MCID: ACT150
MIFTS: 46

Acute Adrenal Insufficiency

Categories: Endocrine diseases, Rare diseases

Aliases & Classifications for Acute Adrenal Insufficiency

MalaCards integrated aliases for Acute Adrenal Insufficiency:

Name: Acute Adrenal Insufficiency 56 52
Addisonian Crisis 56 69
Acute Adrenocortical Insufficiency 56
Acute Adrenal Failure 56
Adrenocortical Crisis 56
Adrenal Crisis 56

Characteristics:

Orphanet epidemiological data:

56
acute adrenal insufficiency
Inheritance: Autosomal recessive,Not applicable,X-linked dominant; Age of onset: All ages; Age of death: any age;

Classifications:

Orphanet: 56  
Rare endocrine diseases


Summaries for Acute Adrenal Insufficiency

MalaCards based summary : Acute Adrenal Insufficiency, also known as addisonian crisis, is related to adrenal insufficiency, congenital, with 46xy sex reversal, partial or complete and sheehan syndrome, and has symptoms including hyperpigmentation of the skin, muscle weakness and failure to thrive. An important gene associated with Acute Adrenal Insufficiency is CYP11A1 (Cytochrome P450 Family 11 Subfamily A Member 1), and among its related pathways/superpathways are Interleukin-4 and 13 signaling and Diseases of metabolism. The drugs Epinephrine and Hydrocortisone have been mentioned in the context of this disorder. Affiliated tissues include skin, pituitary and lung.

Related Diseases for Acute Adrenal Insufficiency

Diseases in the Acute Adrenal Insufficiency family:

Secondary Adrenal Insufficiency

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

(show top 50) (show all 113)
id Related Disease Score Top Affiliating Genes
1 adrenal insufficiency, congenital, with 46xy sex reversal, partial or complete 11.1
2 sheehan syndrome 10.9
3 pfeiffer syndrome 10.3 CYP11A1 POMC
4 congenital nonspherocytic hemolytic anemia 10.2 IL6 POMC
5 centrotemporal epilepsy 10.2 CYP11A1 POMC
6 abnormal retinal correspondence 10.2 IL6 POMC
7 chondroma 10.2 IL6 POMC
8 hypodontia of incisors and premolars 10.2 POMC REN
9 carotid artery thrombosis 10.1 POMC REN
10 cholangiolocellular carcinoma 10.1 POMC REN
11 hypoaldosteronism, congenital, due to cmo ii deficiency 10.1 POMC REN
12 familial hypopituitarism 10.1 POMC REN
13 trabecular follicular adenocarcinoma 10.1 POMC REN
14 uterine corpus adenocarcinofibroma 10.1 POMC REN
15 bronchiectasis 10.1 POMC REN
16 spondylolysis 10.1 IL6 POMC
17 north american indian childhood cirrhosis 10.1 POMC REN
18 cardiovascular organ benign neoplasm 10.1 POMC REN
19 astrakhan spotted fever 10.1 POMC REN
20 urinary bladder posterior wall cancer 10.1 IL6 REN
21 vestibular gland benign neoplasm 10.0 POMC REN
22 adams-oliver syndrome 5 10.0 POMC REN
23 pediatric angiosarcoma 10.0 POMC REN
24 renal clear cell carcinoma 10.0 POMC REN
25 paranasal sinus sarcoma 10.0 IL6 REN
26 liver sarcoma 10.0 CYP11A1 CYP21A2
27 benign struma ovarii 10.0 IL6 REN
28 vallecula cancer 10.0 IL6 POMC
29 cleft lip and palate malrotation cardiopathy 10.0 CYP21A2 POMC
30 kunjin encephalitis 10.0 CYP11A1 CYP21A2
31 male reproductive organ benign neoplasm 10.0 POMC REN
32 dfna2 nonsyndromic hearing loss 9.9 CYP21A2 POMC
33 hypertensive encephalopathy 9.9 POMC REN
34 sphingolipidosis 9.9 CYP21A2 POMC
35 inherited metabolic disorder 9.9 IL6 POMC
36 diabetic encephalopathy 9.9 IL6 REN
37 benign peritoneal mesothelioma 9.9 CYP21A2 POMC
38 acute myocarditis 9.9 CYP21A2 POMC
39 periampullary adenoma 9.9 IL6 REN
40 basal cell carcinoma 7 9.9 CYP11A1 CYP21A2
41 antiphospholipid syndrome 9.9
42 pneumonia 9.9
43 adrenal cortex disease 9.9 CYP21A2 POMC
44 branchiootorenal/branchiootic syndrome 9.9 CYP21A2 REN
45 addison's disease 9.8
46 thyroiditis 9.8
47 partial motor epilepsy 9.8 IL6 POMC
48 pituitary apoplexy 9.8
49 cardiac tamponade 9.8
50 pericarditis 9.8

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:

56 32 (show all 46)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperpigmentation of the skin 56 32 hallmark (90%) Very frequent (99-80%) HP:0000953
2 muscle weakness 56 32 hallmark (90%) Very frequent (99-80%) HP:0001324
3 failure to thrive 56 32 hallmark (90%) Very frequent (99-80%) HP:0001508
4 weight loss 56 32 hallmark (90%) Very frequent (99-80%) HP:0001824
5 diarrhea 56 32 hallmark (90%) Very frequent (99-80%) HP:0002014
6 nausea and vomiting 56 32 hallmark (90%) Very frequent (99-80%) HP:0002017
7 constipation 56 32 hallmark (90%) Very frequent (99-80%) HP:0002019
8 abdominal pain 56 32 hallmark (90%) Very frequent (99-80%) HP:0002027
9 anorexia 56 32 hallmark (90%) Very frequent (99-80%) HP:0002039
10 autoimmunity 56 32 hallmark (90%) Very frequent (99-80%) HP:0002960
11 increased circulating acth level 56 32 hallmark (90%) Very frequent (99-80%) HP:0003154
12 hypovolemia 56 32 hallmark (90%) Very frequent (99-80%) HP:0011106
13 fatigue 56 32 hallmark (90%) Very frequent (99-80%) HP:0012378
14 renal insufficiency 56 32 frequent (33%) Frequent (79-30%) HP:0000083
15 renal salt wasting 56 32 frequent (33%) Frequent (79-30%) HP:0000127
16 increased circulating renin level 56 32 frequent (33%) Frequent (79-30%) HP:0000848
17 seizures 56 32 frequent (33%) Frequent (79-30%) HP:0001250
18 muscular hypotonia 56 32 frequent (33%) Frequent (79-30%) HP:0001252
19 orthostatic hypotension 56 32 frequent (33%) Frequent (79-30%) HP:0001278
20 normocytic anemia 56 32 frequent (33%) Frequent (79-30%) HP:0001897
21 hypoglycemia 56 32 frequent (33%) Frequent (79-30%) HP:0001943
22 hyperuricemia 56 32 frequent (33%) Frequent (79-30%) HP:0002149
23 hyperkalemia 56 32 frequent (33%) Frequent (79-30%) HP:0002153
24 hyponatremia 56 32 frequent (33%) Frequent (79-30%) HP:0002902
25 hyperkalemic metabolic acidosis 56 32 frequent (33%) Frequent (79-30%) HP:0005976
26 androgen insufficiency 56 32 frequent (33%) Frequent (79-30%) HP:0008226
27 acute respiratory tract infection 56 32 frequent (33%) Frequent (79-30%) HP:0011948
28 decreased urinary potassium 56 32 frequent (33%) Frequent (79-30%) HP:0012364
29 delayed puberty 56 32 occasional (7.5%) Occasional (29-5%) HP:0000823
30 adrenal hypoplasia 56 32 occasional (7.5%) Occasional (29-5%) HP:0000835
31 dry skin 56 32 occasional (7.5%) Occasional (29-5%) HP:0000958
32 vitiligo 56 32 occasional (7.5%) Occasional (29-5%) HP:0001045
33 stroke 56 32 occasional (7.5%) Occasional (29-5%) HP:0001297
34 myocardial infarction 56 32 occasional (7.5%) Occasional (29-5%) HP:0001658
35 sparse axillary hair 56 32 occasional (7.5%) Occasional (29-5%) HP:0002215
36 vertigo 56 32 occasional (7.5%) Occasional (29-5%) HP:0002321
37 arthralgia 56 32 occasional (7.5%) Occasional (29-5%) HP:0002829
38 hypercalcemia 56 32 occasional (7.5%) Occasional (29-5%) HP:0003072
39 decreased female libido 56 32 occasional (7.5%) Occasional (29-5%) HP:0030018
40 salt craving 56 32 occasional (7.5%) Occasional (29-5%) HP:0030083
41 primary adrenal insufficiency 56 Obligate (100%)
42 hypocortisolemia 56 Obligate (100%)
43 hypotension 56 Very frequent (99-80%)
44 hypoaldosteronism 56 Frequent (79-30%)
45 decreased circulating aldosterone level 32 frequent (33%) HP:0004319
46 decreased circulating cortisol level 32 obligate (100%) HP:0008163

Drugs & Therapeutics for Acute Adrenal Insufficiency

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

(show top 50) (show all 54)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Epinephrine Approved, Vet_approved Phase 4,Phase 2 51-43-4 5816
2
Hydrocortisone Approved, Vet_approved Phase 4,Phase 2 50-23-7 5754 657311
3 Racepinephrine Approved Phase 4,Phase 2
4
Prednisone Approved, Vet_approved Phase 4,Phase 2 53-03-2 5865
5
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
6
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
7
Butyric Acid Experimental Phase 4,Phase 2 107-92-6 264
8 glucocorticoids Phase 4,Phase 2
9 Respiratory System Agents Phase 4,Phase 2
10 Adrenergic Agents Phase 4,Phase 2
11 Adrenergic Agonists Phase 4,Phase 2
12 Adrenergic alpha-Agonists Phase 4,Phase 2
13 Adrenergic beta-Agonists Phase 4,Phase 2
14 Mydriatics Phase 4,Phase 2
15 Neurotransmitter Agents Phase 4,Phase 2
16 Cortisol succinate Phase 4,Phase 2
17 Hormone Antagonists Phase 4,Phase 2
18 Hormones Phase 4,Phase 2
19 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2
20 Vasoconstrictor Agents Phase 4,Phase 2
21 Anti-Asthmatic Agents Phase 4,Phase 2
22 Peripheral Nervous System Agents Phase 4,Phase 2
23 Hydrocortisone 17-butyrate 21-propionate Phase 4,Phase 2
24 Hydrocortisone acetate Phase 4,Phase 2
25 Hydrocortisone-17-butyrate Phase 4,Phase 2
26 Epinephryl borate Phase 4,Phase 2
27 Anti-Inflammatory Agents Phase 4,Phase 2
28 Autonomic Agents Phase 4,Phase 2
29 Bronchodilator Agents Phase 4,Phase 2
30 Antineoplastic Agents, Hormonal Phase 4,Phase 2
31 Prednisolone acetate Phase 4
32 Methylprednisolone acetate Phase 4
33 Methylprednisolone Hemisuccinate Phase 4
34 Prednisolone hemisuccinate Phase 4
35 Prednisolone phosphate Phase 4
36 Gastrointestinal Agents Phase 4
37 Neuroprotective Agents Phase 4
38 Antiemetics Phase 4
39 Protective Agents Phase 4
40 Immunoglobulins Phase 1
41 Antibodies Phase 1
42
Megestrol acetate Approved, Vet_approved 595-33-5 11683
43
Dehydroepiandrosterone Approved, Nutraceutical 53-43-0 9860744
44
Megestrol 3562-63-8 19090 3080587
45 insulin
46 beta-endorphin
47 Central Nervous System Stimulants
48 Adrenocorticotropic Hormone
49 Contraceptive Agents
50 Contraceptives, Oral

Interventional clinical trials:

(show all 18)

id Name Status NCT ID Phase Drugs
1 Effect of Treatment With Stress-Doses Glucocorticoid in Patients With Acute Respiratory Distress Syndrome (ARDS) Unknown status NCT00773058 Phase 4 hydrocortisone;placebo
2 Comparison of Two Strategies of Glucocorticoid Withdrawal in Rheumatoid Arthritis Patients Not yet recruiting NCT02997605 Phase 4 GlucoCorticoid
3 Adrenal Insufficiency in Septic Shock Terminated NCT00842933 Phase 4 Corticosteroid;Corticosteroid
4 Vaginal Prednisone Administration for Prevention of Adrenal Crisis Completed NCT02689960 Phase 2 100mg prednisone suppository
5 Phase II Pilot Study of Early Cortisol Replacement to Prevent Bronchopulmonary Dysplasia Completed NCT00004669 Phase 2 hydrocortisone
6 Pharmacokinetics of Hydrocortisone After Subcutaneous Administration in Chronic Adrenal Insufficiency Completed NCT01450930 Phase 2 Hydrocortisone intramuscular first;Hydrocortisone subcutaneously first
7 Safety and Immune Response of BMS-936559 in HIV-Infected People Taking Combination Antiretroviral Therapy Completed NCT02028403 Phase 1 BMS-936559;Placebo for BMS-936559
8 Adrenal Insufficiency in Critical Emergencies in Digestive Diseases Unknown status NCT00562445
9 Prospective Study on the Incidence of Adrenal Crisis in Patients With Chronic Adrenal Insufficiency Completed NCT01184209
10 Survey in Patients With Chronic Adrenal Insufficiency in Germany Completed NCT00444119
11 Adrenal Suppression and Adrenal Recovery Induced by Megestrol Acetate Completed NCT00575029 megestrol acetate
12 Metabolic Effects of Acute Cortisol Withdrawal in Adrenal Failure Completed NCT00442390 hydrocortisone substitution therapy versus withdrawal
13 National Education Programme for Patients With Chronic Adrenal Insufficiency Recruiting NCT02694926
14 Natriuresis as a Predictor of the Haemodynamic Response to Steroid Replacement Therapy in Patients in Septic Shock Recruiting NCT03258619
15 Inhalation/Nasal Corticosteroids and Prevalence of Hypothalamic-pituitary-adrenal (HPA) Axis Suppression in Human Immunodeficiency Virus (HIV)-Patients Recruiting NCT02501486
16 A European Post-Authorisation Observational Study (Registry) of Patients With Chronic Adrenal Insufficiency (AI) Recruiting NCT01661387
17 Impact of Pre-operative Steroids on Adrenal Insufficiency Recruiting NCT02956707
18 Rheumatoid Arthritis Adrenal Recovery Study Not yet recruiting NCT03294876

Search NIH Clinical Center for Acute Adrenal Insufficiency

Genetic Tests for Acute Adrenal Insufficiency

Anatomical Context for Acute Adrenal Insufficiency

MalaCards organs/tissues related to Acute Adrenal Insufficiency:

39
Skin, Pituitary, Lung, Spinal Cord, B Cells, Testes, Adrenal Gland

Publications for Acute Adrenal Insufficiency

Articles related to Acute Adrenal Insufficiency:

(show top 50) (show all 104)
id Title Authors Year
1
Acute Adrenal Insufficiency Associated with Rathke's Cleft Cyst. ( 26984082 )
2016
2
Acute adrenal insufficiency secondary to bilateral adrenal B-cell lymphoma: a case report and review of the literature. ( 27170834 )
2016
3
Conduct protocol in emergency: Acute adrenal insufficiency. ( 27992012 )
2016
4
An acute adrenal insufficiency revealing pituitary metastases of lung cancer in an elderly patient. ( 27200139 )
2016
5
Acute adrenal insufficiency: an aide-memoire of the critical importance of its recognition and prevention. ( 26968598 )
2016
6
SOCIETY FOR ENDOCRINOLOGY ENDOCRINE EMERGENCY GUIDANCE: Emergency management of acute adrenal insufficiency (adrenal crisis) in adult patients. ( 27935813 )
2016
7
Peri-portal lymphedema in association with an acute adrenal insufficiency: case report. ( 24661563 )
2014
8
Systemic lupus erythematosus presenting as acute adrenal insufficiency: a rare clinical presentation. ( 24669348 )
2014
9
Transient acute adrenal insufficiency associated with adenovirus serotype 40 infection. ( 24928932 )
2014
10
Acute adrenal insufficiency as a first presentation of myelodysplastic syndrome and sigmoid colon adenocarcinoma: a case report. ( 25988040 )
2014
11
Acute adrenal insufficiency due to primary antiphospholipid antibody syndrome. ( 24251172 )
2013
12
Rare presentation of shock and acute mesenteric ischaemia secondary to acute adrenal insufficiency in an 11-year-old male. ( 22970910 )
2013
13
Acute adrenal insufficiency following arthroplasty: a case report and review of the literature. ( 24028157 )
2013
14
Idiopathic bilateral adrenal haemorrhage related to acute adrenal insufficiency. ( 23729712 )
2013
15
Acute adrenal insufficiency. ( 22712152 )
2012
16
Acute adrenal insufficiency in a neonate with bilateral adrenal hemorrhage and combined prothrombotic risk factors. ( 22652827 )
2012
17
Acute adrenal insufficiency associated with tuberculous vertebral osteomyelitis and lymphadenopathy: case report. ( 22649455 )
2012
18
Acute adrenal insufficiency in cervical spinal cord injury. ( 22120347 )
2012
19
Community acquired bilateral upper lobe pneumonia with acute adrenal insufficiency: A new face of Achromobacter Xylosoxidans. ( 23173016 )
2012
20
A case of acute adrenal insufficiency unmasked during sunitinib treatment for metastatic renal cell carcinoma. ( 22581915 )
2012
21
Acute adrenal insufficiency due to bilateral adrenal hemorrhage. ( 21466057 )
2011
22
Acquired long QT interval in a recurrent septic female patient with polymorphic ventricular tachycardia due to primary acute adrenal insufficiency. ( 21161542 )
2011
23
Hypoglycaemic seizure and neonatal acute adrenal insufficiency after maternal exposure to prednisone during pregnancy: a case report. ( 19902248 )
2010
24
Acute adrenal insufficiency may affect outcome in the trauma patient. ( 19385286 )
2009
25
Primary antiphospholipid syndrome presenting as hyponatremic hypertensive syndrome and acute adrenal insufficiency following it: a rare case. ( 19365633 )
2009
26
Acute adrenal insufficiency associated with heparin-induced thrombocytopenia. ( 18575053 )
2008
27
Evaluation of serum cortisol in the postmortem diagnosis of acute adrenal insufficiency. ( 18520491 )
2008
28
Treatment of acute adrenal insufficiency. ( 17542196 )
2007
29
Acute adrenal insufficiency after a single dose of etomidate. ( 17456730 )
2007
30
[Acute adrenal insufficiency in the newborn]. ( 16962294 )
2006
31
Acute adrenal insufficiency. ( 17127145 )
2006
32
Reversible acute adrenal insufficiency caused by fluconazole in a critically ill patient. ( 16954446 )
2006
33
Images in cardiology: Acute adrenal insufficiency complicated by cardiogenic shock. ( 15772180 )
2005
34
Atypical presentation of shock from acute adrenal insufficiency in an adolescent male. ( 15942517 )
2005
35
Bilateral adrenal haemorrhage and acute adrenal insufficiency in a blunt abdominal trauma: a case-report and literature review. ( 15167178 )
2004
36
Acute adrenal insufficiency after large-dose glucocorticoids for spinal cord injury. ( 15562077 )
2004
37
Acute adrenal insufficiency presenting as high output ileostomy. ( 12648340 )
2003
38
Unusual presentations of thoracic tumors: Case 1. Acute adrenal insufficiency due to metastatic lung cancer. ( 11533102 )
2001
39
Hyponatremia as a consequence of acute adrenal insufficiency and hypothyroidism. ( 10748385 )
2000
40
Acute adrenal insufficiency after cardiac surgery. ( 10708203 )
2000
41
Acute adrenal insufficiency precipitated by isolated involvement of the adrenal gland by tuberculosis. ( 10714806 )
1999
42
Acute adrenal insufficiency in a patient with asthma after changing from fluticasone propionate to budesonide. ( 10329837 )
1999
43
Acute adrenal insufficiency during pregnancy and puerperium: case report and literature review. ( 10546275 )
1999
44
Transient ionized hypocalcemia and secondary hyperparathyroidism accompanying acute adrenal insufficiency. ( 15251746 )
1998
45
Acute adrenal insufficiency following unilateral radical nephrectomy: a case report. ( 9893218 )
1998
46
Bilateral adrenal hemorrhage resulting in acute adrenal insufficiency as an unusual complication of hepatic arterial chemoembolization. ( 9540911 )
1998
47
Acute adrenal insufficiency after coronary artery bypass grafting. ( 9262637 )
1997
48
Acute adrenal insufficiency after coronary artery bypass grafting. ( 8893598 )
1996
49
Evaluation of fluid volume status with a glucose challenge test in a patient with acute adrenal insufficiency. ( 23839557 )
1996
50
Post-thymectomy collapse: an unusual case of acute adrenal insufficiency. ( 7784288 )
1995

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

GO Terms for Acute Adrenal Insufficiency

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

id Name GO ID Score Top Affiliating Genes
1 steroid metabolic process GO:0008202 9.32 CYP11A1 CYP21A2
2 regulation of blood pressure GO:0008217 9.16 POMC REN
3 glucose homeostasis GO:0042593 8.96 IL6
4 steroid biosynthetic process GO:0006694 8.96 CYP11A1 CYP21A2
5 sterol metabolic process GO:0016125 8.62 CYP11A1 CYP21A2

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

id Name GO ID Score Top Affiliating Genes
1 heme binding GO:0020037 9.16 CYP11A1 CYP21A2
2 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen GO:0016705 8.96 CYP11A1 CYP21A2
3 receptor binding GO:0005102 8.8 IL6 POMC REN

Sources for Acute Adrenal Insufficiency

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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