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肢端肥大症

Acromegaly.

作者信息

Nabarro J D

机构信息

Middlesex Hospital, London.

出版信息

Clin Endocrinol (Oxf). 1987 Apr;26(4):481-512. doi: 10.1111/j.1365-2265.1987.tb00805.x.

DOI:10.1111/j.1365-2265.1987.tb00805.x
PMID:3308190
Abstract

A personal series of 256 cases of acromegaly/gigantism seen over a 20-year period from 1963 is described. The insidious nature of the condition resulted in delay in diagnosis which was often made by a doctor when seeing the patient for an unrelated problem. Other features which commonly led to the diagnosis being made were headache, change in appearance, carpal tunnel syndrome, amenorrhoea and diabetes. The Hardy system for grading the radiological appearance of the pituitary tumour was used. Widely invasive tumours were not common but tended to occur in patients with younger age of onset and high GH levels. The occurrence of various symptoms and clinical features was noted and the changes resulting from reducing the GH level to normal. The incidence of hypertension, but not of coronary artery disease, is increased and the blood pressure may be reduced following successful treatment. The effects on the upper and lower respiratory tract are reported as well as sleep apnoea and problems associated with anaesthesia. Skin manifestations included sweating, pigmented skin tags, acanthosis nigricans and cutis verticis gyrata. In the skeletal system the incidence of kyphoscoliosis and osteoarthritis especially of the hip is reported: the question of hip replacement is discussed. Diabetes mellitus disappeared in most cases if the acromegaly was cured. In men but not in women the incidence of colloid nodular goitre was increased as was hyperthyroidism in middle-aged women. In two patients a parathyroid adenoma was present: hypercalcaemia was present in five additional patients, but the cause was not determined. The common occurrence of amenorrhoea in the younger women was noted, it was not always associated with hyperprolactinaemia, and often responded to successful treatment of the acromegaly. The association of acromegaly with hirsutism and galactorrhoea is confirmed. The incidence of impotence and loss of libid in the men is discussed: in a proportion of those in whom the acromegaly was cured, potency returned, but in a number depression occurred and what was believed to be psychogenic impotence persisted. Hyperprolactinaemia was found in 49 out of 151 patients with active acromegaly in whom the prolactin level was measured. Previous reports have indicated a doubling of death rates in acromegalics. In this series there were 47 deaths observed compared to 37.2 expected. The increased death rate was in women of all ages and in men under the age of 55, The increased deaths in the women were from cardiovascular and cerebrovascular causes and from breast cancer.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

本文描述了自1963年起20年间所诊治的256例肢端肥大症/巨人症患者的情况。该病隐匿性强,常导致诊断延误,多由医生在处理无关问题时偶然发现。其他常见的诊断线索包括头痛、外貌改变、腕管综合征、闭经和糖尿病。采用哈代系统对垂体瘤的影像学表现进行分级。广泛侵袭性肿瘤并不常见,多见于发病年龄较轻、生长激素水平较高的患者。记录了各种症状和临床特征的发生情况,以及生长激素水平降至正常后的变化。高血压发病率升高,但冠心病发病率未增加,成功治疗后血压可能降低。报告了对上、下呼吸道的影响以及睡眠呼吸暂停和麻醉相关问题。皮肤表现包括多汗、色素沉着性皮赘、黑棘皮病和回状头皮。骨骼系统方面,报告了脊柱后凸侧弯和骨关节炎尤其是髋关节骨关节炎的发病率,并讨论了髋关节置换问题。若肢端肥大症治愈,多数患者的糖尿病会消失。男性胶体结节性甲状腺肿发病率增加,中年女性甲状腺功能亢进发病率增加,但女性无此情况。两名患者存在甲状旁腺腺瘤,另有五名患者血钙升高,但病因未明。年轻女性闭经常见,并不总是与高催乳素血症相关,肢端肥大症成功治疗后常可缓解。肢端肥大症与多毛症和溢乳的关联得到证实。讨论男性阳痿和性欲减退的发生率:部分肢端肥大症治愈的患者恢复了性功能,但也有部分患者出现抑郁,且存在疑似心因性阳痿。在151例有活动期肢端肥大症且检测了催乳素水平的患者中,49例存在高催乳素血症。既往报告显示肢端肥大症患者死亡率翻倍。本系列观察到47例死亡,预期为37.2例。各年龄段女性及55岁以下男性死亡率增加。女性死亡增加主要源于心血管和脑血管疾病以及乳腺癌。(摘要截选至400字)

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