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肢端肥大症和巨人症的临床表现及生化诊断

The clinical presentation and biochemical diagnosis of acromegaly and gigantism.

作者信息

Jialal I, Nathoo B C, Joubert S, Asmal A C, Pillay N L

出版信息

S Afr Med J. 1982 Apr 24;61(17):617-20.

PMID:6805079
Abstract

Over a 5-year period 14 patients with acromegaly and gigantism were seen at the endocrine clinic of King Edward VIII Hospital: 9 were Blacks and 5 Indians; 8 of the patients were women. The mean age of the patients was 46 years. Surprisingly, only 2 patients complained of acral overgrowth. Symptomatology was varied and not characteristic of the condition. On examination all patients had unequivocal signs of soft-tissue and bony overgrowth, 64% had visual abnormalities and 50% hypertension. Radiologically, 88% showed an enlarged pituitary fossa. On biochemical investigation, the fasting levels of growth hormone (GH) were increased in 12 patients and during oral glucose tolerance tests, the GH levels in these 12 patients were not suppressed. One patient in whom the fasting GH level was not increased had progressed to the stage of panhypopituitarism, in the remaining patient challenge with thyrotrophin-releasing hormone (TRH) led to increased GH levels and L-dopa challenge resulted in a paradoxical decrease in GH levels. Seven patients with increased GH levels who were challenged with L-dopa showed the typical decrease in GH levels found in this condition; in 5 of these patients, challenged with TRH, GH levels increased. The findings emphasize that despite the ease of clinical diagnosis, appropriate biochemical investigations are necessary to confirm the exact status of the disease, which is rare in the population studied.

摘要

在5年期间,爱德华八世医院内分泌门诊共诊治了14例肢端肥大症和巨人症患者:9例为黑人,5例为印度人;8例患者为女性。患者的平均年龄为46岁。令人惊讶的是,只有2例患者主诉有肢端过度生长。症状表现多样,并非该病的典型特征。检查时,所有患者均有明确的软组织和骨骼过度生长体征,64%有视力异常,50%有高血压。放射学检查显示,88%的患者垂体窝扩大。生化检查方面,12例患者空腹生长激素(GH)水平升高,在口服葡萄糖耐量试验期间,这12例患者的GH水平未被抑制。1例空腹GH水平未升高的患者已进展至全垂体功能减退阶段,在其余患者中,促甲状腺激素释放激素(TRH)激发试验导致GH水平升高,左旋多巴激发试验导致GH水平出现反常下降。7例GH水平升高且接受左旋多巴激发试验的患者显示出该病典型的GH水平下降;其中5例接受TRH激发试验的患者,GH水平升高。这些发现强调,尽管临床诊断容易,但仍需要进行适当的生化检查以确诊疾病的确切状况,该病在所研究的人群中较为罕见。

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The clinical presentation and biochemical diagnosis of acromegaly and gigantism.肢端肥大症和巨人症的临床表现及生化诊断
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引用本文的文献

1
Prevalence of clinical signs, symptoms and comorbidities at diagnosis of acromegaly: a systematic review in accordance with PRISMA guidelines.肢端肥大症诊断时的临床体征、症状和合并症的流行情况:按照 PRISMA 指南进行的系统评价。
Pituitary. 2023 Aug;26(4):319-332. doi: 10.1007/s11102-023-01322-7. Epub 2023 May 20.
2
The growth hormone responses to L-dopa and TRH in acromegaly.肢端肥大症患者对左旋多巴和促甲状腺激素释放激素的生长激素反应。
J Endocrinol Invest. 1984 Jun;7(3):235-7. doi: 10.1007/BF03348430.
3
Mammosomatotroph adenoma of the pituitary associated with gigantism and hyperprolactinemia. A morphological study including immunoelectron microscopy.
垂体的乳腺生长激素细胞腺瘤伴巨人症和高催乳素血症。一项包括免疫电子显微镜检查的形态学研究。
Acta Neuropathol. 1986;71(1-2):76-82. doi: 10.1007/BF00687965.