U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Adrenocorticotropin deficient adrenal insufficiency

MedGen UID:
868801
•Concept ID:
C4023211
•
Disease or Syndrome
Synonym: ACTH deficient adrenal insufficiency
 
HPO: HP:0011735

Definition

Adrenal insufficiency secondary to a defect in ACTH production. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdrenocorticotropin deficient adrenal insufficiency

Professional guidelines

PubMed

Guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency: Guidelines from the Association of Anaesthetists, the Royal College of Physicians and the Society for Endocrinology UK.
Woodcock T, Barker P, Daniel S, Fletcher S, Wass JAH, Tomlinson JW, Misra U, Dattani M, Arlt W, Vercueil A
Anaesthesia 2020 May;75(5):654-663. Epub 2020 Feb 3 doi: 10.1111/anae.14963. PMID: 32017012
Guidelines for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in critically ill patients (Part I): Society of Critical Care Medicine (SCCM) and European Society of Intensive Care Medicine (ESICM) 2017.
Annane D, Pastores SM, Rochwerg B, Arlt W, Balk RA, Beishuizen A, Briegel J, Carcillo J, Christ-Crain M, Cooper MS, Marik PE, Umberto Meduri G, Olsen KM, Rodgers S, Russell JA, Van den Berghe G
Intensive Care Med 2017 Dec;43(12):1751-1763. Epub 2017 Sep 21 doi: 10.1007/s00134-017-4919-5. PMID: 28940011
Addison disease: early detection and treatment principles.
Michels A, Michels N
Am Fam Physician 2014 Apr 1;89(7):563-8. PMID: 24695602

Recent clinical studies

Etiology

Hypoglycaemia in adrenal insufficiency.
Lee SC, Baranowski ES, Sakremath R, Saraff V, Mohamed Z
Front Endocrinol (Lausanne) 2023;14:1198519. Epub 2023 Nov 20 doi: 10.3389/fendo.2023.1198519. PMID: 38053731Free PMC Article
Guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency: Guidelines from the Association of Anaesthetists, the Royal College of Physicians and the Society for Endocrinology UK.
Woodcock T, Barker P, Daniel S, Fletcher S, Wass JAH, Tomlinson JW, Misra U, Dattani M, Arlt W, Vercueil A
Anaesthesia 2020 May;75(5):654-663. Epub 2020 Feb 3 doi: 10.1111/anae.14963. PMID: 32017012
Primary adrenal insufficiency in children: Diagnosis and management.
Kirkgoz T, Guran T
Best Pract Res Clin Endocrinol Metab 2018 Aug;32(4):397-424. Epub 2018 Jun 6 doi: 10.1016/j.beem.2018.05.010. PMID: 30086866
Guidelines for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in critically ill patients (Part I): Society of Critical Care Medicine (SCCM) and European Society of Intensive Care Medicine (ESICM) 2017.
Annane D, Pastores SM, Rochwerg B, Arlt W, Balk RA, Beishuizen A, Briegel J, Carcillo J, Christ-Crain M, Cooper MS, Marik PE, Umberto Meduri G, Olsen KM, Rodgers S, Russell JA, Van den Berghe G
Intensive Care Med 2017 Dec;43(12):1751-1763. Epub 2017 Sep 21 doi: 10.1007/s00134-017-4919-5. PMID: 28940011
Pituitary Apoplexy.
Briet C, Salenave S, Bonneville JF, Laws ER, Chanson P
Endocr Rev 2015 Dec;36(6):622-45. Epub 2015 Sep 28 doi: 10.1210/er.2015-1042. PMID: 26414232

Diagnosis

Adrenal insufficiency.
Husebye ES, Pearce SH, Krone NP, Kämpe O
Lancet 2021 Feb 13;397(10274):613-629. Epub 2021 Jan 20 doi: 10.1016/S0140-6736(21)00136-7. PMID: 33484633
Guidelines for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in critically ill patients (Part I): Society of Critical Care Medicine (SCCM) and European Society of Intensive Care Medicine (ESICM) 2017.
Annane D, Pastores SM, Rochwerg B, Arlt W, Balk RA, Beishuizen A, Briegel J, Carcillo J, Christ-Crain M, Cooper MS, Marik PE, Umberto Meduri G, Olsen KM, Rodgers S, Russell JA, Van den Berghe G
Intensive Care Med 2017 Dec;43(12):1751-1763. Epub 2017 Sep 21 doi: 10.1007/s00134-017-4919-5. PMID: 28940011
Pituitary Apoplexy.
Briet C, Salenave S, Bonneville JF, Laws ER, Chanson P
Endocr Rev 2015 Dec;36(6):622-45. Epub 2015 Sep 28 doi: 10.1210/er.2015-1042. PMID: 26414232
Central hypoadrenalism.
Crowley RK, Argese N, Tomlinson JW, Stewart PM
J Clin Endocrinol Metab 2014 Nov;99(11):4027-36. Epub 2014 Aug 20 doi: 10.1210/jc.2014-2476. PMID: 25140404
Addison disease: early detection and treatment principles.
Michels A, Michels N
Am Fam Physician 2014 Apr 1;89(7):563-8. PMID: 24695602

Therapy

Hypoglycaemia in adrenal insufficiency.
Lee SC, Baranowski ES, Sakremath R, Saraff V, Mohamed Z
Front Endocrinol (Lausanne) 2023;14:1198519. Epub 2023 Nov 20 doi: 10.3389/fendo.2023.1198519. PMID: 38053731Free PMC Article
Guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency: Guidelines from the Association of Anaesthetists, the Royal College of Physicians and the Society for Endocrinology UK.
Woodcock T, Barker P, Daniel S, Fletcher S, Wass JAH, Tomlinson JW, Misra U, Dattani M, Arlt W, Vercueil A
Anaesthesia 2020 May;75(5):654-663. Epub 2020 Feb 3 doi: 10.1111/anae.14963. PMID: 32017012
Guidelines for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in critically ill patients (Part I): Society of Critical Care Medicine (SCCM) and European Society of Intensive Care Medicine (ESICM) 2017.
Annane D, Pastores SM, Rochwerg B, Arlt W, Balk RA, Beishuizen A, Briegel J, Carcillo J, Christ-Crain M, Cooper MS, Marik PE, Umberto Meduri G, Olsen KM, Rodgers S, Russell JA, Van den Berghe G
Intensive Care Med 2017 Dec;43(12):1751-1763. Epub 2017 Sep 21 doi: 10.1007/s00134-017-4919-5. PMID: 28940011
Pituitary Apoplexy.
Briet C, Salenave S, Bonneville JF, Laws ER, Chanson P
Endocr Rev 2015 Dec;36(6):622-45. Epub 2015 Sep 28 doi: 10.1210/er.2015-1042. PMID: 26414232
Addison's disease.
Nieman LK, Chanco Turner ML
Clin Dermatol 2006 Jul-Aug;24(4):276-80. doi: 10.1016/j.clindermatol.2006.04.006. PMID: 16828409

Prognosis

A Contemporary Approach to the Diagnosis and Management of Adrenal Insufficiency.
Charoensri S, Auchus RJ
Endocrinol Metab (Seoul) 2024 Feb;39(1):73-82. Epub 2024 Jan 22 doi: 10.3803/EnM.2024.1894. PMID: 38253474Free PMC Article
Familial Glucocorticoid Deficiency: the changing landscape of an eponymous syndrome.
Maharaj AV
Front Endocrinol (Lausanne) 2023;14:1268345. Epub 2023 Dec 21 doi: 10.3389/fendo.2023.1268345. PMID: 38189052Free PMC Article
The impact of transsphenoidal surgery on pituitary function in patients with non-functioning macroadenomas.
Mavromati M, Mavrakanas T, Jornayvaz FR, Schaller K, Fitsiori A, Vargas MI, Lobrinus JA, Merkler D, Egervari K, Philippe J, Leboulleux S, Momjian S
Endocrine 2023 Aug;81(2):340-348. Epub 2023 May 24 doi: 10.1007/s12020-023-03400-z. PMID: 37222882Free PMC Article
Pitfalls in the interpretation of the cosyntropin stimulation test for the diagnosis of adrenal insufficiency.
Burgos N, Ghayee HK, Singh-Ospina N
Curr Opin Endocrinol Diabetes Obes 2019 Jun;26(3):139-145. doi: 10.1097/MED.0000000000000473. PMID: 30855285
Group 1. Epidemiology of primary and secondary adrenal insufficiency: Prevalence and incidence, acute adrenal insufficiency, long-term morbidity and mortality.
Chabre O, Goichot B, Zenaty D, Bertherat J
Ann Endocrinol (Paris) 2017 Dec;78(6):490-494. Epub 2017 Nov 27 doi: 10.1016/j.ando.2017.10.010. PMID: 29174931

Clinical prediction guides

The impact of transsphenoidal surgery on pituitary function in patients with non-functioning macroadenomas.
Mavromati M, Mavrakanas T, Jornayvaz FR, Schaller K, Fitsiori A, Vargas MI, Lobrinus JA, Merkler D, Egervari K, Philippe J, Leboulleux S, Momjian S
Endocrine 2023 Aug;81(2):340-348. Epub 2023 May 24 doi: 10.1007/s12020-023-03400-z. PMID: 37222882Free PMC Article
Pitfalls in the interpretation of the cosyntropin stimulation test for the diagnosis of adrenal insufficiency.
Burgos N, Ghayee HK, Singh-Ospina N
Curr Opin Endocrinol Diabetes Obes 2019 Jun;26(3):139-145. doi: 10.1097/MED.0000000000000473. PMID: 30855285
SFE/SFEDP adrenal insufficiency French consensus: Introduction and handbook.
Reznik Y, Barat P, Bertherat J, Bouvattier C, Castinetti F, Chabre O, Chanson P, Cortet C, Delemer B, Goichot B, Gruson D, Guignat L, Proust-Lemoine E, Sanson MR, Reynaud R, Boustani DS, Simon D, Tabarin A, Zenaty D
Ann Endocrinol (Paris) 2018 Feb;79(1):1-22. Epub 2018 Jan 12 doi: 10.1016/j.ando.2017.12.001. PMID: 29338844
Group 2: Adrenal insufficiency: screening methods and confirmation of diagnosis.
Chanson P, Guignat L, Goichot B, Chabre O, Boustani DS, Reynaud R, Simon D, Tabarin A, Gruson D, Reznik Y, Raffin Sanson ML
Ann Endocrinol (Paris) 2017 Dec;78(6):495-511. Epub 2017 Nov 23 doi: 10.1016/j.ando.2017.10.005. PMID: 29174200
Measuring cortisol in serum, urine and saliva - are our assays good enough?
El-Farhan N, Rees DA, Evans C
Ann Clin Biochem 2017 May;54(3):308-322. Epub 2017 Mar 16 doi: 10.1177/0004563216687335. PMID: 28068807

Recent systematic reviews

Immune checkpoint inhibitor-induced isolated adrenocorticotropic hormone deficiency: a systematic review.
Wang F, Shi X, Yu X, Yang Y
Front Endocrinol (Lausanne) 2024;15:1326684. Epub 2024 Jan 22 doi: 10.3389/fendo.2024.1326684. PMID: 38318292Free PMC Article
Idiopathic isolated adrenocorticotropic hormone deficiency: a systematic review of a heterogeneous and underreported disease.
Van Mieghem E, De Block C, De Herdt C
Pituitary 2024 Feb;27(1):23-32. Epub 2023 Dec 27 doi: 10.1007/s11102-023-01366-9. PMID: 38151529
Isolated ACTH deficiency induced by cancer immunotherapy: a systematic review.
Iglesias P, Sánchez JC, Díez JJ
Pituitary 2021 Aug;24(4):630-643. Epub 2021 Mar 24 doi: 10.1007/s11102-021-01141-8. PMID: 33761049
Guidelines for the diagnosis and management of critical illness-related corticosteroid insufficiency (CIRCI) in critically ill patients (Part I): Society of Critical Care Medicine (SCCM) and European Society of Intensive Care Medicine (ESICM) 2017.
Annane D, Pastores SM, Rochwerg B, Arlt W, Balk RA, Beishuizen A, Briegel J, Carcillo J, Christ-Crain M, Cooper MS, Marik PE, Umberto Meduri G, Olsen KM, Rodgers S, Russell JA, Van den Berghe G
Intensive Care Med 2017 Dec;43(12):1751-1763. Epub 2017 Sep 21 doi: 10.1007/s00134-017-4919-5. PMID: 28940011
Pituitary Dysfunction After Aneurysmal Subarachnoid Hemorrhage: A Systematic Review and Meta-analysis.
Can A, Gross BA, Smith TR, Dammers R, Dirven CM, Woodmansee WW, Laws ER, Du R
Neurosurgery 2016 Aug;79(2):253-64. doi: 10.1227/NEU.0000000000001157. PMID: 26645970

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...