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唐氏综合征中生长激素-胰岛素样生长因子-I轴的评估

Assessment of growth hormone insulin like growth factor-I axis in Down's syndrome.

作者信息

Barreca A, Rasore Quartino A, Acutis M S, Ponzani P, Damonte G, Miani E, Balestra V, Giordano G, Minuto F

机构信息

Dipartimento di Scienze Endocrinologiche e Metaboliche, Università di Genova, Italy.

出版信息

J Endocrinol Invest. 1994 Jun;17(6):431-6. doi: 10.1007/BF03347731.

Abstract

As GH therapy has been reported to increase growth velocity in children with Down's syndrome (DS), we studied the GH-IGF-I axis in some DS patients affected by growth retardation without serious congenital malformation, malnutrition or pathological thyroid or adrenal function. IGF-I and IGF-II were evaluated in 39 patients in basal conditions. The patients were subsequently divided into two groups with respect to the IGF-I basal value: Group 1 (GR 1) consisting of patients with abnormally low basal IGF-I concentration as compared to age matched control subjects, group 2 (GR 2) consisting of patients with IGF-I in the normal range. In 6 GR 1 patients and 12 GR 2 patients we evaluated GH and IGF-I concentrations after stimulation with arginine (0.5 g/kg bw), and recombinant GH (4 IU im). In the same patients, GH radioreceptor assay and serum GH-binding protein were evaluated. In all patients IGF-II proved normal (534 +/- 23 ng/ml; mean +/- SE), while IGF-I was pathological in 36% of subjects. The cause of the defective IGF-I secretion in these patients does not seem to depend on an impaired GH axis, as no significant difference in arginine-stimulated GH peak values was seen between GR 1 (29.6 +/- 5.3 ng/ml) and GR 2 (15.1 +/- 2.24 ng/ml). IGF-I concentration evaluated 12, 24, and 48 h after arginine stimulation was significantly increased only in GR 2 patients (peak value: 0.95 +/- 0.1, p = 0.0003 vs baseline; GR 1: 0.34 +/- 0.05 U/ml).(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

由于已有报道称生长激素(GH)治疗可提高唐氏综合征(DS)患儿的生长速度,我们对一些生长发育迟缓、无严重先天性畸形、营养不良或甲状腺及肾上腺功能异常的DS患者的GH-IGF-I轴进行了研究。在基础状态下对39例患者的IGF-I和IGF-II进行了评估。随后根据IGF-I基础值将患者分为两组:第1组(GR 1),其基础IGF-I浓度与年龄匹配的对照受试者相比异常低;第2组(GR 2),其IGF-I在正常范围内。对6例GR 1患者和12例GR 2患者,我们评估了精氨酸(0.5 g/kg体重)和重组生长激素(4 IU肌肉注射)刺激后的GH和IGF-I浓度。在相同患者中,评估了GH放射受体测定和血清GH结合蛋白。所有患者的IGF-II均正常(534±23 ng/ml;平均值±标准误),而36%的受试者IGF-I异常。这些患者IGF-I分泌缺陷的原因似乎不取决于GH轴受损,因为GR 1组(29.6±5.3 ng/ml)和GR 2组(15.1±2.24 ng/ml)在精氨酸刺激后的GH峰值之间没有显著差异。仅在GR 2组患者中,精氨酸刺激后12、24和48小时评估的IGF-I浓度显著升高(峰值:0.95±0.1,与基线相比p = 0.0003;GR 1组:0.34±0.05 U/ml)。(摘要截短于250字)

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