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Acute adrenal insufficiency

MedGen UID:
56227
Concept ID:
C0151467
Disease or Syndrome
Synonyms: Addisonian crisis; Adrenal crisis
SNOMED CT: Acute adrenal failure (766986002); Acute adrenal insufficiency (766986002); Adrenocortical crisis (766986002); Adrenal crisis (766986002); Addisonian crisis (766986002); Acute adrenocortical insufficiency (766986002); Severe adrenal insufficiency (24867002)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
X-linked dominant inheritance
MedGen UID:
376232
Concept ID:
C1847879
Finding
Source: Orphanet
A mode of inheritance that is observed for dominant traits related to a gene encoded on the X chromosome. In the context of medical genetics, X-linked dominant disorders tend to manifest very severely in affected males. The severity of manifestation in females may depend on the degree of skewed X inactivation.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019801
Orphanet: ORPHA95409

Definition

A rare but severe condition caused by a sudden defective production of adrenal steroids (cortisol and aldosterone). It represents an emergency and may occur at any age. Steroid withdrawal is the most common cause in patients with chronic adrenal insufficiency. A precipitating illness, surgery without adrenal support, pregnancy, any acute or chronic disease, or acute trauma are other potential causes of an acute adrenal crisis. The disorder may result from an acute exacerbation of chronic primary adrenal insufficiency. Laboratory exams show signs of adrenal insufficiency (hypoglycemia, hyponatremia and elevated natriuresis, hyperkaliemia, hemoconcentration, hypochloremic metabolic acidosis and functional renal failure) confirmed by hypocortisolemia, increased adrenocorticotropic hormone (ACTH) and an insufficient response to rapid ACTH stimulation testing. [from SNOMEDCT_US]

Term Hierarchy

Professional guidelines

PubMed

Martel-Duguech L, Poirier J, Bourdeau I, Lacroix A
Rev Endocr Metab Disord 2024 Jun;25(3):619-637. Epub 2024 Feb 27 doi: 10.1007/s11154-024-09877-x. PMID: 38411891
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
Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, Falorni A, Gan EH, Hulting AL, Kasperlik-Zaluska A, Kämpe O, Løvås K, Meyer G, Pearce SH
J Intern Med 2014 Feb;275(2):104-15. Epub 2013 Dec 16 doi: 10.1111/joim.12162. PMID: 24330030

Recent clinical studies

Etiology

Ali SR, Bryce J, Krone NP, Claahsen-van der Grinten HL, Ahmed SF
Horm Res Paediatr 2022;95(4):363-373. Epub 2022 May 13 doi: 10.1159/000525075. PMID: 35569445
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
Guignat L
Ann Endocrinol (Paris) 2018 Jun;79(3):167-173. Epub 2018 Mar 29 doi: 10.1016/j.ando.2018.03.002. PMID: 29606279
Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, Falorni A, Gan EH, Hulting AL, Kasperlik-Zaluska A, Kämpe O, Løvås K, Meyer G, Pearce SH
J Intern Med 2014 Feb;275(2):104-15. Epub 2013 Dec 16 doi: 10.1111/joim.12162. PMID: 24330030
Ascoli P, Cavagnini F
Pituitary 2006;9(4):335-42. doi: 10.1007/s11102-006-0416-5. PMID: 17077946

Diagnosis

Martel-Duguech L, Poirier J, Bourdeau I, Lacroix A
Rev Endocr Metab Disord 2024 Jun;25(3):619-637. Epub 2024 Feb 27 doi: 10.1007/s11154-024-09877-x. PMID: 38411891
Kunhivalappil FT, Hefny AF, Abu-Zidan FM
Injury 2019 May;50(5):1049-1052. Epub 2018 Dec 28 doi: 10.1016/j.injury.2018.12.033. PMID: 30606456
Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, Falorni A, Gan EH, Hulting AL, Kasperlik-Zaluska A, Kämpe O, Løvås K, Meyer G, Pearce SH
J Intern Med 2014 Feb;275(2):104-15. Epub 2013 Dec 16 doi: 10.1111/joim.12162. PMID: 24330030
Ascoli P, Cavagnini F
Pituitary 2006;9(4):335-42. doi: 10.1007/s11102-006-0416-5. PMID: 17077946
Werbel SS, Ober KP
Endocrinol Metab Clin North Am 1993 Jun;22(2):303-28. PMID: 8325289

Therapy

Ali SR, Bryce J, Krone NP, Claahsen-van der Grinten HL, Ahmed SF
Horm Res Paediatr 2022;95(4):363-373. Epub 2022 May 13 doi: 10.1159/000525075. PMID: 35569445
Caron P, Grunenwald S, Persani L, Borson-Chazot F, Leroy R, Duntas L
Rev Endocr Metab Disord 2022 Jun;23(3):463-483. Epub 2021 Oct 20 doi: 10.1007/s11154-021-09691-9. PMID: 34671932Free PMC Article
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
Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, Falorni A, Gan EH, Hulting AL, Kasperlik-Zaluska A, Kämpe O, Løvås K, Meyer G, Pearce SH
J Intern Med 2014 Feb;275(2):104-15. Epub 2013 Dec 16 doi: 10.1111/joim.12162. PMID: 24330030
Ascoli P, Cavagnini F
Pituitary 2006;9(4):335-42. doi: 10.1007/s11102-006-0416-5. PMID: 17077946

Prognosis

Gao L, Zhang L, Luo J, Zhang G, Shen Y, Wang Y, Li M
Clin Rheumatol 2024 Jul;43(7):2223-2227. Epub 2024 May 9 doi: 10.1007/s10067-024-06938-z. PMID: 38724818
Karwacka IM, Obołończyk Ł, Sworczak K
Adv Clin Exp Med 2018 May;27(5):681-687. doi: 10.17219/acem/68897. PMID: 29616752
Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, Falorni A, Gan EH, Hulting AL, Kasperlik-Zaluska A, Kämpe O, Løvås K, Meyer G, Pearce SH
J Intern Med 2014 Feb;275(2):104-15. Epub 2013 Dec 16 doi: 10.1111/joim.12162. PMID: 24330030
Werbel SS, Ober KP
Endocrinol Metab Clin North Am 1993 Jun;22(2):303-28. PMID: 8325289
Lairmore TC, Ball DW, Baylin SB, Wells SA Jr
Ann Surg 1993 Jun;217(6):595-601; discussion 601-3. doi: 10.1097/00000658-199306000-00001. PMID: 8099474Free PMC Article

Clinical prediction guides

Gao L, Zhang L, Luo J, Zhang G, Shen Y, Wang Y, Li M
Clin Rheumatol 2024 Jul;43(7):2223-2227. Epub 2024 May 9 doi: 10.1007/s10067-024-06938-z. PMID: 38724818
Karwacka IM, Obołończyk Ł, Sworczak K
Adv Clin Exp Med 2018 May;27(5):681-687. doi: 10.17219/acem/68897. PMID: 29616752
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
Guignat L, Proust-Lemoine E, Reznik Y, Zenaty D
Ann Endocrinol (Paris) 2017 Dec;78(6):544-558. Epub 2017 Dec 1 doi: 10.1016/j.ando.2017.10.009. PMID: 29183634
Kim KT, Suk KS, Kim JM, Lee SH
Int Orthop 2003;27(2):65-9. Epub 2003 Mar 26 doi: 10.1007/s00264-002-0418-5. PMID: 12700926Free PMC Article

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