Congenital gigantism due to growth hormone-releasing hormone excess and pituitary hyperplasia with adenomatous transformation.
Congenital gigantism due to growth hormone-releasing hormone excess and pituitary hyperplasia with adenomatous transformation.
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DOI：
10.1210/jcem.76.1.8421089
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发表时间：
1993
期刊：
影响因子：
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通讯作者：
D. Zimmerman;W. Young;M. Ebersold;B. Scheithauer;K. Kovacs;É. Horváth;M. Whitaker;N. Eberhardt;T. Downs;L. Frohman
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文献类型：
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作者：
D. Zimmerman;W. Young;M. Ebersold;B. Scheithauer;K. Kovacs;É. Horváth;M. Whitaker;N. Eberhardt;T. Downs;L. Frohman
大多数患者的垂体瘤是生长激素分泌型垂体瘤。在本报告中，描述了一例可能因GH释放激素（GHRH）中央超分泌而导致的先天性巨人症病例。出生时正常（4.4 kg; 53 cm），我们的7岁男性患者此后逐渐生长，在我们评估时达到182 cm的身高和99.4 kg的体重。在标准的3小时口服葡萄糖耐量试验中，GH基线水平（730微克/L）的显著升高并未受到抑制，但在静脉输注GHRH后确实升高了54%。胰岛素样生长因子-I、PRL和免疫反应性GHRH的基线血浆水平也显著升高。头部电脑影像显示一个大的，部分囊性鞍和鞍上肿块。广泛的影像学研究没有定位GHRH的潜在来源。术前用奥曲肽和溴隐亭治疗4个月，导致鞍上组织质量减少25%。经蝶和经额手术切除的垂体组织显示大量的生长激素、催乳激素和乳房生长激素增生。生长激素和催乳素分泌细胞腺瘤性转化的地区也很明显。没有组织学或免疫组织化学证据表明GHRH来源于垂体，外周血浆免疫反应性GHRH浓度不受药物和手术干预的影响。我们怀疑，先天性下丘脑调节缺陷可能是负责在这种情况下的GHRH过量。
The cause of gigantism in most patients is a GH-secreting pituitary tumor. In this report, a case of congenital gigantism due to probable central hypersection of GH-releasing hormone (GHRH) is described. Normal at birth (4.4 kg; 53 cm), our 7-yr-old male patient grew progressively thereafter to attain a height of 182 cm and a weight of 99.4 kg at the time of our evaluation. The markedly increased baseline plasma levels of GH (730 micrograms/L) did not suppress during a standard 3-h oral glucose tolerance test, but did increase 54% after iv infusion of GHRH. Baseline plasma levels of insulin-like growth factor-I, PRL, and immunoreactive GHRH were also markedly increased. Computed imaging of the head showed a large, partially cystic sellar and suprasellar mass. Extensive imaging studies did not localize a potential source of GHRH. Preoperative treatment with octreotide and bromocriptine for 4 months resulted in a 25% reduction of suprasellar tissue mass. The pituitary tissue removed at transsphenoidal and transfrontal operations showed massive somatotroph, lactotroph, and mammosomatotroph hyperplasia. Areas of GH- and PRL-secreting cell adenomatous transformation were also evident. No histological or immunohistochemical evidence of a pituitary source of GHRH was found. The peripheral plasma immunoreactive GHRH concentration remained unaffected by pharmacological and surgical interventions. We suspect that a congenital hypothalamic regulatory defect may be responsible for the GHRH excess in this case.