute biochemical cure?

New developments in the management of acromegaly. Should we achieve absolute biochemical cure?
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肢端肥大症治疗的新进展。

DOI:
10.1677/joe.0.155s023
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发表时间:
1997
期刊:
The Journal of endocrinology
影响因子:
--
通讯作者:
R. Clayton
R. Clayton
中科院分区:
--
文献类型:
--
作者:
R. Clayton

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肢端肥大症是一种不常见的疾病,在欧洲人口中的患病率约为每百万人40例,每年每百万人中有4至6例新病例。绝大多数(99%)肢端肥大症是由垂体生长激素瘤引起的,偶尔罕见的病例是由支气管类癌、胰腺神经内分泌或下丘脑肿瘤异位产生生长激素释放激素引起的,从而引起生长激素增生。这种情况的发作通常是非常隐蔽的,尤其是在老年患者中;照片可在生化诊断前5-10年显示肢端扩张的证据。治疗前组织长期暴露于生长激素(GH)的过量分泌可能对长期死亡率结果具有重要意义。此外,这也可以解释为什么大约60-70%的生长激素瘤在诊断时是大腺瘤(直径为10 - 1cm),而只有少数(30-40%)是微腺瘤。肢端肥大症的表现是非常多变的,相当比例的病例是根据患者的特征外观诊断出不相关的问题。然而,生长激素过量的后果,如手、脚、下巴和面部肿大、出汗、皮肤油腻和腕管综合征是最常见的表现形式,尽管糖尿病、高血压和肾结石形成的代谢后果可能为诊断提供初步线索。其他患者的症状可直接归因于垂体肿大,如头痛、视力障碍或垂体功能减退。这种多样的表现和罕见的情况往往意味着这种疾病不被非专业医生发现。因此,内分泌专家经常遇到出现并发症的病人。此外,内分泌学家致力于肢端肥大症患者的长期护理,因为并发症和生长激素过量对长期发病率和死亡率的影响。这种情况不应该由通才来处理。肢端肥大症的发病率和死亡率
Acromegaly is an uncommon condition with a prevalence in European populations of about 40 per million, and an incidence of four to six new cases per million population per year. The vast majority (99%) of cases of acromegaly are due to a pituitary somatotrophinoma, the occasional rare case being caused by ectopic production of growth hormone-releasing hormone by bronchial carcinoid, pancreatic neuroendocrine, or hypothalamic tumours, giving rise to somatotroph hyperplasia. The onset of the condition is most often very insidious, especially so in the older patient; photographs may reveal evidence of acral expansion 5–10 years before biochemical confirmation of the diagnosis. This long exposure of tissues to excessive secretion of growth hormone (GH) before treatment may be significant with respect to the long-term mortality outcome. Furthermore, it may also explain why about 60–70% of somatotrophinomas are macroadenomas (>1 cm in diameter) at diagnosis, and only a minority (30–40%) are microadenomas. The presentation of acromegaly is very variable, and a fair proportion of cases are diagnosed coincidentally on the basis of the characteristic appearance of a patient presenting with unrelated problems. However, the consequences of GH excess such as enlargement of the hands, feet, jaw and face, sweating, greasy skin and carpal tunnel syndrome are the most common forms of presentation, although the metabolic consequences of diabetes mellitus, hypertension and renal calculus formation may provide the initial clues to the diagnosis. Other patients may present with symptoms directly attributable to an expanded pituitary gland, such as headache, visual impairment or hypopituitarism. This varied presentation and rarity of the condition often means that the disease goes unrecognised by the non-specialist physician. Accordingly, the specialist endocrinologist frequently encounters the patient when complications have developed. Moreover, the endocrinologist is committed to the long-term care of patients with acromegaly because of the implications of the complications and GH excess for long-term morbidity and mortality. This is not a condition that should be managed by generalists. Morbidity and mortality in acromegaly
成功切除产生 GH 的垂体肿瘤后,快速生长激素 (GH) 搏动持续存在。
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影响因子: --
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影响因子: --
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影响因子: --
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