Section of Investigative Medicine, Division of Diabetes, Endocrinology and Metabolism, Imperial College London, Hammersmith Hospital, London, UK.
Hormones (Athens). 2011 Apr-Jun;10(2):156-61. doi: 10.14310/horm.2002.1306.
Patients with acromegaly have characteristic clinical features caused by soft tissue overgrowth. The most common cause of acromegaly is a growth hormone-secreting adenoma of the anterior pituitary. Both somatic and metabolic features of acromegaly are due to excess growth hormone (GH) secretion and high serum concentrations of insulin-like growth factor-I (IGF-I). Here we present a case of "pseudoacromegaly" with an acromegaloid phenotype, insulin resistance, history of adenomatous colonic polyp, and suppressed IGF-I levels. Patients with this rare condition are likely to have a selective post-receptor defect of insulin signalling, leading to the impairment of metabolic but preservation of mitogenic signalling. Endocrinologists should consider this diagnosis when assessing patients with clinical features of acromegaly and insulin resistance, in the absence of elevated levels of GH and IGF-I.
肢端肥大症患者存在由软组织过度生长引起的特征性临床特征。肢端肥大症最常见的原因是垂体前叶生长激素分泌腺瘤。肢端肥大症的躯体和代谢特征均归因于生长激素 (GH) 分泌过多和胰岛素样生长因子-I (IGF-I) 血清浓度升高。本文报道了一例具有肢端肥大症表型、胰岛素抵抗、腺瘤性结肠息肉病史和 IGF-I 水平降低的“假性肢端肥大症”病例。这种罕见疾病的患者可能存在胰岛素信号的选择性受体后缺陷，导致代谢受损但有丝分裂信号得以保留。当评估具有肢端肥大症和胰岛素抵抗临床特征而 GH 和 IGF-I 水平不高的患者时，内分泌科医生应考虑这种诊断。
