Berberoğlu Merih, Sıklar Zeynep, Darendeliler Feyza, Poyrazoğlu Sükran, Darcan Sükran, Işgüven Pınar, Bideci Aysun, Ocal Gönül, Bundak Rüveyde, Yüksel Bilgin, Arslanoğlu Ilknur
Ankara University School of Medicine, Department of Pediatrics, Division of Pediatric Endocrinology, Ankara, Turkey.
J Clin Res Pediatr Endocrinol. 2008;1(1):30-7. doi: 10.4008/jcrpe.v1i1.7. Epub 2008 Aug 5.
Reconfirming the diagnosis of childhood onset growth hormone deficiency (GHD) in young adults is necessary to demonstrate the need for continuation of GH therapy.
This nationally-based study was planned to establish GH status during adulthood in childhood-onset GH deficient patients and to evaluate factors that would predict persistency of the GHD.
In this multicenter study, 70 GH deficient patients who had reached final height were evaluated after completion of GH treatment. Fifty-two patients (74%) had isolated GHD and 18 patients (26%) had multiple pituitary hormone deficiency (MPHD). Patients who had reached final height and the pubertal Tanner stage 5 were reevaluated for GH status. After at least 6 weeks of cessation of GH treatment, patients were retested with insulin induced hypoglycemia.
GHD was found to be transient in 64.3% of all patients. Of the isolated GH deficient patients 82.7% had transient GHD, whereas 88.9% of the MPHD patients showed persistent GHD. Comparison of isolated GH deficient and multiple hormone deficient patients indicated higher peak GH, IGF-I and IGFBP-3 levels in isolated GH deficient patients. No parameter was significantly different in the transiently and persistently GH deficient patients with respect to gender. Although specificity of IGF-I value of less than -2 SD showing persistency of GHD was lower than the specificity of IGFBP-3 value of less than -2 SD (65.7% vs 84%), negative predictive values were similar for the two parameters (85.2% and 84%, respectively).
Most of the cases of childhood onset GHD are idiopathic and the GHD is transient. In patients with MPHD, GHD is generally permanent. Low IGF-I and IGFBP-3 levels are supporting findings to show persistency of the GHD.
重新确认年轻成人期儿童期起病的生长激素缺乏症(GHD)的诊断对于证明继续进行生长激素(GH)治疗的必要性至关重要。
这项基于全国范围的研究旨在确定儿童期起病的GH缺乏症患者成年期的GH状态,并评估可预测GHD持续性的因素。
在这项多中心研究中,70例已达到最终身高的GH缺乏症患者在GH治疗结束后接受评估。52例患者(74%)为孤立性GHD,18例患者(26%)为多发性垂体激素缺乏症(MPHD)。对已达到最终身高且处于青春期坦纳5期的患者进行GH状态的重新评估。在停止GH治疗至少6周后,患者接受胰岛素诱发低血糖试验重新检测。
发现所有患者中64.3%的GHD为暂时性。在孤立性GH缺乏症患者中,82.7%有暂时性GHD,而MPHD患者中88.9%表现为持续性GHD。孤立性GH缺乏症患者与多发性激素缺乏症患者的比较表明,孤立性GH缺乏症患者的GH峰值、胰岛素样生长因子-I(IGF-I)和胰岛素样生长因子结合蛋白-3(IGFBP-3)水平更高。暂时性和持续性GH缺乏症患者在性别方面没有显著差异。尽管IGF-I值低于-2标准差显示GHD持续性的特异性低于IGFBP-3值低于-2标准差的特异性(65.7%对84%),但这两个参数的阴性预测值相似(分别为85.2%和84%)。
大多数儿童期起病的GHD病例为特发性,且GHD为暂时性。在MPHD患者中,GHD通常是永久性的。低IGF-I和IGFBP-3水平是支持GHD持续性的发现。