A 14-yr-old native American girl from the Iroquois Nation was referred as a
potential patient with the syndrome of apparent mineralocorticoid excess.
Instead, her evaluation revealed resistance to glucocorticoids, mineralocor
ticoids, and androgens, but no resistance to vitamin D or thyroid hormones.
She lacked Cushin-goid features despite significantly high cortisol levels
. Menstruation was regular, and there was no clinical evidence of masculini
zation despite high serum androgen levels in the male range. The patient's
sister had similar clinical features. Partial resistance to exogenous gluco
corticoid and mineralocorticoid administration was well demonstrated in bot
h patients. It is proposed that these patients represent the first cases of
partial resistance to multiple steroids, possibly due to a coactivator def
ect.