Clayton P E, Price D A, Shalet S M
Arch Dis Child. 1987 Mar;62(3):222-6. doi: 10.1136/adc.62.3.222.
After completion of treatment with growth hormone (GH) 19 patients with isolated 'idiopathic' GH deficiency and 15 with post-irradiation GH deficiency underwent retesting of GH secretion with an insulin tolerance test or an arginine stimulation test, or both. Patients with post-irradiation GH deficiency comprised 13 patients with central nervous system tumours distant from the hypothalamo-pituitary axis and two with acute lymphoblastic leukaemia, who had received cranial or craniospinal irradiation. All 15 patients with post-irradiation GH deficiency remained GH deficient (peak GH response less than 7 mU/l (n = 10) and 7-15 mU/l (n = 5)). Of the 19 retested patients with idiopathic GH deficiency, however, five (26%) had peak GH responses of greater than 15 mU/l (regarded now as transient or false idiopathic GH deficiency) and were indistinguishable from the remainder (permanent or true idiopathic GH deficiency, peak GH responses less than 7 mU/l (n = 12) and 7-15 mU/l (n = 2)), by pretreatment anthropometry and post-treatment height standard deviation score, but had a lower first year height velocity (mean (SD) velocity 5.6 (0.5) cm/year for false idiopathic deficiency v 8.7 (1.75) cm/year for true idiopathic deficiency, p less than 0.01) and height increment on treatment (mean (SD) increment 2.2 (1.5) cm/year for false idiopathic deficiency v 5.2 (2.3) cm/year for true idiopathic deficiency, p less than 0.05). By current practices two patients with false idiopathic deficiency may have been distinguished by sex steroid priming. Thus post-irradiation GH deficiency seems to be permanent, but errors in diagnosis in idiopathic GH deficiency are common.
用生长激素(GH)完成治疗后,19例孤立性“特发性”GH缺乏患者和15例放疗后GH缺乏患者接受了胰岛素耐量试验或精氨酸刺激试验,或两者兼用,以重新检测GH分泌情况。放疗后GH缺乏患者包括13例患有远离下丘脑-垂体轴的中枢神经系统肿瘤患者和2例接受过颅脑或全脊髓照射的急性淋巴细胞白血病患者。15例放疗后GH缺乏患者均仍存在GH缺乏(GH峰值反应低于7 mU/l(n = 10)和7 - 15 mU/l(n = 5))。然而,在19例重新检测的特发性GH缺乏患者中,有5例(26%)的GH峰值反应大于15 mU/l(现被视为短暂性或假性特发性GH缺乏),通过治疗前人体测量和治疗后身高标准差评分,他们与其余患者(永久性或真性特发性GH缺乏,GH峰值反应低于7 mU/l(n = 12)和7 - 15 mU/l(n = 2))并无差异,但第一年身高增长速度较低(假性特发性缺乏的平均(标准差)速度为5.6(0.5)cm/年,真性特发性缺乏为8.7(1.75)cm/年,p < 0.01),且治疗期间身高增加值也较低(假性特发性缺乏的平均(标准差)增加值为2.2(1.5)cm/年,真性特发性缺乏为5.2(2.3)cm/年,p < 0.05)。按照目前的做法,两名假性特发性缺乏患者可能已通过性类固醇激发试验得以区分。因此,放疗后GH缺乏似乎是永久性的,但特发性GH缺乏的诊断错误很常见。