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The metabolism of [³H]pregnenolone and [³H]dehydroepiandrosterone ([³H]DHA) by tissue from the separated zones and whole adrenal glands from newborn anencephalic infants was investigated.

Pregnenolone was metabolized by the whole gland homogenates mainly to pregnenolone sulphate and unconjugated 17-hydroxypregnenolone and also to small amounts of DHA and dehydroepiandrosterone sulphate (DHAS). DHA was metabolized by the whole gland homogenates mainly to androstenedione and DHAS and small amounts of 11β-hydroxyandrostenedione.

Adrenal gland tissue from a 42-week-old anencephalic infant containing three histologically distinct zones in the adult cortex in addition to residual foetal zone had sulphokinase, 17-hydroxylase, 17–20 lyase and 3β-hydroxy-steroid dehydrogenase/^4,5 unsaturated isomerase activity in each of these zones. These enzymes were also present in the adrenal adult zone of another anencephalic infant.

Omission of ATP from the incubation medium greatly reduced the conversion of DHA to DHAS in the homogenates and separated zones.

These results are discussed in relation to oestrogen biosynthesis in anencephaly and to foetal 4-en-3-oxosteroid synthesis in utero.