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MCID: ACT150
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Acute Adrenal Insufficiency

Categories: Endocrine diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Acute Adrenal Insufficiency

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MalaCards integrated aliases for Acute Adrenal Insufficiency:

Name: Acute Adrenal Insufficiency 58 54
Addisonian Crisis 58 70 32
Acute Adrenocortical Insufficiency 58
Acute Adrenal Failure 58
Adrenocortical Crisis 58
Adrenal Crisis 58

Characteristics:

Orphanet epidemiological data:

58
acute adrenal insufficiency
Inheritance: Autosomal recessive,Not applicable,X-linked dominant; Age of onset: All ages; Age of death: any age;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Endocrine diseases
See all MalaCards categories (disease lists)
ICD10: 32 33
Orphanet: 58  
Rare endocrine diseases


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Summaries for Acute Adrenal Insufficiency

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MalaCards based summary : Acute Adrenal Insufficiency, also known as addisonian crisis, is related to classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency and adrenal insufficiency, congenital, with 46,xy sex reversal, partial or complete. 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 Aldosterone synthesis and secretion and Interleukin-4 and 13 signaling. The drugs Epinephrine and Racepinephrine have been mentioned in the context of this disorder. Affiliated tissues include adrenal gland, pituitary and cortex, and related phenotypes are decreased circulating cortisol level and failure to thrive

Wikipedia : 73 Adrenal crisis is a potentially life-threatening medical condition requiring immediate emergency... more...

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Related Diseases for Acute Adrenal Insufficiency

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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 308)
# Related Disease Score Top Affiliating Genes
1 classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency 31.5 POMC CYP21A2
2 adrenal insufficiency, congenital, with 46,xy sex reversal, partial or complete 31.4 POMC CYP21A2 CYP11A1
3 waterhouse-friderichsen syndrome 30.2 POMC CYP21A2
4 diabetes insipidus 30.1 REN POMC
5 thyroid gland disease 29.9 POMC IL6
6 pericardial effusion 29.9 REN IL6
7 neurotic disorder 29.8 POMC IL6
8 gastroenteritis 29.8 REN IL6
9 hypokalemia 29.8 REN POMC
10 placenta disease 29.7 REN IL6
11 gynecomastia 29.5 POMC CYP21A2
12 diarrhea 29.4 REN POMC IL6
13 adrenal cortical carcinoma 29.3 POMC CYP21A2 CYP11A1
14 adenoma 29.3 REN POMC CYP21A2
15 chronic kidney disease 29.2 REN POMC IL6
16 pseudohermaphroditism 29.2 POMC CYP21A2 CYP11A1
17 hypoadrenocorticism, familial 29.1 REN POMC CYP21A2 CYP11A1
18 lipoid congenital adrenal hyperplasia 29.1 REN POMC CYP21A2 CYP11A1
19 familial glucocorticoid deficiency 28.9 REN POMC CYP21A2 CYP11A1
20 adrenal cortical adenoma 28.9 REN POMC CYP21A2 CYP11A1
21 autoimmune addison disease 11.2
22 hypothyroidism, congenital, nongoitrous, 4 11.1
23 toxic shock syndrome 10.4
24 antiphospholipid syndrome 10.3
25 histoplasmosis 10.3
26 primary adrenal insufficiency 10.3
27 dowling-degos disease 1 10.3
28 thrombocytopenia 10.2
29 heparin-induced thrombocytopenia 10.2
30 hypothyroidism 10.1
31 pneumonia 10.1
32 hypopituitarism 10.1
33 secondary adrenal insufficiency 10.1
34 atrial standstill 1 10.1
35 cardiac tamponade 10.1
36 pericarditis 10.1
37 hypogonadism 10.1
38 somatization disorder 10.1 POMC IL6
39 atypical depressive disorder 10.1 POMC IL6
40 acute stress disorder 10.1 POMC IL6
41 euthyroid sick syndrome 10.1 POMC IL6
42 melancholia 10.1 POMC IL6
43 dysthymic disorder 10.1 POMC IL6
44 autoimmune disease 10.1
45 graves disease 1 10.1
46 premature ovarian failure 7 10.1
47 cardiac arrest 10.1
48 adenocarcinoma 10.1
49 hyperglycemia 10.1
50 thyroiditis 10.1

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



Diseases related to Acute Adrenal Insufficiency
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Symptoms & Phenotypes for Acute Adrenal Insufficiency

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Human phenotypes related to Acute Adrenal Insufficiency:

31 58 (show all 48)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 decreased circulating cortisol level 31 obligate (100%) HP:0008163
2 failure to thrive 58 31 hallmark (90%) Very frequent (99-80%) HP:0001508
3 nausea and vomiting 58 31 hallmark (90%) Very frequent (99-80%) HP:0002017
4 constipation 58 31 hallmark (90%) Very frequent (99-80%) HP:0002019
5 muscle weakness 58 31 hallmark (90%) Very frequent (99-80%) HP:0001324
6 fatigue 58 31 hallmark (90%) Very frequent (99-80%) HP:0012378
7 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
8 abdominal pain 58 31 hallmark (90%) Very frequent (99-80%) HP:0002027
9 anorexia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002039
10 weight loss 58 31 hallmark (90%) Very frequent (99-80%) HP:0001824
11 diarrhea 58 31 hallmark (90%) Very frequent (99-80%) HP:0002014
12 hyperpigmentation of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0000953
13 hypovolemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0011106
14 increased circulating acth level 58 31 hallmark (90%) Very frequent (99-80%) HP:0003154
15 renal insufficiency 58 31 frequent (33%) Frequent (79-30%) HP:0000083
16 hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0001943
17 hyperuricemia 58 31 frequent (33%) Frequent (79-30%) HP:0002149
18 hyponatremia 58 31 frequent (33%) Frequent (79-30%) HP:0002902
19 hyperkalemia 58 31 frequent (33%) Frequent (79-30%) HP:0002153
20 orthostatic hypotension 58 31 frequent (33%) Frequent (79-30%) HP:0001278
21 decreased circulating aldosterone level 58 31 frequent (33%) Frequent (79-30%) HP:0004319
22 normocytic anemia 58 31 frequent (33%) Frequent (79-30%) HP:0001897
23 renal salt wasting 58 31 frequent (33%) Frequent (79-30%) HP:0000127
24 increased circulating renin level 58 31 frequent (33%) Frequent (79-30%) HP:0000848
25 androgen insufficiency 58 31 frequent (33%) Frequent (79-30%) HP:0008226
26 decreased urinary potassium 58 31 frequent (33%) Frequent (79-30%) HP:0012364
27 hyperkalemic metabolic acidosis 58 31 frequent (33%) Frequent (79-30%) HP:0005976
28 seizure 31 frequent (33%) HP:0001250
29 hypotonia 31 frequent (33%) HP:0001252
30 recurrent acute respiratory tract infection 31 frequent (33%) HP:0011948
31 delayed puberty 58 31 occasional (7.5%) Occasional (29-5%) HP:0000823
32 dry skin 58 31 occasional (7.5%) Occasional (29-5%) HP:0000958
33 vitiligo 58 31 occasional (7.5%) Occasional (29-5%) HP:0001045
34 myocardial infarction 58 31 occasional (7.5%) Occasional (29-5%) HP:0001658
35 vertigo 58 31 occasional (7.5%) Occasional (29-5%) HP:0002321
36 arthralgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002829
37 stroke 58 31 occasional (7.5%) Occasional (29-5%) HP:0001297
38 hypercalcemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003072
39 sparse axillary hair 58 31 occasional (7.5%) Occasional (29-5%) HP:0002215
40 adrenal hypoplasia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000835
41 decreased female libido 58 31 occasional (7.5%) Occasional (29-5%) HP:0030018
42 salt craving 58 31 occasional (7.5%) Occasional (29-5%) HP:0030083
43 seizures 58 Frequent (79-30%)
44 hypotension 58 Very frequent (99-80%)
45 muscular hypotonia 58 Frequent (79-30%)
46 primary adrenal insufficiency 58 Obligate (100%)
47 hypocortisolemia 58 Obligate (100%)
48 acute respiratory tract infection 58 Frequent (79-30%)

MGI Mouse Phenotypes related to Acute Adrenal Insufficiency:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.55 CYP11A1 CYP21A2 IL6 POMC REN
2 hematopoietic system MP:0005397 9.35 CYP11A1 CYP21A2 IL6 POMC REN
3 immune system MP:0005387 9.02 CYP11A1 CYP21A2 IL6 POMC REN
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Drugs & Therapeutics for Acute Adrenal Insufficiency

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

(show all 28)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Epinephrine Approved, Vet_approved Phase 4 51-43-4 5816
2
Racepinephrine Approved Phase 4 329-65-7 838
3
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 5754
4
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
5
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
6
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
7
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
8
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
9
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
10
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
11
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
12 Hormones Phase 4
13 Epinephryl borate Phase 4
14 Hydrocortisone 17-butyrate 21-propionate Phase 4
15 glucocorticoids Phase 4
16 Hydrocortisone hemisuccinate Phase 4
17 Hormone Antagonists Phase 4
18 Antineoplastic Agents, Hormonal Phase 4
19 Anti-Inflammatory Agents Phase 4
20 Methylprednisolone Acetate Phase 4
21 Gastrointestinal Agents Phase 4
22 Vasoconstrictor Agents Phase 4
23 Hydrocortisone-17-butyrate Phase 4
24 Antiemetics Phase 4
25 Neuroprotective Agents Phase 4
26 Protective Agents Phase 4
27
Dehydroepiandrosterone Approved, Investigational, Nutraceutical 53-43-0 5881
28 DHEA (Dehydroepiandrosterone)

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;Corticosteroid
2 Vaginal Prednisone Administration for Prevention of Adrenal Crisis - a Bioequivalence Study Completed NCT02689960 Phase 2 100mg prednisone suppository
3 National (German-wide) Education Programme for Patients With Chronic Adrenal Insufficiency Completed NCT02694926
4 Survey in Patients With Chronic Adrenal Insufficiency in Germany Completed NCT00444119
5 Prospective Cohort-study on the Incidence of Adrenal Crisis in Patients With Chronic Adrenal Insufficiency Completed NCT01184209
6 Prospective Study to Investigate Adrenal Crisis in Patients With Chronic Adrenal Insufficiency (Six-year Follow-up) Completed NCT03499990
7 Predisposing Factors for the Incidence of Adrenal Crisis in Chronic Adrenal Insufficiency Active, not recruiting NCT03410160

Search NIH Clinical Center for Acute Adrenal Insufficiency

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Genetic Tests for Acute Adrenal Insufficiency

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Anatomical Context for Acute Adrenal Insufficiency

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MalaCards organs/tissues related to Acute Adrenal Insufficiency:

40
Adrenal Gland, Pituitary, Cortex, Kidney, Adrenal Cortex, Heart, Spinal Cord
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Publications for Acute Adrenal Insufficiency

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Articles related to Acute Adrenal Insufficiency:

(show top 50) (show all 600)
# Title Authors PMID Year
1
Hypopituitarism. 61 54
17077946 2006
2
Congenital adrenal hyperplasia. 61 54
15568308 2004
3
Lessons of the month: A challenging presentation of hypopituitarism secondary to an intracerebral aneurysm. 61
33762391 2021
4
Medical Management of Heparin-Induced Thrombocytopenia Causing Acute Adrenal Insufficiency. 61
33754099 2021
5
Bilateral adrenal hemorrhage after colectomy for perforated diverticulitis: A case report. 61
33388513 2021
6
A Case of Lung Adenocarcinoma with Long-Term Response after Late-Onset Pembrolizumab-Induced Acute Adrenal Insufficiency. 61
33613234 2021
7
Takotsubo cardiomyopathy associated with adrenal insufficiency in the context of long-term steroid use mimicking acute coronary syndrome. 61
33495195 2021
8
Typical and reverse Takotsubo syndromes as initial manifestations of consecutive Addisonian crises in a 38-year-old patient: the heart has its reasons! 61
33509872 2021
9
Acute adrenal insufficiency due to paracoccidiodomycosis. Report of 2 cases. 61
32874849 2020
10
Bilateral Adrenal Hemorrhage in Coronavirus Disease 2019 Patient: A Case Report. 61
32738040 2020
11
Case report: Managing profound circulatory collapse post-atrial fibrillation ablation: a methodical approach. 61
33442609 2020
12
Bilateral Adrenocortical Carcinoma Presenting as Acute Adrenal Insufficiency. 61
33117452 2020
13
Reversibility of Acute Adrenal Insufficiency after hip Replacement: A Case Series. 61
33050871 2020
14
A pictorial review of non-traumatic adrenergic crisis. 61
32300900 2020
15
Adrenal insufficiency in coronavirus disease 2019: a case report. 61
32838801 2020
16
Allgrove Syndrome: A Report of New Pathological Variants in the AAAS Gene. 61
32073457 2020
17
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. 61
32017012 2020
18
Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with Bilateral Adrenal Hemorrhage in Two Caucasian Patients. 61
32249274 2020
19
Multiple endocrinological failures as a clinical presentation of a metastatic lung adenocarcinoma. 61
32478672 2020
20
Fatal adrenal crisis due to Addison's disease arising in the context of autoimmune polyglandular syndrome type 1. 61
31782110 2020
21
Acute adrenal insufficiency as a mysterious cause of shock following percutaneous coronary intervention: a cardiologist's nightmare. 61
32169991 2020
22
Acute management of Addisonian crisis - a quality improvement project. 61
32409395 2020
23
Surgical approach to patients with pheochromocytoma. 61
32206597 2020
24
Retroperitoneoscopic partial adrenalectomy for metachronous renal cell carcinoma metastasis to solitary adrenal gland. 61
31950008 2020
25
A rare case of adrenal collision tumor: Myelolipoma and schwannoma in the adrenal gland. 61
32477562 2020
26
Cortisol and Aldosterone Responses to Hypoglycemia and Na Depletion in Women With Non-Classic 21-Hydroxylase Deficiency. 61
31529070 2020
27
[Fatal outcome of Netherton syndrome due to excessive use of topical corticosteroids in an adult patient]. 61
31653452 2020
28
Rare Bilateral Adrenal Haemorrhage with Addisonian Crisis: When Risk Factors Come in Droves. 61
32832170 2020
29
A Rare Presentation of Possible Disseminated Histoplasmosis with Adrenal Insufficiency Leading to Adrenal Crisis in an Immunocompetent Adult: A Case Report. 61
32256604 2020
30
Potential risk of impertinent administration of methylprednisolone in lumbar spine surgery: A case report. 61
32971446 2020
31
Disseminated histoplasmosis in India presenting as addisonian crisis with epiglottis involvement. 61
32514395 2020
32
Acute Adrenal Insufficiency in a Patient with Metastatic Infiltrating Ductal Carcinoma. 61
33312414 2020
33
SEEN guidelines for the management and prevention of acute adrenal insufficiency. 61
31003863 2020
34
A Case of a Post-Operative Addisonian Crisis from HPA Axis Suppression from Inhaled Corticosteroids. 61
31638547 2019
35
ELECTROCARDIOGRAM CHANGES IN ADDISON DISEASE: POTENTIAL CLINICAL MARKER FOR ADRENAL CRISIS. 61
31967059 2019
36
Perioperative management in a patient with panhypopituitarism - evidence based approach: a case report. 61
31660506 2019
37
Acute Adrenal Insufficiency Precipitated by the Discontinuation of a Betamethasone and Dextrochlorpheniramine Combination: The Diagnostic Utility of an Echocardiographic Assessment of Systemic Vascular Resistance. 61
30996179 2019
38
Fluorescence techniques in adrenal surgery. 61
31404180 2019
39
Management of Endocrine Emergencies in the ICU. 61
30852927 2019
40
Acute Adrenal Insufficiency After an Antiemetic Dose of Dexamethasone: A Case Report. 61
30633007 2019
41
Management of blunt adrenal gland injury in a community-based hospital. 61
30606456 2019
42
Multi-detector computed tomography in the diagnosis and characterization of adrenal gland traumatic injuries. 61
31183326 2019
43
Neuropsychiatric Manifestation of Addison's Disease: A Rare Case Report. 61
31192061 2019
44
Acute Adrenal Insufficiency in the Perioperative Period: A Case Report. 61
30020102 2019
45
Bilateral adrenal myelolipomas presenting as acute adrenal insufficiency in an adult with congenital adrenal hyperplasia. 61
30739087 2019
46
Catastrophic antiphospholipid syndrome causing ST-segment elevation myocardial infarction with non-obstructive coronary arteries. 61
30635301 2019
47
A case of subacute combined degeneration of the spinal cord due to folic acid and copper deficiency. 61
30104084 2019
48
Pituitary Apoplexy Following Systemic Anticoagulation. 61
31893198 2019
49
Frequent and prolonged administration of glucocorticoid for acute adrenal insufficiency treatment can cause diabetes mellitus: A case of holoprosencephaly. 61
31037021 2019
50
A Case of Acute Exacerbation of Chronic Adrenal Insufficiency Due to Ipilimumab Treatment for Advanced Melanoma. 61
30679413 2019
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Variations for Acute Adrenal Insufficiency

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Expression for Acute Adrenal Insufficiency

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

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

# Super pathways Score Top Affiliating Genes
1
Aldosterone synthesis and secretion 36
Show member pathways
 12.27 POMC CYP21A2 CYP11A1
2
Interleukin-4 and 13 signaling 65
11.46 POMC IL6
3
Diseases of metabolism 65
Show member pathways
 11.34 POMC CYP11A1
4
Glucocorticoid receptor regulatory network 1
11.17 POMC IL6
5
Corticotropin-releasing hormone signaling pathway 1
11.1 POMC CYP21A2 CYP11A1
6
Steroid hormone biosynthesis 36
Show member pathways
 10.98 CYP21A2 CYP11A1
7
Metabolism of steroid hormones 65
Show member pathways
 10.7 POMC CYP21A2 CYP11A1
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GO Terms for Acute Adrenal Insufficiency

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

# Name GO ID Score Top Affiliating Genes
1 glucose homeostasis GO:0042593 9.32 POMC IL6
2 regulation of blood pressure GO:0008217 9.26 REN POMC
3 steroid biosynthetic process GO:0006694 9.16 CYP21A2 CYP11A1
4 sterol metabolic process GO:0016125 8.96 CYP21A2 CYP11A1
5 glucocorticoid biosynthetic process GO:0006704 8.62 CYP21A2 CYP11A1

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

# Name GO ID Score Top Affiliating Genes
1 monooxygenase activity GO:0004497 8.96 CYP21A2 CYP11A1
2 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen GO:0016705 8.62 CYP21A2 CYP11A1
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Sources for Acute Adrenal Insufficiency

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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