MCID: CPL003
MIFTS: 53

Capillary Leak Syndrome malady

Cardiovascular diseases, Rare diseases, Bone diseases categories
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Summaries for Capillary Leak Syndrome

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Wikipedia:65 Capillary leak syndrome (also known as systemic capillary leak syndrome, SCLS, or Clarkson?s disease) is... more...

MalaCards based summary: Capillary Leak Syndrome is related to c1 inhibitor deficiency and psoriasis. An important gene associated with Capillary Leak Syndrome is C1S (complement component 1, s subcomponent), and among its related pathways are IL-15 Signaling and its Primary Biological Effects in Different Immune Cell Types and Selected targets of C EBPalpha. The compounds pegfilgrastim and molgramostim have been mentioned in the context of this disorder. Affiliated tissues include endothelial, bone marrow and bone, and related mouse phenotypes are other and tumorigenesis.

Aliases & Classifications for Capillary Leak Syndrome

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Sources:
8Disease Ontology, 10DISEASES, 44Novoseek, 62UMLS, 34MeSH, 57SNOMED-CT, 39NCIt
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Capillary Leak Syndrome, Aliases & Descriptions:

Name: Capillary Leak Syndrome 8 10 44 62


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Cardiovascular diseases, Bone diseases


External Ids:

Disease Ontology8 DOID:14400
MeSH34 D019559
SNOMED-CT57 87730004
NCIt39 C62578

Related Diseases for Capillary Leak Syndrome

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Diseases in the Systemic Capillary Leak Syndrome family:

capillary leak syndrome

Diseases related to Capillary Leak Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 132)
idRelated DiseaseScoreTop Affiliating Genes
1c1 inhibitor deficiency30.9SERPING1
2psoriasis30.6IL8, IL2, IL6, ELANE
3adult respiratory distress syndrome30.6IL8, ELANE
4pancytopenia30.5CSF3, CSF2
5pancreatitis30.3IL6, C1S, ELANE, ALB
6myeloma30.1ALB, CSF3, IL6, IL2, CSF2
7thrombocytopenia29.8IL2, IL6, CSF2, CSF3, ALB
8sepsis28.3IL6, C3, C1S, SERPING1, ELANE, IL8
9systemic capillary leak syndrome11.2
10compartment syndrome10.7
11endotheliitis10.6
12macroglossia10.6C1S
13pustulosis palmaris et plantaris10.5ELANE
14hepatopulmonary syndrome10.5ALB
15toxic shock syndrome10.5IL2
16malaria10.4ALB
17hepatitis10.4
18influenza10.4
19common cold10.4IL8
20t-cell large granular lymphocyte leukemia10.4IL2, CSF2
21periodontitis10.4IL8
22leukemia10.3
23brucellosis10.3
24cutaneous t cell lymphoma10.3
25generalized pustular psoriasis10.3
26bronchiolitis10.3IL8
27hereditary angioedema10.3SERPING1, C1S
28pulmonary sarcoidosis10.3CSF2, IL2
29ulcerative colitis10.3ALB
30angioedema10.3C1S, SERPING1
31acute kidney failure10.3C1S, ALB
32bronchopulmonary dysplasia10.2IL8, ELANE
33extrinsic allergic alveolitis10.2IL8, ELANE
34tetanus10.2IL2, CSF2, ALB
35pulmonary tuberculosis10.2IL2, C3
36bronchiectasis10.2IL8, ELANE
37alcoholic hepatitis10.2ALB, IL8
38mastitis10.2IL8, ALB
39bronchitis10.2ELANE, IL8
40periodontal disease10.2IL6, ELANE
41urticaria10.2C1S, SERPING1
42complement deficiency10.2C1S, C3, C4A
43leukemoid reaction10.2CSF3, CSF2
44alcoholic liver cirrhosis10.2IL8, ALB
45lupus erythematosus10.2C1S, C4A, C3
46acute lymphoblastic leukemia10.2
47anaplastic large cell lymphoma10.2
48b-cell lymphomas10.2
49diffuse large b-cell lymphoma10.2
50hemophagocytic lymphohistiocytosis10.2

Graphical network of the top 20 diseases related to Capillary Leak Syndrome:



Diseases related to capillary leak syndrome

Symptoms for Capillary Leak Syndrome

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Drugs & Therapeutics for Capillary Leak Syndrome

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Drug clinical trials:

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Search NIH Clinical Center for Capillary Leak Syndrome

Genetic Tests for Capillary Leak Syndrome

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Anatomical Context for Capillary Leak Syndrome

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MalaCards organs/tissues related to Capillary Leak Syndrome:

32
Endothelial, Bone marrow, Bone, Lung, T cells, Breast, Heart, Skeletal muscle, Small intestine, Kidney, Liver, B cells

Animal Models for Capillary Leak Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Capillary Leak Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053959.4CSF2, C3, IL2
2MP:00020068.3IL6, IL2, ALB, ELANE, CSF2
3MP:00053677.9C3, CSF2, CSF3, ALB, IL6
4MP:00053797.9ALB, IL2, IL6, C3, CSF2
5MP:00053977.2ELANE, CSF3, C3, IL6, IL8, IL2
6MP:00053877.2C3, IL8, CSF3, ELANE, IL2, CSF2

Publications for Capillary Leak Syndrome

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Articles related to Capillary Leak Syndrome:

(show top 50)    (show all 184)
idTitleAuthorsYear
1
Invasive pulmonary aspergillosis in a patient presenting with idiopathic systemic capillary leak syndrome. (24859554)
2014
2
Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). (25405070)
2014
3
Clarkson disease - systemic capillary leak syndrome in a 6-year-old girl: case report. (25311880)
2014
4
Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature. (24490827)
2014
5
Systemic capillary leak syndrome associated with a rare abdominal and four-limb compartment syndrome: a case report. (24934689)
2014
6
An unusual cause of secondary capillary leak syndrome in a child: rotavirus diarrhea. (23692840)
2013
7
Genome-wide SNP analysis of the Systemic Capillary Leak Syndrome (Clarkson disease). (24808988)
2013
8
Episodic idiopathic systemic capillary leak syndrome in a girl. (23910812)
2013
9
Systemic capillary leak syndrome: recognition prevents morbidity and mortality. (24134172)
2013
10
Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma. (22953081)
2012
11
The systemic capillary leak syndrome: a scarcely known nephrological entity. (22135035)
2012
12
A case of brucellosis complicated with fatal capillary leak syndrome. (22897381)
2012
13
The systemic capillary leak syndrome: a case series of 28 patients from a European registry. (21464348)
2011
14
Idiopathic systemic capillary leak syndrome preceding diagnosis of infiltrating lobular carcinoma of the breast with quiescence during neoadjuvant chemotherapy. (21364118)
2011
15
Capillary leak syndrome: initial presentation in a patient with ALK+a88anaplastic large cell lymphoma associated with increased levels of serum cytokines. (21463119)
2011
16
Rapid improvement of life-threatening capillary leak syndrome after stem cell transplantation by bevacizumab. (20360479)
2010
17
Neurologic involvement in a child with systemic capillary leak syndrome. (20123768)
2010
18
Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience. (20634497)
2010
19
A case report of systemic capillary leak syndrome (Clarkson's disease). (20148770)
2010
20
Recurrent capillary leak syndrome following bortezomib therapy in a patient with relapsed myeloma. (20150504)
2010
21
The systemic capillary leak syndrome. (21135304)
2010
22
Systemic sclerosis, capillary leak syndrome and nasopharyngeal carcinoma: an unusual association or paraneoplastic manifestations? (19052056)
2009
23
Systemic capillary leak syndrome or Clarkson's disease: a case report. (19526356)
2009
24
Chronic systemic capillary leak syndrome: a case responding to prednisolone treatment. (20166237)
2009
25
High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome. (18552679)
2008
26
Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis. (16802499)
2006
27
Severe gemcitabine-induced capillary-leak syndrome mimicking cardiac failure in a patient with advanced pancreatic cancer and high-risk cardiovascular disease. (15630853)
2004
28
Intraperitoneal photodynamic therapy causes a capillary-leak syndrome. (12794017)
2003
29
An unusual evolution of the systemic capillary leak syndrome. (11865098)
2002
30
Systemic capillary leak syndrome. (12487153)
2002
31
Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome. (11591575)
2001
32
G-CSF related capillary leak syndrome in a child with leukemia. (11911434)
2001
33
Pulmonary capillary leak syndrome with intravenous cyclosporin A in pediatric renal transplantation. (10731056)
2000
34
Hypoalbuminemia, surgical leak, and clinical capillary leak syndrome. (10636355)
2000
35
Systemic toxicity following administration of sirolimus (formerly rapamycin) for psoriasis: association of capillary leak syndrome with apoptosis of lesional lymphocytes. (10328196)
1999
36
Capillary leak syndrome in a patient with septicemia and granulocyte-colony-stimulating factor (G-CSF)-induced accelerated granulopoiesis. (9680943)
1998
37
Multisystem organ failure and capillary leak syndrome in severe necrotizing enterocolitis of very low birth weight infants. (9537561)
1998
38
Granulocyte-macrophage colony-stimulating factor-associated histiocytosis and capillary-leak syndrome following autologous bone marrow transplantation: two case reports and a review of the literature. (9489640)
1998
39
Systemic capillary leak syndrome: where does the fluid go? (9799931)
1998
40
Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment. (9428835)
1997
41
Pulmonary capillary leak syndrome associated with the use of intravenous cyclosporin. (8941609)
1996
42
Biclonal SAczary syndrome with capillary leak syndrome. (8136545)
1994
43
Capillary leak syndrome associated with elevated IL-2 serum levels after allogeneic bone marrow transplantation. (8110879)
1994
44
Idiopathic capillary leak syndrome complicated by massive rhabdomyolysis. (8325053)
1993
45
Control of systemic capillary leak syndrome with aminophylline and terbutaline. (1580299)
1992
46
Pulmonary capillary leak syndrome complicating generalized pustular psoriasis: possible role of cytokines. (1751356)
1991
47
Muscle biopsy findings in systemic capillary leak syndrome. (2179110)
1990
48
Systemic capillary leak syndrome. (2563486)
1989
49
Anterior tibial compartment syndrome secondary to systemic capillary leak syndrome. (3625226)
1987
50
Capillary leak syndrome with pulmonary edema. (5035983)
1972

Variations for Capillary Leak Syndrome

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Expression for genes affiliated with Capillary Leak Syndrome

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Expression patterns in normal tissues for genes affiliated with Capillary Leak Syndrome

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Pathways for genes affiliated with Capillary Leak Syndrome

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Pathways related to Capillary Leak Syndrome according to GeneCards/GeneDecks:

(show all 40)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.8CSF2, IL2
29.8ELANE, ALB
39.6IL8, CSF2
49.3ELANE, IL6
5
Show member pathways
9.3C4A, C3, C1S
69.3C1S, C3, C4A
7
Show member pathways
Calcium signaling in the CD4+ TCR pathway37
9.2IL8, IL2, CSF2
89.0IL8, IL6
99.0IL6, IL8
10
Show member pathways
9.0CSF2, IL6, IL2
11
Show member pathways
9.0IL2, IL6, CSF2
12
Show member pathways
BCR signaling pathway37
TCR Signaling Pathway37
9.0IL2, CSF2, IL6
139.0IL2, IL6, CSF2
14
Show member pathways
9.0C4A, ELANE, C1S, C3
15
Show member pathways
8.9IL2, IL6, C4A
16
Show member pathways
8.9IL6, C3, IL2
178.8IL6, CSF3, CSF2
18
Show member pathways
Complement Activation, Classical Pathway37
Complement and Coagulation Cascades37
8.8C1S, C3, SERPING1, C4A
19
Show member pathways
Immune response Alternative complement pathway60
Immune response Lectin Induced complement pathway60
Immune response Classic complement pathway60
8.8C1S, SERPING1, C3, C4A
20
Show member pathways
8.7IL6, IL8, CSF2
218.7IL8, IL6, CSF2
228.7IL6, IL8, CSF2
238.7IL2, IL6, IL8
248.7IL2, IL8, IL6
258.7IL6, IL8, IL2
268.6C4A, IL6, IL8
278.6C3, IL6, IL8
288.5IL8, CSF3, IL6
29
Show member pathways
Type III interferon signaling37
8.5CSF2, IL2, CSF3, IL6
308.5IL6, CSF3, IL2, CSF2
318.4ELANE, IL8, IL6, CSF2
328.4IL8, IL2, CSF2, IL6
33
Show member pathways
8.2C3, IL2, IL6, IL8
34
Show member pathways
Toll-like receptor signaling pathway37
Regulation of toll-like receptor signaling pathway37
8.2C3, IL2, IL6, IL8
35
Show member pathways
8.2IL8, CSF3, CSF2, IL6
36
Show member pathways
7.9CSF2, CSF3, IL6, IL8, IL2
37
Show member pathways
7.9IL6, IL2, IL8, CSF2, CSF3
38
Show member pathways
7.9IL2, CSF2, IL8, IL6, CSF3
39
Show member pathways
7.9CSF3, IL6, IL8, IL2, CSF2
407.4C1S, IL6, C4A, C3, IL8, SERPING1

Compounds for genes affiliated with Capillary Leak Syndrome

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Compounds related to Capillary Leak Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 158)
idCompoundScoreTop Affiliating Genes
1pegfilgrastim44 1110.7CSF2, ELANE, CSF3
2molgramostim449.7CSF3, IL2, CSF2
3dacarbazine44 1110.6CSF3, IL2, CSF2
4bcnu449.5CSF3, IL2, CSF2
5salmeterol44 50 28 1112.5IL2, CSF2, IL8
6zidovudine44 50 1111.3CSF2, CSF3, ALB, IL2
7pixy321449.2IL6, CSF2, CSF3
8okt3449.2CSF2, IL6, IL2
9fluticasone propionate44 50 61 1112.1ELANE, IL8, CSF2
10sivelestat449.1IL6, ELANE, IL8
11olopatadine44 1110.1IL6, CSF2, IL8
12tepoxalin449.1IL8, IL2, IL6
13n-formylmethionyl-leucylphenylalanine449.1IL8, CSF2, CSF3, ELANE
14gabexate mesilate449.1IL6, IL8, ELANE
15taurine chloramine449.1IL6, IL8, IL2
16ulinastatin449.1IL6, ELANE, IL8
17mip 1alpha449.1IL6, CSF2, IL8
18imiquimod44 61 28 1112.0CSF2, IL6, IL8
19filgrastim44 119.9CSF3, CSF2, IL6, ELANE
20fmlp448.9ELANE, IL8, CSF2, CSF3
21groalpha448.9CSF2, IL8, IL6
22il-12448.8IL8, IL6, CSF2, IL2
23aprotinin44 119.8IL6, IL8, ELANE, C1S
24triptolide448.8IL2, IL6, IL8
25il 10448.8IL6, IL8, IL2, CSF2
26protamine448.8IL2, IL6, ELANE, IL8
27etanercept44 50 1110.7C1S, IL8, IL6
28leukotriene b444 61 2410.7IL6, ELANE, CSF2, IL8
29prednisolone44 28 1110.7IL6, CSF3, ALB, IL2
30rituximab44 50 1110.6CSF3, CSF2, IL6, IL2, C1S
31calcitriol44 61 24 1111.6ALB, IL2, IL6, CSF2
32herbimycin a44 619.6IL2, IL6, IL8, CSF2
33fibrinogen448.6C1S, ELANE, ALB, IL6, C3
34pentoxifylline44 119.5CSF2, IL6, IL8, IL2, ELANE
35hydrocortisone44 2 61 1111.5IL2, IL6, ELANE, IL8, CSF2
36hyaluronic acid44 249.5CSF2, IL6, ALB, IL8, ELANE
37polyinosinic-polycytidylic acid448.4IL8, IL6, IL2
38histamine44 28 2410.3IL8, IL2, IL6, CSF2, ALB
39pge2448.3IL6, CSF2, IL8, IL2, ELANE
40rantes448.3IL6, CSF3, CSF2, IL8, IL2
41neopterin448.1ELANE, IL2, IL8, IL6, C3, CSF2
42heparin44 28 24 1111.0C1S, SERPING1, ELANE, IL8, ALB, C3
43anaphylatoxin448.0C4A, ELANE, IL6, IL8, C3, CSF2
44gm-csf44 289.0IL2, IL8, IL6, CSF2, CSF3, ELANE
45lactate448.0IL6, ALB, CSF2, ELANE, IL8, C3
46cyclosporin a44 28 6110.0CSF3, ELANE, IL2, IL8, IL6, CSF2
47cycloheximide447.9IL8, CSF3, IL2, IL6, CSF2
48endotoxin447.9C4A, CSF3, IL6, IL8, ELANE, CSF2
49serine447.8C3, IL8, IL2, C4A, ELANE, SERPING1
50dexamethasone44 50 28 1110.7CSF2, C3, CSF3, IL6, IL2, IL8

GO Terms for genes affiliated with Capillary Leak Syndrome

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Cellular components related to Capillary Leak Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:0310939.6ALB, SERPING1
2blood microparticleGO:0725628.1SERPING1, C3, ALB, C4A, C1S
3extracellular spaceGO:0056156.4SERPING1, IL2, IL8, IL6, C3, CSF2
4extracellular regionGO:0055766.0C4A, C1S, SERPING1, ELANE, IL2, IL8

Biological processes related to Capillary Leak Syndrome according to GeneCards/GeneDecks:

(show all 15)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of tyrosine phosphorylation of Stat5 proteinGO:0425239.9CSF2, IL2
2complement activationGO:0069569.6C1S, C3, C4A
3negative regulation of chemokine biosynthetic processGO:0450799.6IL6, ELANE
4regulation of complement activationGO:0304499.5C3, C4A
5positive regulation of immunoglobulin secretionGO:0510249.5IL2, IL6
6positive regulation of smooth muscle cell proliferationGO:0486619.3IL6, ELANE
7negative regulation of neuron deathGO:19012159.3CSF3, IL6
8cellular response to lipopolysaccharideGO:0712229.3CSF3, CSF2, IL8
9complement activation, classical pathwayGO:0069589.1C4A, C3, SERPING1, C1S
10positive regulation of DNA replicationGO:0457409.1CSF2, IL6
11innate immune responseGO:0450878.9C1S, SERPING1, C3, C4A
12platelet activationGO:0301688.8ALB, IL6, SERPING1
13positive regulation of cell proliferationGO:0082848.6IL2, IL6, CSF2, CSF3
14immune responseGO:0069558.5IL2, IL8, C3, CSF2, CSF3
15inflammatory responseGO:0069548.4IL8, IL6, C3, C4A

Molecular functions related to Capillary Leak Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1endopeptidase inhibitor activityGO:0048669.6C3, C4A
2growth factor activityGO:0080838.5IL2, IL6, CSF3, CSF2
3cytokine activityGO:0051258.4IL2, IL6, CSF2, CSF3
4protein bindingGO:0055156.5C1S, SERPING1, ELANE, IL8, IL6, C3

Products for genes affiliated with Capillary Leak Syndrome

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Sources for Capillary Leak Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet