Acromegaly.
Acromegaly.
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DOI：
10.1186/1750-1172-3-17
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发表时间：
2008-06-25
影响因子：
3.7
通讯作者：
Salenave S
中科院分区：
文献类型：
--
作者：
Chanson P;Salenave S
肢端肥大症是一种与生长激素（GH）过度产生有关的获得性疾病，其特征是进行性躯体畸形（主要涉及面部和四肢）和全身表现。患病率估计为 1:140,000–250,000。该病最常见于中年人（平均年龄 40 岁，男性和女性患病率相同）。由于起病隐匿且进展缓慢，肢端肥大症通常在发病四到十多年后才被诊断出来。主要临床特征为四肢（手、足）增宽、手指增宽、粗短、软组织增厚。面部特征包括宽而厚的鼻子、突出的颧骨、突出的额头、厚厚的嘴唇和明显的面部线条。前额和上面的皮肤变厚，有时会导致额头隆起。下颌有过度生长的倾向，伴有下颌前突、上颌增宽、牙齿分离和颌咬合不正。该疾病还具有风湿病、心血管、呼吸和代谢方面的后果，这些后果决定了其预后。在大多数情况下，肢端肥大症与垂体腺瘤有关，无论是纯粹的 GH 分泌型（60%）还是混合型。在极少数情况下，肢端肥大症是由于生长激素释放激素 (GHRH) 异位分泌导致垂体增生所致。临床诊断通过口服葡萄糖耐量试验 (OGTT) 后血清 GH 浓度升高以及检测胰岛素样生长因子-I (IGF-I) 水平升高而得到生化证实。肿瘤体积和扩展的评估基于影像学研究。超声心动图和睡眠呼吸暂停测试用于确定肢端肥大症的临床影响。治疗的目的是通过切除致病病变来纠正（或预防）肿瘤压迫，并将 GH 和 IGF-I 水平降低至正常值。经蝶手术通常是一线治疗方法。当手术无法纠正 GH/IGF-I 分泌过多时，可以使用生长抑素类似物和/或放射疗法进行治疗。 GH 拮抗剂（培维索孟）用于对生长抑素类似物耐药的患者。在大多数情况下，荷尔蒙疾病得到了充分的控制，从而使预期寿命与普通人群相似。然而，即使患者被治愈或控制良好，后遗症（关节疼痛、畸形和生活质量改变）也常常残留。
Acromegaly is an acquired disorder related to excessive production of growth hormone (GH) and characterized by progressive somatic disfigurement (mainly involving the face and extremities) and systemic manifestations. The prevalence is estimated at 1:140,000–250,000. It is most often diagnosed in middle-aged adults (average age 40 years, men and women equally affected). Due to insidious onset and slow progression, acromegaly is often diagnosed four to more than ten years after its onset. The main clinical features are broadened extremities (hands and feet), widened thickened and stubby fingers, and thickened soft tissue. The facial aspect is characteristic and includes a widened and thickened nose, prominent cheekbones, forehead bulges, thick lips and marked facial lines. The forehead and overlying skin is thickened, sometimes leading to frontal bossing. There is a tendency towards mandibular overgrowth with prognathism, maxillary widening, tooth separation and jaw malocclusion. The disease also has rheumatologic, cardiovascular, respiratory and metabolic consequences which determine its prognosis. In the majority of cases, acromegaly is related to a pituitary adenoma, either purely GH-secreting (60%) or mixed. In very rare cases, acromegaly is due to ectopic secretion of growth-hormone-releasing hormone (GHRH) responsible for pituitary hyperplasia. The clinical diagnosis is confirmed biochemically by an increased serum GH concentration following an oral glucose tolerance test (OGTT) and by detection of increased levels of insulin-like growth factor-I (IGF-I). Assessment of tumor volume and extension is based on imaging studies. Echocardiography and sleep apnea testing are used to determine the clinical impact of acromegaly. Treatment is aimed at correcting (or preventing) tumor compression by excising the disease-causing lesion, and at reducing GH and IGF-I levels to normal values. Transsphenoidal surgery is often the first-line treatment. When surgery fails to correct GH/IGF-I hypersecretion, medical treatment with somatostatin analogs and/or radiotherapy can be used. The GH antagonist (pegvisomant) is used in patients that are resistant to somatostatin analogs. Adequate hormonal disease control is achieved in most cases, allowing a life expectancy similar to that of the general population. However, even if patients are cured or well-controlled, sequelae (joint pain, deformities and altered quality of life) often remain.
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影响因子：
3.7
作者：
Bertherat, Jerome
通讯作者：
Bertherat, Jerome
影响因子：
5.8
作者：
Bihan, H;Espinosa, C;Chanson, P
通讯作者：
Chanson, P
影响因子：
5.8
作者：
Bevan, JS;Atkin, SL;Wardlaw, JM
通讯作者：
Wardlaw, JM
影响因子：
5.8
作者：
Caron, P;Beckers, A;Zgliczynski, W
通讯作者：
Zgliczynski, W
DOI：
10.1055/s-2008-1058157
发表时间：
1999-01-01
期刊：
SKULL BASE SURGERY
影响因子：
--
作者：
Cappabianca, P;Alfieri, A;de Divitiis, E
通讯作者：
de Divitiis, E