MCID: ACT150
MIFTS: 47

Acute Adrenal Insufficiency

Categories: Endocrine diseases, Rare diseases
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Aliases & Classifications for Acute Adrenal Insufficiency

MalaCards integrated aliases for Acute Adrenal Insufficiency:

Name: Acute Adrenal Insufficiency 58 53 33
Addisonian Crisis 58 71 31 33
Adrenal Crisis 58 75 33
Acute Adrenocortical Insufficiency 58 33
Adrenocortical Crisis 58 33
Severe Adrenal Insufficiency 33
Acute Adrenal Failure 58
Adrenal Collapse 33
Addison Crisis 33
Adrenal Shock 33

Characteristics:


Inheritance:

Autosomal recessive,X-linked dominant 58

Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare endocrine diseases


Summaries for Acute Adrenal Insufficiency

Orphanet: 58 A primary adrenal insufficiency caused by a sudden defective production of adrenal steroids (cortisol and aldosterone). It represents an emergency, thus the rapid recognition and prompt therapy are critical for survival even before the diagnosis is made.

MalaCards based summary: Acute Adrenal Insufficiency, also known as addisonian crisis, is related to adrenal insufficiency, congenital, with 46,xy sex reversal, partial or complete and hypoadrenocorticism, familial. An important gene associated with Acute Adrenal Insufficiency is CYP21A2 (Cytochrome P450 Family 21 Subfamily A Member 2), and among its related pathways/superpathways are Metapathway biotransformation Phase I and II and Diseases of glycosylation. The drugs Racepinephrine and Prednisolone phosphate have been mentioned in the context of this disorder. Affiliated tissues include adrenal gland, skin and pituitary, and related phenotypes are decreased circulating cortisol level and failure to thrive

Wikipedia: 75 Adrenal crisis is a potentially life-threatening medical condition requiring immediate emergency... more...

Related Diseases for Acute Adrenal Insufficiency

Diseases in the Rare Adrenal Disease family:

Secondary Adrenal Insufficiency Acute Adrenal Insufficiency
Acquired Chronic Primary Adrenal Insufficiency Chronic Primary Adrenal Insufficiency
Primary Adrenal Insufficiency

Diseases related to Acute Adrenal Insufficiency via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 313)
# Related Disease Score Top Affiliating Genes
1 adrenal insufficiency, congenital, with 46,xy sex reversal, partial or complete 31.3 POMC CYP21A2 CYP11A1
2 hypoadrenocorticism, familial 29.9 POMC CYP21A2 CYP11A1
3 autoimmune polyendocrine syndrome, type i, with or without reversible metaphyseal dysplasia 29.8 CYP21A2 CYP11A1
4 achalasia-addisonianism-alacrima syndrome 29.5 POMC CYP11A1
5 lipoid congenital adrenal hyperplasia 29.5 POMC CYP21A2 CYP11A1
6 conn's syndrome 29.3 POMC CYP21A2 CYP11A1
7 familial glucocorticoid deficiency 29.2 POMC CYP21A2 CYP11A1
8 adrenal hypoplasia, congenital 29.1 POMC CYP21A2 CYP11A1
9 adrenal cortical adenoma 29.1 POMC CYP21A2 CYP11A1
10 adrenal adenoma 29.1 POMC CYP21A2 CYP11A1
11 adrenal cortical carcinoma 29.1 POMC CYP21A2 CYP11A1
12 pseudohermaphroditism 29.0 POMC CYP21A2 CYP11A1
13 premature menopause 28.9 POMC CYP21A2 CYP11A1
14 intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies 11.4
15 waterhouse-friderichsen syndrome 11.3
16 sheehan syndrome 11.2
17 inherited isolated adrenal insufficiency due to partial cyp11a1 deficiency 11.1
18 premature ovarian failure 7 11.1
19 toxic shock syndrome 10.4
20 bilateral massive adrenal hemorrhage 10.4
21 antiphospholipid syndrome 10.4
22 primary adrenal insufficiency 10.3
23 histoplasmosis 10.3
24 autoimmune polyendocrine syndrome, type ii 10.3
25 thrombocytopenia 10.2
26 hypoglycemia 10.2
27 heparin-induced thrombocytopenia 10.2
28 thrombophilia due to thrombin defect 10.1
29 hypothyroidism 10.1
30 hypopituitarism 10.1
31 secondary adrenal insufficiency 10.1
32 lymphoma, hodgkin, classic 10.1
33 cardiac tamponade 10.1
34 autoimmune polyendocrine syndrome 10.1
35 pericarditis 10.1
36 central diabetes insipidus 10.1
37 graves' disease 10.1
38 hypogonadism 10.1
39 diabetes insipidus 10.1
40 broken heart syndrome 10.1
41 autoimmune disease 10.1
42 pituitary hormone deficiency, combined, 2 10.1
43 graves disease 1 10.1
44 cardiac arrest 10.1
45 diarrhea 10.1
46 hyperparathyroidism 10.1
47 adenocarcinoma 10.1
48 hypokalemia 10.1
49 thyroiditis 10.1
50 meningococcemia 10.1

Graphical network of the top 20 diseases related to Acute Adrenal Insufficiency:



Diseases related to Acute Adrenal Insufficiency

Symptoms & Phenotypes for Acute Adrenal Insufficiency

Human phenotypes related to Acute Adrenal Insufficiency:

30 58 (show all 46)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 decreased circulating cortisol level 30 Obligate (100%) HP:0008163
2 failure to thrive 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001508
3 nausea and vomiting 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002017
4 constipation 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002019
5 muscle weakness 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001324
6 fatigue 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0012378
7 autoimmunity 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002960
8 abdominal pain 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002027
9 anorexia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002039
10 weight loss 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001824
11 diarrhea 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002014
12 hyperpigmentation of the skin 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000953
13 increased circulating acth level 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0003154
14 hypovolemia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0011106
15 seizure 58 30 Frequent (33%) Frequent (79-30%)
HP:0001250
16 hypotonia 58 30 Frequent (33%) Frequent (79-30%)
HP:0001252
17 renal insufficiency 58 30 Frequent (33%) Frequent (79-30%)
HP:0000083
18 hypoglycemia 58 30 Frequent (33%) Frequent (79-30%)
HP:0001943
19 hyperuricemia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002149
20 hyponatremia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002902
21 hyperkalemia 58 30 Frequent (33%) Frequent (79-30%)
HP:0002153
22 orthostatic hypotension 58 30 Frequent (33%) Frequent (79-30%)
HP:0001278
23 decreased circulating aldosterone level 58 30 Frequent (33%) Frequent (79-30%)
HP:0004319
24 normocytic anemia 58 30 Frequent (33%) Frequent (79-30%)
HP:0001897
25 renal salt wasting 58 30 Frequent (33%) Frequent (79-30%)
HP:0000127
26 increased circulating renin level 58 30 Frequent (33%) Frequent (79-30%)
HP:0000848
27 decreased urinary potassium 58 30 Frequent (33%) Frequent (79-30%)
HP:0012364
28 hyperkalemic metabolic acidosis 58 30 Frequent (33%) Frequent (79-30%)
HP:0005976
29 androgen insufficiency 58 30 Frequent (33%) Frequent (79-30%)
HP:0008226
30 recurrent acute respiratory tract infection 30 Frequent (33%) HP:0011948
31 delayed puberty 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000823
32 dry skin 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000958
33 vitiligo 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001045
34 myocardial infarction 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001658
35 vertigo 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002321
36 arthralgia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002829
37 stroke 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001297
38 hypercalcemia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003072
39 sparse axillary hair 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002215
40 adrenal hypoplasia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000835
41 decreased female libido 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0030018
42 salt craving 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0030083
43 hypotension 58 Very frequent (99-80%)
44 primary adrenal insufficiency 58 Obligate (100%)
45 hypocortisolemia 58 Obligate (100%)
46 acute respiratory tract infection 58 Frequent (79-30%)

GenomeRNAi Phenotypes related to Acute Adrenal Insufficiency according to GeneCards Suite gene sharing:

25 (show all 17)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.9 CYP11A1 CYP21A2
2 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.9 CYP21A2
3 Increased shRNA abundance (Z-score > 2) GR00366-A-11 9.9 CYP11A1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.9 CYP11A1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-139 9.9 CYP21A2
6 Increased shRNA abundance (Z-score > 2) GR00366-A-145 9.9 CYP21A2
7 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.9 CYP21A2
8 Increased shRNA abundance (Z-score > 2) GR00366-A-166 9.9 CYP11A1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-184 9.9 CYP11A1 CYP21A2
10 Increased shRNA abundance (Z-score > 2) GR00366-A-185 9.9 CYP21A2
11 Increased shRNA abundance (Z-score > 2) GR00366-A-203 9.9 CYP21A2
12 Increased shRNA abundance (Z-score > 2) GR00366-A-206 9.9 CYP21A2
13 Increased shRNA abundance (Z-score > 2) GR00366-A-208 9.9 CYP21A2
14 Increased shRNA abundance (Z-score > 2) GR00366-A-213 9.9 CYP21A2
15 Increased shRNA abundance (Z-score > 2) GR00366-A-42 9.9 CYP11A1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.9 CYP21A2
17 Increased shRNA abundance (Z-score > 2) GR00366-A-8 9.9 CYP21A2

Drugs & Therapeutics for Acute Adrenal Insufficiency

Drugs for Acute Adrenal Insufficiency (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 25)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Racepinephrine Approved, Vet_approved Phase 4 51-43-4, 329-65-7 838 5816
2
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
3
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
4
Prednisolone Approved, Vet_approved Phase 4 50-24-8 4894 5755
5
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
6
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5 1875
7
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 4159 6741
8
Hydrocortisone succinate Approved Phase 4 2203-97-6 3643
9
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
10
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 3640 5754
11
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7 4897
12 Epinephryl borate Phase 4
13 Antineoplastic Agents, Hormonal Phase 4
14 Neuroprotective Agents Phase 4
15 Hormones Phase 4
16 Hormone Antagonists Phase 4
17 Vasoconstrictor Agents Phase 4
18 Antiemetics Phase 4
19 glucocorticoids Phase 4
20 Anti-Inflammatory Agents Phase 4
21
Methylprednisolone Acetate Phase 4 584547
22 Gastrointestinal Agents Phase 4
23 Hydrocortisone-17-butyrate Phase 4
24 Hydrocortisone 17-butyrate 21-propionate Phase 4
25 Protective Agents Phase 4

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 An Open-label, Prospective, Randomized, Controlled Clinical Trial of the Use of Reduced Duration Versus Standard Duration Steroid Replacement Therapy for Acute Adrenal Insufficiency in Patients With Septic Shock Terminated NCT00842933 Phase 4 Corticosteroid
2 Vaginal Prednisone Administration for Prevention of Adrenal Crisis - a Bioequivalence Study Completed NCT02689960 Phase 2 100mg prednisone suppository
3 Predisposing Factors for the Incidence of Adrenal Crisis in Chronic Adrenal Insufficiency Unknown status NCT03410160
4 Prospective Study to Investigate Adrenal Crisis in Patients With Chronic Adrenal Insufficiency (Six-year Follow-up) Completed NCT03499990
5 Prospective Cohort-study on the Incidence of Adrenal Crisis in Patients With Chronic Adrenal Insufficiency Completed NCT01184209
6 Novel Treatment of Adrenal Crisis: an Early Clinical Trial With Nebulized Prednisolone Recruiting NCT05639127 Early Phase 1 Prednisolone

Search NIH Clinical Center for Acute Adrenal Insufficiency

Genetic Tests for Acute Adrenal Insufficiency

Anatomical Context for Acute Adrenal Insufficiency

Organs/tissues related to Acute Adrenal Insufficiency:

MalaCards : Adrenal Gland, Skin, Pituitary, Adrenal Cortex, Spinal Cord, Cortex, Lung

Publications for Acute Adrenal Insufficiency

Articles related to Acute Adrenal Insufficiency:

(show top 50) (show all 1530)
# Title Authors PMID Year
1
Hypopituitarism. 53 62
17077946 2006
2
Congenital adrenal hyperplasia. 53 62
15568308 2004
3
The Assessment of the Hypothalamic-Pituitary-Adrenal Axis After Oncological Treatment in Pediatric Patients with Acute Lymphoblastic Leukemia 62
35633644 2022
4
Approach to the Patient with Adrenal Hemorrhage. 62
36404284 2022
5
Intracardiac thrombosis following intravenous zoledronate treatment in a child with steroid-induced osteoporosis. 62
36427218 2022
6
End-of-life impact of concurrent diabetes mellitus and adrenal insufficiency as immune-related adverse events in an advanced non-small cell lung cancer patient. 62
36195556 2022
7
The management of congenital adrenal hyperplasia during preconception, pregnancy, and postpartum. 62
36399318 2022
8
Acute adrenal insufficiency in a patient with panhypopituitarism after vaccination against COVID-19 (BNT162b2 Pfizer-BioNTech). 62
36428206 2022
9
[Acute adrenal insufficiency in a patient with panhypopituitarism after vaccination against COVID-19 (BNT162b2 Pfizer-Biontech)]. 62
34957378 2022
10
Effect of long-term management of hypoadrenocorticism on the quality of life of affected dogs and their owners. 62
35941756 2022
11
Doctors, teach your adrenal insufficiency patients well: provide them with a European Emergency Card! 62
36327148 2022
12
Congenital adrenal hyperplasia in patients with adrenal tumors: a population-based case-control study. 62
36269558 2022
13
Glucocorticoid receptor blockers. 62
35507245 2022
14
Prevalence and risk factors for hypothalamus-pituitary-adrenal axis suppression in infants receiving glucocorticoid eye drops after ocular surgery. 62
36165330 2022
15
Emphasizing Pulmonary Function With Respiratory Therapy in an Infant With Congenital Adrenal Hyperplasia, Bronchopneumonia and Leptomeningitis. 62
36199651 2022
16
Congenital lipoid adrenal hyperplasia with Graves' disease: A case report. 62
36159444 2022
17
Congenital lipoid adrenal hyperplasia in a Saudi infant. 62
36053179 2022
18
Central hypothyroidism with myxoedema: a less known but clinically challenging presentation. 62
36100285 2022
19
Delayed diagnosis of complex glycerol kinase deficiency in a Chinese male infant: a case report. 62
36050749 2022
20
Chinese primary adrenal lymphoma complicated with adrenal crisis: A case report and literature review. 62
36204896 2022
21
Adult height of children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. 62
36155706 2022
22
Survey of glucocorticoid dose escalation in patients with adrenal insufficiency during the peri-COVID-19 vaccination period. 62
36104247 2022
23
[Acute adrenal insufficiency: an entity that should not be missed]. 62
36082384 2022
24
Adrenal insufficiency among children treated with hormonal therapy for infantile spasms. 62
35759339 2022
25
Diagnosis and Management of Adrenal Insufficiency and Adrenal Crisis in the Emergency Department. 62
36038436 2022
26
Serological Evidence and Self-reported Outcomes in Patients with Adrenal Insufficiency during the first waves of COVID-19 in the North-East Italy. 62
35959624 2022
27
Case of Junctional Rhythm in the Setting of Acute Adrenal Insufficiency. 62
36059370 2022
28
Adrenal Gland Function and Dysfunction During COVID-19. 62
35944524 2022
29
Hypothalamic-pituitary-adrenal axis recovery after treatment of Cushing's syndrome. 62
35726348 2022
30
Adrenal Crisis Mimicking COVID-19 Encephalopathy in a Teenager with Craniopharyngioma. 62
36010128 2022
31
Pallister-Hall syndrome diagnosed in a young man after an acute adrenal crisis. 62
36017114 2022
32
Management of patients at risk of adrenal crisis in the dental setting: a review of current practice in UK dental teaching hospitals. 62
35931749 2022
33
The ISAQ Score Does Not Predict Adrenal Crisis in Patients with Primary Adrenal Insufficiency. 62
35240692 2022
34
Devastating salt-wasting crisis in a four-month-old male child with congenital adrenal hyperplasia, highlighting the essence of neonatal screening. 62
35846927 2022
35
Isolated ACTH deficiency following immunization with the BNT162b2 SARS-CoV-2 vaccine: a case report. 62
35854260 2022
36
Adrenal insufficiency. 62
35534201 2022
37
Adrenal Crisis Presenting as Recurrent Encephalopathy Mimicking Autoimmune, Infectious Encephalitis, and Common Variable Immune Deficiency: A Case Report. 62
34855666 2022
38
Adrenal Crisis Secondary to COVID-19 Vaccination in a Patient With Hypopituitarism. 62
35496468 2022
39
Adrenal insufficiency in pregnancy: Physiology, diagnosis, management and areas for future research. 62
35816262 2022
40
Interventions for the prevention of adrenal crisis in adults with primary adrenal insufficiency: a systematic review. 62
35536876 2022
41
Critical Illness-induced Corticosteroid Insufficiency: What It Is Not and What It Could Be. 62
35358303 2022
42
Postoperative adrenal insufficiency in Conn's syndrome-does it occur frequently? 62
34615973 2022
43
Approach to the Patient With Glucocorticoid-induced Adrenal Insufficiency. 62
35302603 2022
44
Antibody and T cell responses to COVID-19 vaccination in patients receiving anticancer therapies. 62
35732350 2022
45
Adrenal crises in adolescents and young adults. 62
35583847 2022
46
A Novel SCNN1A Variation in a Patient with Autosomal-recessive Pseudohypoaldosteronism Type 1 62
33829730 2022
47
Factors influencing the levothyroxine dose in the hormone replacement therapy of primary hypothyroidism in adults. 62
34671932 2022
48
Atypical Case of Schmidt's Syndrome in a Young Male. 62
35911265 2022
49
Regarding "Fatal adrenal crisis due to Addison's disease arising in the context of autoimmune polyglandular syndrome type 1". 62
34851495 2022
50
Author Correction: Regarding "Fatal adrenal crisis due to Addison's disease arising in the context of autoimmune polyglandular syndrome type 1". 62
35275325 2022

Variations for Acute Adrenal Insufficiency

Expression for Acute Adrenal Insufficiency

Search GEO for disease gene expression data for Acute Adrenal Insufficiency.

Pathways for Acute Adrenal Insufficiency

Pathways related to Acute Adrenal Insufficiency according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.42 POMC CYP21A2 CYP11A1
2
Show member pathways
12.15 POMC CYP21A2 CYP11A1
3
Show member pathways
12.07 POMC CYP21A2 CYP11A1
4
Show member pathways
11.89 POMC CYP21A2 CYP11A1
5
Show member pathways
11.12 POMC CYP21A2 CYP11A1
6 10.9 CYP21A2 CYP11A1
7 10.76 POMC CYP21A2 CYP11A1

GO Terms for Acute Adrenal Insufficiency

Biological processes related to Acute Adrenal Insufficiency according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 steroid metabolic process GO:0008202 9.43 CYP21A2 CYP11A1
2 steroid biosynthetic process GO:0006694 9.33 CYP21A2 CYP11A1
3 sterol metabolic process GO:0016125 9.26 CYP21A2 CYP11A1
4 glucocorticoid biosynthetic process GO:0006704 8.92 CYP21A2 CYP11A1

Molecular functions related to Acute Adrenal Insufficiency according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 heme binding GO:0020037 9.56 CYP21A2 CYP11A1
2 iron ion binding GO:0005506 9.46 CYP21A2 CYP11A1
3 monooxygenase activity GO:0004497 8.96 CYP21A2 CYP11A1
4 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen GO:0016705 8.62 CYP21A2 CYP11A1

Sources for Acute Adrenal Insufficiency

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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