尿17-ketogenic steroid分画の臨床的研究

第3編 健康人並びに各種内分泌疾患におけるSU-4885負荷による脳下垂体予備能試験について

抄録

In normal adults, administration of SU-4885 3.0 gm caused an increase of urinary 17-KGS and 11-deoxy 17-KGS to 9.0-22.0 mg/day and 7.0-20.0 mg/day, respectively on the first day after the administration of SU-4885, but did not cause any decrease of 11-oxy 17-KGS. Therefore, 11-deoxy/11-oxy became higher, 1.80-4.00. In the aged, the increase of 11-deoxy 17-KGS was greater than that in adults,. consequently showing a remarkable elevation of 11-deoxy/11-oxy. This finding was similar to the results observed in ACTH-Z test.
One among 5 cases with adrenogenital syndrome showed no reaction at all, revealing a very low reserve of pituitary function, but the other 4 cases showed a remarkable increase of 11-deoxy 17-KGS. This fact indicates that the block of 21-hydroxylation enzyme system is incomplete and many cases with this disorder still retain a sufficient reserve of pituitary function. There was observed a person who had no physical sign of adrenogenital syndrome, although his two children showed typical adrenogenital syndromes, and revealed unusually high 11-deoxy/11-oxy in urine at resting state and showed excessive reaction in SU-4885 test which was similar to that of his children. This is not a phenotype of adrenogenital syndrome, but a suspected genotype. In that event, it is interesting to note that some genotype of adrenogenital syndrome without any of its clinical sign shows similar endocrinological disturbances to the genotype.
Of patients with Cushing's syndrome, those due to adrenal hyperplasia revealed all excessive reaction in the test and those due to adrenal adenoma showed normal or decreased reaction in the test, and thus the differentiation in them was made possible by the test. Decreased reaction was observed in the test in cases with panhypopituitarism, acromegaly or myxedema.
In the cases showing positive reaction in the test, decrease of 11-oxy 17-KGS was frequently observed. Therefore, 11-deoxy 17-KGS was found to be the most reliable and specific index for the positive reaction to SU-4885. In general, 11-deoxy/11-oxy seems to be closely related to the degree of block of 11-hydroxylation by SU-4885. This is supported by the fact that clear reversed correlation was observed between the maximum value of 11-deoxy/11-oxy in SU-4885 test and the maximum value of urinary 17-KGS in ACTH-Z test.