儿童垂体巨人症和早熟肾上腺素:生长激素和/或PRL提前肾上腺素的发作?

N Iwatani, M Kodama, H Seto
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引用次数: 12

摘要

我们报告一位患有垂体巨人症及肾上腺早衰的女童。从两岁开始,她就表现出异常的过度生长,5岁时,她身高133.5厘米(+ 5.5 SD),体重40.5公斤。她的早熟表现为阴毛、寻常性痤疮、多毛症和骨质老化。内分泌检查显示血清生长激素(GH)和催乳素(PRL)明显升高,这与TRH测试相矛盾。此外,血清脱氢表雄酮(DHA)及其硫酸盐(DHAS)浓度升高至成人水平,并随ACTH变化而变化,提示这些雄激素是由肾上腺功能分泌的。这些雄激素似乎是她部分早熟的原因。先前的报告表明,生长激素和/或PRL的过量生产可能在肾上腺素的诱导中起作用。此外,在以往报道的9例10岁以下巨人症患者中,有8例表现为肾上腺早衰。需要进一步研究GH和PRL对垂体巨人症患儿肾上腺雄激素分泌的影响。另一方面,对于生长激素分泌正常的矮个子儿童,应注意儿童早期的生长激素治疗是否会诱发肾上腺素早熟。
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A child with pituitary gigantism and precocious adrenarche: does GH and/or PRL advance the onset of adrenarche?

We describe a female child with pituitary gigantism and precocious adrenarche. From two years of age she showed unusual overgrowth, and at 5 years old she was 133.5 cm (+ 5.5 SD) tall and weighed 40.5 kg. Her precocious manifestations were public hair, acne vulgaris, hirsutism, and advanced bone age. Endocrinological examination revealed markedly increased serum growth hormone (GH) and prolactin (PRL), which responded paradoxically to a TRH test. In addition, the concentrations of serum dehydroepiandrosterone (DHA) and its sulfate (DHAS) were increased to adult levels, moving in accordance with changes in ACTH, which suggested that these androgens were secreted from the adrenal glands functionally. These androgens seemed to be responsible for her partial precocity. Prior reports have suggested that GH and/or PRL overproduction might have played a role in the induction of adrenarche. Also, in previous reports of 9 gigantism patients under 10 years old, the manifestation of precocious adrenarche was suggested in 8. Further investigation of the influence of GH and PRL on adrenal androgen production in children with pituitary gigantism is required. On the other hand, in short children with normal GH secretion, attention should be paid to whether or not the GH therapy in early childhood induces precocious adrenarche.

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