Feuillan P P, Jones J, Ross J L
Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA.
J Clin Endocrinol Metab. 1995 Apr;80(4):1357-60. doi: 10.1210/jcem.80.4.7714111.
GH every 20 min for 24 h, insulin-like growth factor I (IGF-I), IGF-binding protein 3, and estradiol (E2) were measured in a 7.3-yr-old girl with precocious puberty due to McCune-Albright syndrome (MAS) who developed stigmata of early acromegaly and in 9 other MAS patients who had no signs of acromegaly. To determine whether the MAS patients had subtle abnormalities in GH secretion, a computerized pulse analysis program was used to compare the MAS data with those from 27 control girls with central precocious puberty who had a similar rate of bone age advance, E2, and body mass index. We found no differences in mean GH, GH pulse frequency, pulse height, or pulse area between MAS patients and controls except in patient 1, who had an elevated mean +/- SD GH compared with controls (15.4 +/- 2 vs. 4.8 +/- 2.3 micrograms/L; P < 0.01) and an elevated IGF-I (908 micrograms/L) and IGF-binding protein 3 (5.6 mg/L). None of the GH parameters correlated with body mass index, age, bone age, or E2 levels in either group. The serum GH in patient 1 fell to near-undetectable levels from 60-180 min after a 100-micrograms sc dose of long-acting somatostatin, confirming that this form of therapy can be effective in cases of GH hypersecretion due to MAS.
对一名因McCune-Albright综合征(MAS)导致性早熟且出现早期肢端肥大症体征的7.3岁女孩以及另外9名无肢端肥大症体征的MAS患者,每20分钟测定一次生长激素(GH),持续24小时,并检测胰岛素样生长因子I(IGF-I)、IGF结合蛋白3和雌二醇(E2)。为确定MAS患者在GH分泌方面是否存在细微异常,使用计算机化脉冲分析程序将MAS患者的数据与27名中枢性性早熟对照女孩的数据进行比较,这些对照女孩的骨龄进展速度、E2和体重指数相似。我们发现,除患者1外,MAS患者与对照在平均GH、GH脉冲频率、脉冲高度或脉冲面积方面无差异。患者1的平均±标准差GH水平高于对照(15.4±2 vs. 4.8±2.3微克/升;P<0.01),IGF-I(908微克/升)和IGF结合蛋白3(5.6毫克/升)也升高。两组中,GH参数均与体重指数、年龄、骨龄或E2水平无关。皮下注射100微克长效生长抑素后60至180分钟,患者1的血清GH降至几乎检测不到的水平,证实这种治疗方法对MAS所致GH分泌过多有效。