Following the previous report, the author intended, in this study, to investigate how the adrenal cortex of children with various diseases respond to the administration of adrenocorticotropin (ACTH). The subjects consisted of 32 cases with endocrine or metabolic diseases, 11 cases with central nervous disease and 22 cases who were previously treated with corticosteroids. Daily doses of 20 units ACTH-Z were given intramuscularly for 2 to 5 days, and the urinary total 17-hydroxycorticosteroids (17-OHCS) excretion was determined. The results were compared with those obtained from the healthy infants and children which were described in the previous report.
1) Diseases of endocrine and metabolism i) Dwarfism-The response in two of six examined cases with pituitary dwarfism showed the prolonged type (prolonged response). In a patient with progeria no significant elevation of urinary 17-OHCS excretion was observed during the administration of ACTH. However, such abnormal responses were observed in none of six cases with primordial dwarfism.
ii) Obesity-The response in three of eight cases with simple obesity showed the excessive type (excessive response). On the contrary, two of six cases with Laurence-Moon-Biedl syndrome showed the prolonged response.
iii) Miscellaneous Diseases-No abnormal response was obtained in the cases of chondrodystrophia foetalis (two cases), gargoylism (two cases) and leucine sensitive hypoglycemia (one case).
2) Diseases of central nervous system i) Cerebral palsy-Two of six cases examined showed the weak response, which the author described as the “transitory” type. Other types of abnormal response, such as the prolonged response, excessive response were also obtained in this disease.
ii) Brain tumor-One of five cases examined showed the prolonged response and the other one showed the excessive response.
3) Children with a previous administration of corticosteroids. Nine of twenty-two cases examined showed the prolonged response. No significant relation between the appearance of this response and the ages of the subjects was found.
4) The adrenocortical responsiveness of children, subjected for the study, was commented according to the above-mentioned results.