Pessoa de Queiroz Andréa Noronha, Collett-Solberg Paulo F, Cardoso Monique Esteves, Jusan Rafaela Cattan, Vaisman Mario, Guimarães Marília Martins
Serviço de Endocrinologia da Faculdade de Medicina, Universidade Federal do Rio de Janeiro - UFRJ, Rio de Janeiro, RJ, Brazil.
Growth Horm IGF Res. 2007 Jun;17(3):254-60. doi: 10.1016/j.ghir.2007.03.003. Epub 2007 May 30.
The pathophysiology of the short stature in girls with Turner syndrome (TS) is not well understood. The "IGF-I generation test" is used to assess the sensitivity to growth hormone. We compared the biochemical response to four days of growth hormone of TS and controls.
Pre-pubertal TS were recruited to participate in the study. Their siblings served as controls. IGF-I, IGFBP-3 and ALS were measured before and 5 days after using hGH (0.05mg/kg/day). Student-t test was used to compare the differences in their responses.
Eleven TS (mean age of 8.5+/-2.4) and 11 siblings (6 females and 5 males) (mean age of 7.0+/-2.0) participated in the study. The basal serum levels of IGF-I, IGFBP-3 and ALS were normal and not different between groups (p=0.62 for IGF-I, p=0.91 for IGFBP-3 and p=0.51 for ALS). The IGF-I generation test was positive in all controls and in 10/11 TS. The IGFBP-3 generation test was positive in 6/11 controls and 4/11 TS. After hGH the mean IGFBP-3 was lower in TS than in controls (p=0.08). The ALS response to hGH was not uniform between groups.
The IGF-I and ALS generation test results were not different between controls and TS. The IGFBP-3 results were higher in the control group but more than 50% of tested children did not pass. The IGF-I/IGFBP-3 generation tests, as presently done, did not help in the understanding of the short stature in TS. The use of different GH dosages and number of doses need to be investigated.
特纳综合征(TS)女童身材矮小的病理生理学机制尚未完全明确。“IGF-I生成试验”用于评估对生长激素的敏感性。我们比较了TS患者和对照组对四天生长激素治疗的生化反应。
招募青春期前的TS患者参与研究。其兄弟姐妹作为对照。在使用hGH(0.05mg/kg/天)前及使用后5天测量IGF-I、IGFBP-3和ALS。采用学生t检验比较其反应差异。
11例TS患者(平均年龄8.5±2.4岁)和11名兄弟姐妹(6名女性和5名男性)(平均年龄7.0±2.0岁)参与了研究。IGF-I、IGFBP-3和ALS的基础血清水平正常,组间无差异(IGF-I为p=0.62,IGFBP-3为p=0.91,ALS为p=0.51)。所有对照组和11例TS患者中的10例IGF-I生成试验呈阳性。11例对照组中的6例和11例TS患者中的4例IGFBP-3生成试验呈阳性。使用hGH后,TS患者的平均IGFBP-3低于对照组(p=0.08)。两组间ALS对hGH的反应不一致。
对照组和TS患者的IGF-I和ALS生成试验结果无差异。对照组的IGFBP-3结果较高,但超过50%的受试儿童未通过。目前进行的IGF-I/IGFBP-3生成试验无助于理解TS患者的身材矮小。需要研究使用不同的生长激素剂量和给药次数。