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[日本孤立性促肾上腺皮质激素缺乏患者促甲状腺激素和催乳素的高反应性及生长激素反应受损]

[Hyperresponsiveness of TSH and prolactin and impaired responsiveness of GH in Japanese patients with isolated ACTH deficiency].

作者信息

Hashimoto K, Nishioka T, Iyota K, Nakayama T, Itoh H, Takeda K, Takamatsu K, Numata Y, Ogasa T

机构信息

Second Department of Internal Medicine, Kochi Medical School.

出版信息

Nihon Naibunpi Gakkai Zasshi. 1992 Oct 20;68(10):1096-111. doi: 10.1507/endocrine1927.68.10_1096.

Abstract

Two hundred and forty-one cases of isolated ACTH deficiency have been reported in Japan since 1969. Pituitary hormone responsiveness to stimulation tests before and after hydrocortisone supplementation was investigated in these cases. Plasma ACTH level showed no or little change in response to lysine vasopressin, metyrapone, CRF or insulin-induced hypoglycemia in 97.3-100% of the cases. Serum GH level changed little or not at all in response to GRF, insulin-induced hypoglycemia, glucagon, 1-dopa and arginine in 26.9, 29.3, 40.0, 50.0 and 56.1%, respectively. Serum TSH and prolactin (PRL) levels showed hyperresponse to TRH in 34.7 and 35.6%, respectively. After hydrocortisone therapy, GH secretion was more responsive than before therapy in 78.9% of the cases. After supplementation, TSH level was less responsive to TRH stimulation than before therapy in 59.3% of the cases. After hydrocortisone supplementation, TSH response to TRH decreased in 75% of ACTH-deficient patients without primary hypothyroidism but did not decrease in more than half of those with primary hypothyroidism. TSH response to TRH decreased after supplementation in 76.5% of the patients with TSH hyperresponsiveness before therapy, and increased after therapy in 66.7% of those with normal TSH responses before therapy. After supplementation, PRL response to TRH was less than that before therapy in 43.5% of ACTH--deficient patients, and greater than that before therapy in 30.4%. PRL response to TRH decreased after therapy in 66.7% of the patients with PRL hyperresponsiveness before therapy, and increased in 63.6% of those with normal PRL response before therapy. Primary hypothyroidism and Hashimoto's thyroiditis were complicated in 21.6 and 11.6%, respectively, of the 241 patients with isolated ACTH deficiency. In patients who had TSH hyperresponsiveness and/or high basal TSH levels and PRL hyperresponsiveness and/or high basal PRL levels, primary hypothyroidism was complicated in 58.4 and 42.3%, respectively. Hashimoto's thyroiditis was complicated in 29.8 and 20.5%, respectively, of these patients. Pituitary cell antibody (PCA) was detected in 36.6% of ACTH-deficient patients who were examined. Pituitary cell surface antibody (PCSA) to AtT-20 cells and GH3 cells was detected in 50.0 and 28.0% of the examined cases, respectively. The prevalence of PCA and PCSA did not differ between TSH-hyperresponsive patients and those with normal TSH basal levels and response, whereas PCA and PCSA were significantly more prevalent in PRL-hyperresponsive patients than in those with normal PRL levels and response. An empty sella was found in 30.2% of the examined case.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

自1969年以来,日本已报告241例孤立性促肾上腺皮质激素(ACTH)缺乏症病例。对这些病例补充氢化可的松前后垂体激素对刺激试验的反应性进行了研究。在97.3% - 100%的病例中,血浆ACTH水平对赖氨酸加压素、甲吡酮、促肾上腺皮质激素释放因子(CRF)或胰岛素诱导的低血糖反应无变化或变化很小。血清生长激素(GH)水平对生长激素释放因子(GRF)、胰岛素诱导的低血糖、胰高血糖素、左旋多巴和精氨酸的反应分别在26.9%、29.3%、40.0%、50.0%和56.1%的病例中变化很小或无变化。血清促甲状腺激素(TSH)和催乳素(PRL)水平分别在34.7%和35.6%的病例中对促甲状腺激素释放激素(TRH)呈高反应。氢化可的松治疗后,78.9%的病例中GH分泌比治疗前更有反应。补充后,59.3%的病例中TSH水平对TRH刺激的反应性比治疗前降低。在无原发性甲状腺功能减退的ACTH缺乏患者中,75%补充氢化可的松后TSH对TRH的反应降低,但在半数以上原发性甲状腺功能减退患者中未降低。治疗前TSH高反应性的患者中,76.5%补充后TSH对TRH的反应降低,治疗前TSH反应正常的患者中,66.7%治疗后反应增加。补充后,43.5%的ACTH缺乏患者PRL对TRH的反应低于治疗前,30.4%高于治疗前。治疗前PRL高反应性的患者中,66.7%治疗后PRL对TRH的反应降低,治疗前PRL反应正常的患者中,63.6%治疗后反应增加。241例孤立性ACTH缺乏患者中,分别有21.6%和!1.6%合并原发性甲状腺功能减退和桥本甲状腺炎。在TSH高反应性和/或基础TSH水平高以及PRL高反应性和/或基础PRL水平高的患者中,分别有58.4%和42.3%合并原发性甲状腺功能减退。这些患者中分别有29.8%和20.5%合并桥本甲状腺炎。在接受检查的ACTH缺乏患者中,36.6%检测到垂体细胞抗体(PCA)。分别在50.0%和28.0%的受检病例中检测到针对AtT - 20细胞和GH3细胞的垂体细胞表面抗体(PCSA)。TSH高反应性患者与TSH基础水平和反应正常的患者之间PCA和PCSA的患病率无差异,而PRL高反应性患者中PCA和PCSA的患病率显著高于PRL水平和反应正常的患者。在30.2%的受检病例中发现空蝶鞍。(摘要截于400字)

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