抄録
The prevalence of the syndrome of ACTH-ADH discharge in Japan was studied by means of a questionnaire to all members of the Japanese Society for Pediatric Endocrinology. Ninety-one patients were reported by 107 members, and clinical details of 46 cases were obtained. The age of the patients was widely distributed, from one to 15 yrs (median, eight yrs), while the age of onset was usually between zero and four yrs, indicating a long duration of the symptoms. Eight patients had various congenital diseases. As for the three cardinal symptoms of vomiting, hypertension and psychotic depression, five cases (10.8%) did not have hypertension. Association of autonomic symptoms was common (85%). Laboratory data showed a consistent rise in ACTH and ADH at the initial phase of the attack, whereas hyponatremia did not occur in 30% of patients. Elevation of plasma norepinephrine and a concomitant decrease of dopamine were also constantly found. Eleven (26.8%) of 41 cases had an abnormal EEG, showing high voltage slow bursts. A wide variety of drugs was used for treatment, but the most effective were anticonvulsants, especially phenytoin, while antagonists of catecholamine were only transiently effective. These data indicate that (1) the syndrome of ACTH-ADH discharge is a fairly common disorder in Japan, (2) it is a quite different syndrome from cyclic vomiting in childhood, (3) the symptoms last for a long time, in some cases beyond adolescence, and (4) the most effective drugs are anticonvulsants. It is suggested that this syndrome may be due to periodic epileptic discharge in the hypothalamus involving paraventricular CRH- and ADH- neurons and the emetic center in the medulla.
