MCID: WTR001
MIFTS: 35

Waterhouse-Friderichsen Syndrome

Categories: Endocrine diseases, Infectious diseases, Rare diseases

Aliases & Classifications for Waterhouse-Friderichsen Syndrome

MalaCards integrated aliases for Waterhouse-Friderichsen Syndrome:

Name: Waterhouse-Friderichsen Syndrome 12 73 20 58 44 15 17 70 32
Fatal Pneumococcal Waterhouse-Friderichsen Syndrome 20 70
Waterhouse-Friderichsen Syndrome, Meningococcal 70
Meningococcal Hemorrhagic Adrenalitis 12

Classifications:

Orphanet: 58  
Rare endocrine diseases


External Ids:

Disease Ontology 12 DOID:9931
MeSH 44 D014884
NCIt 50 C85225
SNOMED-CT 67 267484005
ICD10 32 A39.1
MESH via Orphanet 45 D014884
ICD10 via Orphanet 33 A39.1+ E35.1*
UMLS via Orphanet 71 C0043068 C1403891
Orphanet 58 ORPHA100067
UMLS 70 C0043068 C1403891 C2931288

Summaries for Waterhouse-Friderichsen Syndrome

GARD : 20 Waterhouse-Friderichsen syndrome is adrenal gland failure due to bleeding into the adrenal gland. It is usually caused by severe meningococcal infection or other severe, bacterial infection. Symptoms include acute adrenal gland insufficiency, and profound shock. Most patients with this condition are children, although adults may rarely be affected. It is deadly if not treated immediately.

MalaCards based summary : Waterhouse-Friderichsen Syndrome, also known as fatal pneumococcal waterhouse-friderichsen syndrome, is related to acute adrenal insufficiency and purpura. An important gene associated with Waterhouse-Friderichsen Syndrome is POMC (Proopiomelanocortin), and among its related pathways/superpathways are Formation of Fibrin Clot (Clotting Cascade) and Complement and coagulation cascades. Affiliated tissues include adrenal gland, heart and cortex, and related phenotypes are cellular and hematopoietic system

Disease Ontology : 12 An adrenal gland disease that is characterized by failure of the adrenal gland due to bleeding into the gland.

Wikipedia : 73 Waterhouse-Friderichsen syndrome (WFS) is defined as adrenal gland failure due to bleeding into the... more...

Related Diseases for Waterhouse-Friderichsen Syndrome

Diseases related to Waterhouse-Friderichsen Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 221)
# Related Disease Score Top Affiliating Genes
1 acute adrenal insufficiency 32.3 POMC CYP21A2
2 purpura 30.6 SERPINC1 F3 F2
3 disseminated intravascular coagulation 30.5 SERPINC1 F3 F2
4 purpura fulminans 30.4 SERPINC1 F3 F2
5 exanthem 30.3 F3 F2
6 lipoid congenital adrenal hyperplasia 30.2 POMC MC2R CYP21A2
7 meningitis 10.8
8 meningococcemia 10.7
9 haemophilus influenzae 10.7
10 meningococcal infection 10.6
11 toxic shock syndrome 10.6
12 meningococcal meningitis 10.5
13 neisseria meningitidis infection 10.5
14 chickenpox 10.4
15 premature ovarian failure 7 10.3
16 kidney cortex necrosis 10.3
17 shwartzman phenomenon 10.3
18 medulloadrenal hyperfunction 10.2 POMC F3
19 corticosteroid-binding globulin deficiency 10.2 POMC MC2R
20 glucocorticoid deficiency 1 10.2 POMC MC2R
21 cytochrome p450 oxidoreductase deficiency 10.2 POMC CYP21A2
22 classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency 10.2 POMC CYP21A2
23 adrenal hypoplasia, congenital 10.2 POMC MC2R
24 adrenal insufficiency, congenital, with 46,xy sex reversal, partial or complete 10.2 POMC CYP21A2
25 bronchopneumonia 10.2 F3 ACR
26 systemic lupus erythematosus 10.2
27 thrombocytopenic purpura, autoimmune 10.2
28 cyanosis, transient neonatal 10.2
29 streptococcus pneumonia 10.2
30 variola major 10.2
31 metabolic acidosis 10.2
32 optic papillitis 10.2
33 cholera 10.2
34 pericarditis 10.2
35 cholecystitis 10.2
36 pleural empyema 10.2
37 poliomyelitis 10.2
38 liver cirrhosis 10.2
39 peritonitis 10.2
40 scarlet fever 10.2
41 smallpox 10.2
42 cholangitis 10.2
43 bacterial meningitis 10.2
44 encephalitis 10.2
45 fasciitis 10.2
46 necrotizing fasciitis 10.2
47 toxoplasmosis 10.2
48 hypoglycemia 10.2
49 sickle cell disease 10.2
50 back pain 10.2

Graphical network of the top 20 diseases related to Waterhouse-Friderichsen Syndrome:



Diseases related to Waterhouse-Friderichsen Syndrome

Symptoms & Phenotypes for Waterhouse-Friderichsen Syndrome

MGI Mouse Phenotypes related to Waterhouse-Friderichsen Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.7 ACR F2 F3 MARCO POMC SCIN
2 hematopoietic system MP:0005397 9.5 CYP21A2 F3 MARCO MC2R POMC SCIN
3 immune system MP:0005387 9.23 CYP21A2 F2 F3 MARCO MC2R POMC

Drugs & Therapeutics for Waterhouse-Friderichsen Syndrome

Search Clinical Trials , NIH Clinical Center for Waterhouse-Friderichsen Syndrome

Cochrane evidence based reviews: waterhouse-friderichsen syndrome

Genetic Tests for Waterhouse-Friderichsen Syndrome

Anatomical Context for Waterhouse-Friderichsen Syndrome

MalaCards organs/tissues related to Waterhouse-Friderichsen Syndrome:

40
Adrenal Gland, Heart, Cortex, Pituitary, Spleen, Bone Marrow, Endothelial

Publications for Waterhouse-Friderichsen Syndrome

Articles related to Waterhouse-Friderichsen Syndrome:

(show top 50) (show all 307)
# Title Authors PMID Year
1
Fatal Waterhouse-Friderichsen syndrome in an adult due to serogroup Y Neisseria meningitidis. 61
33541984 2021
2
Fast Clinical, but Long-Term, Biochemical Remission after Waterhouse-Friderichsen Syndrome. 61
33542844 2021
3
Illustrated Forensic Pathology: Fatal Waterhouse-Friderichsen Syndrome. 61
32796209 2020
4
A Rare Case of Waterhouse-Friderichsen Syndrome Without Purpura Secondary to Haemophilus Influenzae. 61
32923222 2020
5
Neisseria meningitidis Induced Fatal Waterhouse-Friderichsen Syndrome in a Patient Presenting With Disseminated Intravascular Coagulation and Multiple Organ Failure. 61
32192003 2020
6
Waterhouse-Friderichsen Syndrome in an Infant. 61
32234194 2020
7
A unique fatal case of Waterhouse-Friderichsen syndrome caused by Proteus mirabilis in an immunocompetent subject: Case report and literature analysis. 61
31441842 2019
8
The importance of the postmortem interval for the diagnosis of Waterhouse-Friderichsen syndrome by Neisseria meningitidis in a series of forensic cases. 61
31372361 2019
9
Rare case of meningococcal sepsis-induced testicular failure, primary hypothyroidism and hypoadrenalism: Is there a link? 61
30219775 2018
10
[The Waterhouse-Friderichsen syndrome]. 61
30040272 2018
11
A case report of spontaneous staphylococcal meningitis in a cynomolgus monkey. 61
29399828 2018
12
[Management of severe sepsis using a Cytokin-adsorber]. 61
29247319 2018
13
Why Asplenic Patients Should Not Take Care of the Neighbour's Dog? A Fatal Course of Capnocytophaga canimorsus Sepsis. 61
30174969 2018
14
A rare case of Waterhouse- Friderichsen syndrome during primary Varicella zoster infection. 61
29219830 2017
15
Atriobronchial Fistula Complicated by Septic Cerebral Air Emboli After Pulmonary Vein Ablation. 61
28441232 2017
16
Waterhouse-Friderichsen syndrome due to Neisseria meningitidis infection in a young adult with thrombotic microangiopathy and eculizumab treatment: case report and review of management. 61
28213751 2017
17
Streptococcus Pneumoniae Detection Long Time After Death in a Fatal Case of Waterhouse-Friderichsen Syndrome. 61
28009598 2017
18
[Dyspnea and skin rash in a 49-year-old male patient]. 61
27900398 2017
19
Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult. 61
27583208 2016
20
A Case of Waterhouse-Friderichsen Syndrome Resulting from an Invasive Pneumococcal Infection in a Patient with a Hypoplastic Spleen. 61
26942021 2016
21
A rare case of Waterhouse-Friderichsen syndrome caused by Capnocytophaga canimorsus in an immunocompetent patient. 61
25676131 2015
22
An autopsy case of pneumococcal Waterhouse-Friderichsen syndrome with possible functional asplenia/hyposplenia. 61
26261663 2015
23
Fatal Waterhouse-Friderichsen syndrome due to serotype C Neisseria meningitidis in a young HIV negative MSM (men who have sex with men). 61
25267809 2014
24
Waterhouse Friderichsen syndrome complicating fulminant Enterobacter cloacae sepsis in a preterm infant: the unresolved issue of corticosteroids. 61
24328998 2014
25
[Acute adrenal insufficiency. Easy to miss Waterhouse-Friderichsen syndrome, as shown in two cases]. 61
24187895 2013
26
A fatal case of streptococcal and meningococcal meningitis in a 2-years-old child occurring as Waterhouse-Friderichsen Syndrome. 61
23910860 2013
27
Adrenomegaly and septic adrenal hemorrhage (Waterhouse-Friderichsen syndrome) in the setting of congenital adrenal hyperplasia. 61
23814386 2013
28
The significance of adrenal hemorrhage: undiagnosed Waterhouse-Friderichsen syndrome, a case series. 61
23458363 2013
29
Waterhouse-friderichsen syndrome in an adult patient with meningococcal meningitis. 61
23716842 2013
30
[Case report: A case of invasive pneumococcal disease with purpura fulminans and Waterhouse-Friderichsen syndrome]. 61
22693858 2012
31
Neisseria meningitidis induces platelet inhibition and increases vascular endothelial permeability via nitric oxide regulated pathways. 61
22072136 2011
32
A case of functional asplenia and pneumococcal sepsis. 61
21730800 2011
33
Waterhouse-friderichsen syndrome in an adult patient with meningococcal meningitis. 61
21772601 2011
34
Fatal dog bite in the absence of significant trauma: Capnocytophaga canimorsus infection and unexpected death. 61
20407357 2010
35
Waterhouse-friderichsen syndrome caused by invasive haemophilus influenzae type B infection in a previously healthy young man. 61
20191807 2010
36
Systemic inflammatory response syndrome in nonhuman primates culminating in multiple organ failure, acute lung injury, and disseminated intravascular coagulation. 61
19773593 2009
37
Fatal pneumococcal Waterhouse-Friderichsen syndrome in a vaccinated adult with congenital asplenia. 61
19751638 2009
38
[Puerperal septic shock due to beta-hemolytic Streptococcus and Waterhouse-Friderichsen syndrome]. 61
19287940 2009
39
[Foudroyant meningococcal sepsis in emergency medicine. Medical treatment and post-exposure prophylaxis]. 61
18270671 2008
40
Overwhelming postsplenectomy infection syndrome in adults - a clinically preventable disease. 61
18186551 2008
41
[Waterhouse-Friderichsen syndrome associated to a Morganella morganii and Enterococcus faecium peritonitis]. 61
17766079 2007
42
Medical image. Meningococcal septic shock with adrenal apoplexy--Waterhouse-Friderichsen syndrome. 61
16868589 2006
43
Invasive meningococcal disease associated with a very high case fatality rate in the North-West of Poland. 61
16487304 2006
44
Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children. 61
16177250 2005
45
Case of Waterhouse Friderichsen Syndrome during outbreak of meningococcal disease in Delhi in May 2005. 61
16749283 2005
46
OPSI (overwhelming postsplenectomy infection) syndrome: a case report. 61
15639588 2004
47
[Special emergencies in dermatology]. 61
15133618 2004
48
Waterhouse-Friderichsen syndrome as a result of non-meningococcal infection. 61
14747454 2004
49
Cardiac rescue with enoximone in volume and catecholamine refractory septic shock. 61
14525645 2003
50
Fatal Waterhouse-Friderichsen syndrome due to Ewingella americana infection. 61
12604997 2003

Variations for Waterhouse-Friderichsen Syndrome

Expression for Waterhouse-Friderichsen Syndrome

Search GEO for disease gene expression data for Waterhouse-Friderichsen Syndrome.

Pathways for Waterhouse-Friderichsen Syndrome

Pathways related to Waterhouse-Friderichsen Syndrome according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.33 SERPINC1 F3 F2
2 10.96 SERPINC1 F3 F2

GO Terms for Waterhouse-Friderichsen Syndrome

Biological processes related to Waterhouse-Friderichsen Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 blood coagulation GO:0007596 9.33 SERPINC1 F3 F2
2 regulation of blood coagulation GO:0030193 8.96 SERPINC1 F2
3 hemostasis GO:0007599 8.8 SERPINC1 F3 F2

Molecular functions related to Waterhouse-Friderichsen Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 9.13 F3 F2 ACR
2 protease binding GO:0002020 8.8 SERPINC1 F3 ACR

Sources for Waterhouse-Friderichsen Syndrome

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
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