A trial of intranasal ACTH(1-24) administration to a patient with isolated ACTH deficiency.
BACKGROUND
Isolated adrenocorticotropic hormone (ACTH) deficiency is a rare cause of secondary adrenocortical insufficiency. Normally it could be used therapeutically as an alternative to glucocorticoid treatment in these patients. We investigated the possibility of therapeutic approach as intranasal ACTH replacement therapy in patients with ACTH deficiency.


METHODS
A 32-year-old woman with general fatigue, weakness of legs and loss of consciousness due to severe hyponatremia was admitted to our hospital. Endocrinological studies showed low levels of plasma ACTH and serum cortisol with the loss of circadian rhythm. Plasma ACTH and serum cortisol levels failed to respond after intravenous injection of human corticotropin releasing hormone (hCRH), however, serum cortisol showed a blunted response to ACTH(1-24) stimulation test. She was diagnosed isolated ACTH deficiency. We performed continuous intranasal administration of ACTH(1-24) to the patient. There were no cortisol, aldosterone and dehydroepiandrosterone (DHEA) responses to a single intranasal ACTH(1-24) administration while these levels increased 6 days after intranasal treatment of ACTH(1-24).


CONCLUSIONS
These data demonstrate that adrenocortical steroids production are stimulated by intranasal administration of ACTH(1-24) in this patient with isolated ACTH deficiency. We suggest that intranasal administration of ACTH offers a therapeutic approach as ACTH replacement therapy in patients with ACTH deficiency. The latter may be more physiologic than glucocorticoid replacement.
Hiroi
Naoki
N
Division of Diabetes, Metabolism and Endocrinology, Department of Medicine, Toho University School of Medicine, 6-11-1 Omorinshi, Ota-ku, Tokyo 143-0015, Japan. n-hiroi@tkf.att.ne.jp
Ichijo
Takamasa
T
Tsuchida
Yasuyo
Y
Miyachi
Yukitaka
Y
D000281:Administration, Intranasal; D000324:Adrenocorticotropic Hormone / Q000097:blood / Q000172:deficiency*; D000328:Adult; D000450:Aldosterone / Q000097:blood; D001774:Blood Chemical Analysis; D003366:Cosyntropin / Q000008:administration & dosage* / Q000494:pharmacology; D003687:Dehydroepiandrosterone / Q000097:blood; D019314:Dehydroepiandrosterone Sulfate / Q000097:blood; D004334:Drug Administration Schedule; D005260:Female; D006403:Hematologic Tests; D006728:Hormones / Q000097:blood; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage / Q000097:blood / Q000652:urine; D016482:Urinalysis
D006728:Hormones; D003366:Cosyntropin; D003687:Dehydroepiandrosterone; D000450:Aldosterone; D019314:Dehydroepiandrosterone Sulfate; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone