[Studies on the metabolic abnormality of cortisol and corticosterone in a case of dexamethasone responsive mineralocorticoid excess (author's transl)].

[Studies on the metabolic abnormality of cortisol and corticosterone in a case of dexamethasone responsive mineralocorticoid excess (author's transl)].
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地塞米松反应性盐皮质激素过量所致皮质醇和皮质酮代谢异常的研究(作者译)。

DOI:
10.1507/endocrine1927.55.10_1341
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发表时间:
1979
期刊:
Nihon Naibunpi Gakkai zasshi
影响因子:
--
通讯作者:
H. Kawaguchi
H. Kawaguchi
中科院分区:
--
文献类型:
--
作者:
Y. Igarashi;S. Egi;A. Takehiro;T. Ohzeki;H. Kawaguchi

文献摘要

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In a case of dexamethasone responsive mineralocorticoid excess, we found a peculiar phenomenon; namely, that the administration of cortisol or cortisone in a replacement dose produced apparent hypokalemia even under dexamethasone suppression. The clinical course of this Japanese girl is described in Table 1. At the age of three years, a laparotomy was performed under the diagnosis of Conn's syndrome, but no adrenal tumor was found on either side, and subtotal adrenalectomy was done bilaterally. Microscopic findings of both adrenals showed a disarrangement of adrenocortical zonation, and the initial stage of adrenal hyperplasia was suspected. One month after surgery, hypertension and hypokalemia reappeared. The administration of dexamethasone was dramatically effective in suppressing the symptoms of mineralocorticoid excess. At the age of 13 years, sexual maturation and ovarial estrogen secretion were normal and a regular menstrual cycle was observed. From these clinical pictures and the results of steroid secretion studies, the 17-alpha-hydroxylase deficiency syndrome was ruled out. In an attempt to alter the therapeutic agent from dexamethasone to natural glucocorticoid, it was found that a single