Abstract
The urinary neutral 17-ketosteroids of eleven patients with congenital adrenal hyperplasia, of which 3 were of hypertensive form and 8 were of simple virilizing from, were fractionated on a gradient elution chromatographic column.The urinary 17-ketosteroid excretion patterns seen in the first family members, consisting of parents, two unaffected children, two affected children and a patient's female baby, and seen in the second family members, of parents, an unaffected child and two affected children were also studied.The findings are summerized as follows : 1) The urinary excretion of 11-deoxy-17-ketosteroids, especially of both androsterone and etiocholanolone were elevated in all eleven patients with congenital adrenal hyperplasia, but the excretion of dehydroisoandrosterone was elevated in only two cases of the simple virilizing form. In the simple virilizing from, the excretion of 11β-hydroxyandrosterone and 11-oxoetiocholanolone was elevated in all cases, whereas that of 11β-hydroxyetiocholanolone varied by cases, being normal, below normal or even zero in some instances. In the hypertensive form, the excretion values of individual 11-oxy-genated 17-ketosteroids were below normal or normal and therefore a significant decrease in the ratio of 11-oxygenated 17-ketosteroids to 11-deoxy-17-ketosteroids was observed.2) An abnormal fraction, which is eluated into the more polar fraction than that of 11β-hydroxyetiocholanolone on the author's gradient elution chromatogram, was observed in the majority of patients with congenital adrenal hyperplasia of simple virilizing form. Eluant of this fraction indicates no characteristics of 17-ketosteroid by the Zimmermann reaction. This fraction was not found on the chromatogram in the patients of hypertensive form.3) After zinc-Corticotropin stimulation in two cases of the simple virilizing form, increased excretion of individual 11-deoxy-17-ketosteroids and less increased excretion of individual 11-oxygenated 17-ketosteroids were seen. With SU4885 administration in four cases of this form, variable increases in the excretion of 11-deoxy-17-ketosteroids and remarkable decreases in the excretion of 11-oxygenated 17-ketosteroids, especially of 11β-hydroxyandrosterone and 11-oxoetiocholanolone, were observed.4) A patient with congenital adrenal hyperplasia of simple virilizing form who became pregnant showed no definite increase in excretion of both androsterone and etiocholanolone during gestation and after delivery although glucocorticoid administration was omitted when the patient was known to be pregnant.5) In the first family, all members except two affected children showed normal urinary excretion for total 17-ketosteroid. However, the father showed an abnormally high 11-oxy-AND/11-oxy-ETIO ratio and a sister showed an increased excretion of predehydroisoandrosterone fraction. The mother and a patient's female baby showed a normal excretion pattern of all 17-ketosteroids. In the second family, the father, mother and a brother showed a normal excretion pattern of all 17-ketosteroids and normal responses to SU4885 administration.
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