A CASE OF AN ISOLATED ADRENOCORTICOTROPIN (ACTH) DEFICIENCY : RESOLVED AND DELIVERED A CHILD AFTER 8 YEARS OF TREATMENT

Abstract

We describe a 19-year-old Japanese woman with isolated ACTH deficiency who improved and delivered a child after 8 years hydrocortisone replacement therapy. Menarche was at the age of 13 y, and menstruation continued regularly. At the age of 16 y general fatigue and amenorrhea developed, and she was referred to our hospital. Her plasma ACTH and cortisol levels were low, and ACTH provocation tests revealed no response. Although the plasma ACTH and cortisol levels were low, dexamethasone suppressed them. Results of other pituitary function tests were normal. Autoantibodies against ACTH-producing cells were identified. She was diagnosed with isolated ACTH deficiency, and we initiated hydrocortisone replacement therapy. Her general 6ondition and anemia were improved, but the amenorrhea persisted. Seven years later, the hydrocortisone was tapered off and menstruation started again. At that time, the test for pituitary autoantibody was negative. One year after fisishl'ng hydrocortisone replacement therapy, she gave birth to a healthy child by natural delivery. This was an atypical case of isolated ACTH deficiency because the ACTH levels were not responsive to provocation tests but were suppressed by dexamethasone. We suggest that the deficiency of ACTH secretion was functional and transient due to the autoimmune mechanism.