GLUCOCORTICOID DEFICIENCY WITH ACHALASIA OF THE CARDIA AND LACK OF LACRIMATION
GLUCOCORTICOID DEFICIENCY WITH ACHALASIA OF THE CARDIA AND LACK OF LACRIMATION
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糖皮质激素缺乏伴贲门失弛缓症和缺乏泪液
DOI：
10.1111/j.1365-2265.1985.tb00219.x
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发表时间：
1985
影响因子：
3.2
通讯作者：
J. Peña
中科院分区：
文献类型：
--
作者：
M. Pombo;J. Devesa;A. Taborda;M. Iglesias;F. Garcia;G. J. Gaudiero;M. Jm;M. Castro‐Gago;J. Peña
最近的四份报告描述了一种多系统疾病，其中ACTH不敏感与失弛缓症和失弛缓症相关。我们报告了一名患有这种罕见三联症的男性患者的研究。患者从出生起就患有失弛缓症；孤立性糖皮质激素缺乏症在3·5岁时被诊断出来，而失弛缓症在6岁时被诊断为失弛缓症。已经提出了这种综合征可能是由于副交感神经变性所致的可能性；然而，糖皮质激素缺乏的原因尚不清楚。对其他区域的副交感神经功能进行了调查，以确定是否存在更普遍的异常。副交感神经功能的特殊心脏测试显示，患者的心脏副交感神经输入受到影响，而艾迪生儿童的相同测试是正常的。因此，我们在该综合征患者中发现了迄今为止未发现的副交感神经异常，提示该系统的一种全身性障碍。在此基础上，我们可以假设糖皮质激素衰竭可能是失去副交感神经对肾上腺输入的结果，尽管这一点仍有待实验证明。
Four recent reports describe a multisystem disorder in which ACTH insensitivity is associated with achalasia and alacrima. We report studies on a male patient with this rare triad. The patient had alacrima from birth; isolated glucocorticoid deficiency had been diagnosed at 3·5 years of age and achalasia at age 6. The possibility that this syndrome could be due to a parasympathetic degeneration has already been proposed; the cause of the glucocorticoid deficiency, however, remains unclear. Parasympathetic function in other areas was investigated to determine whether there might be a more generalized abnormality. Specific cardiac tests of parasympathetic function showed that parasympathetic input to the heart was affected in the patient, while the same tests in an Addisonian child were normal. We show, then, a hitherto undetected parasympathetic abnormality in a patient with this syndrome, suggesting a generalized disturbance of this system. On this basis we may hypothesize that the glucocorticoid failure may be a consequence of the loss of parasympathetic input to the adrenal gland, although this remains to be demonstrated experimentally.