Isolated corticotrophin deficiency

Research output: Contribution to journalArticle

73 Citations (Scopus)

Abstract

Isolated ACTH deficiency (IAD) is a rare disorder, characterized by secondary adrenal insufficiency (AI) with low or absent cortisol production, normal secretion of pituitary hormones other than ACTH and the absence of structural pituitary defects. In adults, IAD may appear after a traumatic injury or a lymphocytic hypophysitis, the latter possibly due to autoimmune etiology. Conversely, a genetic origin may come into play in neonatal or childhood IAD. Patients with IAD usually fare relatively well during unstressed periods until intervening events spark off an acute adrenal crisis presenting with non specific symptoms, such as asthenia, anorexia, unintentional weight loss and tendency towards hypoglycemia. Blood chemistry may reveal mild hypoglycemia, hyponatremia and normal-high potassium levels, mild anemia, lymphocytosis and eosinophilia. Morning serum cortisol below 3 μg/dl are virtually diagnostic for adrenal insufficiency. whereas cortisol values comprised between 5-18 μg/dl require additional investigations: insulin tolerance test (ITT) is considered the gold standard but - when contraindicated - high or low dose-ACTH stimulation test with serum cortisol determination provides a viable alternative. Plasma ACTH concentration and prolonged ACTH infusion test are useful in differential diagnosis between primary and secondary adrenal insufficiency. For some patients with mild, near-to-asymptomatic disease, glucocorticoid replacement therapy may not be required except during stressful events; for symptomatic patients, replacement doses i.e., mean daily dose 20 mg (0.30 mg/kg) hydrocortisone or 25 mg (0.35 mg/kg) cortisone acetate, are usually sufficient. Administration of mineralocorticoids is generally not necessary as their production is maintained.

Original languageEnglish
Pages (from-to)289-295
Number of pages7
JournalPituitary
Volume9
Issue number4
DOIs
Publication statusPublished - Dec 2006

Fingerprint

Adrenocorticotropic Hormone
Hydrocortisone
Adrenal Insufficiency
Hypoglycemia
Asymptomatic Diseases
Asthenia
Addison Disease
Lymphocytosis
Mineralocorticoids
Pituitary Hormones
Hyponatremia
Eosinophilia
Anorexia
Serum
Glucocorticoids
Anemia
Weight Loss
Potassium
Differential Diagnosis
Insulin

Keywords

  • ACTH deficicency
  • Hypoglycemia
  • Hypophysitis
  • Hypopituitarism

ASJC Scopus subject areas

  • Endocrinology

Cite this

Isolated corticotrophin deficiency. / Andrioli, Massimiliano; Giraldi, Francesca Pecori; Cavagnini, Francesco.

In: Pituitary, Vol. 9, No. 4, 12.2006, p. 289-295.

Research output: Contribution to journalArticle

Andrioli, Massimiliano ; Giraldi, Francesca Pecori ; Cavagnini, Francesco. / Isolated corticotrophin deficiency. In: Pituitary. 2006 ; Vol. 9, No. 4. pp. 289-295.
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