SCLS
MCID: SYS007
MIFTS: 30

Systemic Capillary Leak Syndrome (SCLS) malady

Categories: Rare diseases, Bone diseases, Cardiovascular diseases

Aliases & Classifications for Systemic Capillary Leak Syndrome

Aliases & Descriptions for Systemic Capillary Leak Syndrome:

Name: Systemic Capillary Leak Syndrome 50 56
Capillary Leak Syndrome 50 56 69
Capillary Leak Syndrome with Monoclonal Gammopathy 50 69
Clarkson Disease 50 56
Scls 50 56
Periodic Systemic Capillary Leak Syndrome 50
Capillary Hyperpermeability Syndrome 56
Idiopathic Capillary Leak Syndrome 56

Characteristics:

Orphanet epidemiological data:

56
systemic capillary leak syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: adult;

Classifications:



External Ids:

Orphanet 56 ORPHA188
ICD10 via Orphanet 34 I78.8
MESH via Orphanet 43 D019559
UMLS via Orphanet 70 C0343084

Summaries for Systemic Capillary Leak Syndrome

NIH Rare Diseases : 50 systemic capillary leak syndrome is a condition in which fluid and proteins leak out of tiny blood vessels and flow into surrounding tissues, resulting in dangerously low blood pressure. attacks frequently last for several days and require emergency care. most cases of capillary leak occur randomly in previously healthy adults. treatment involves preventing attacks using medications which may decrease capillary leakage and interfere with hormones that may cause future leakage. once an attack is underway, treatment is aimed at controlling blood pressure to maintain blood flow to vital organs and prevention of swelling due to fluid accumulation. capillary leak syndrome may lead to multiple organ failure, shock and even death. last updated: 9/11/2014

MalaCards based summary : Systemic Capillary Leak Syndrome, also known as capillary leak syndrome, is related to capillary leak syndrome and compartment syndrome. An important gene associated with Systemic Capillary Leak Syndrome is EDNRA (Endothelin Receptor Type A). The drugs Isotretinoin and Aldesleukin have been mentioned in the context of this disorder. Affiliated tissues include endothelial, bone and breast.

Related Diseases for Systemic Capillary Leak Syndrome

Diseases in the Capillary Leak Syndrome family:

Systemic Capillary Leak Syndrome

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

(show all 26)
id Related Disease Score Top Affiliating Genes
1 capillary leak syndrome 11.5
2 compartment syndrome 10.3
3 endotheliitis 10.2
4 influenza 10.0
5 lymphoma 9.9
6 cerebritis 9.9
7 leukemia 9.8
8 c1 inhibitor deficiency 9.8
9 cardiac arrest 9.8
10 colorectal cancer 9.8
11 dermatomyositis 9.8
12 breast cancer 9.8
13 pulmonary edema 9.8
14 renal cell carcinoma 9.8
15 multiple myeloma 9.8
16 aspergillosis 9.8
17 kawasaki disease 9.8
18 peritonitis 9.8
19 adult respiratory distress syndrome 9.8
20 polycythemia 9.8
21 anaplastic large cell lymphoma 9.8
22 b-cell lymphomas 9.8
23 chronic lymphocytic leukemia 9.8
24 alk-negative anaplastic large cell lymphoma 9.8
25 diffuse large b-cell lymphoma 9.8
26 hemolytic anemia 9.8

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



Diseases related to Systemic Capillary Leak Syndrome

Symptoms & Phenotypes for Systemic Capillary Leak Syndrome

Drugs & Therapeutics for Systemic Capillary Leak Syndrome

Drugs for Systemic Capillary Leak Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 58)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Isotretinoin Approved Phase 3,Phase 2 4759-48-2 5538 5282379
2
Aldesleukin Approved Phase 3,Phase 2 85898-30-2, 110942-02-4
3
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
4
Acetaminophen Approved Phase 3 103-90-2 1983
5
Ibuprofen Approved Phase 3 15687-27-1 3672
6
Metoclopramide Approved, Investigational Phase 3 364-62-5 4168
7
Ondansetron Approved Phase 3 99614-02-5 4595
8
Codeine Approved, Illicit Phase 3 76-57-3 5284371
9
Guaifenesin Approved, Vet_approved Phase 3 93-14-1 3516
10
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 11103-57-4, 68-26-8 445354
11
Tretinoin Approved, Investigational, Nutraceutical Phase 3,Phase 2 302-79-4 5538
12 Interleukin-2 Phase 3,Phase 2
13 Analgesics Phase 3,Phase 2
14 Analgesics, Non-Narcotic Phase 3,Phase 2
15 Dermatologic Agents Phase 3,Phase 2
16 Peripheral Nervous System Agents Phase 3,Phase 2
17 Vitamins Phase 3
18 Immunoglobulins Phase 3,Phase 2
19 Anti-HIV Agents Phase 3,Phase 2
20 Anti-Infective Agents Phase 3,Phase 2
21 Antibodies, Monoclonal Phase 3
22 Keratolytic Agents Phase 3,Phase 2
23 Anti-Retroviral Agents Phase 3,Phase 2
24 Retinol palmitate Phase 3
25 Antibodies Phase 3,Phase 2
26 Antiviral Agents Phase 3,Phase 2
27 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
28
Serotonin Phase 3 50-67-9 5202
29 Serotonin Agents Phase 3
30 Serotonin Antagonists Phase 3
31 Narcotics Phase 3
32 Gastrointestinal Agents Phase 3
33 Neurotransmitter Agents Phase 3
34 Cyclooxygenase Inhibitors Phase 3
35 Analgesics, Opioid Phase 3
36 Tranquilizing Agents Phase 3
37 Dopamine Agents Phase 3
38 Dopamine Antagonists Phase 3
39 Dopamine D2 Receptor Antagonists Phase 3
40 Anti-Anxiety Agents Phase 3
41 Antiemetics Phase 3
42 Anti-Inflammatory Agents Phase 3
43 Psychotropic Drugs Phase 3
44 Anti-Inflammatory Agents, Non-Steroidal Phase 3
45 Respiratory System Agents Phase 3
46 Antipsychotic Agents Phase 3
47 Antipyretics Phase 3
48 Antirheumatic Agents Phase 3
49 Autonomic Agents Phase 3
50 Antitussive Agents Phase 3

Interventional clinical trials:


id Name Status NCT ID Phase
1 Monoclonal Antibody Ch14.18, Sargramostim, Aldesleukin, and Isotretinoin After Autologous Stem Cell Transplant in Treating Patients With Neuroblastoma Completed NCT01041638 Phase 3
2 Toxicity Substudy of Evaluation of Subcutaneous Proleukin in a Randomised International Trial (ESPRIT): TOXIL-2 Substudy Terminated NCT00147355 Phase 3
3 NB2013-HR German (GPOH) / Dutch (DCOG) Trial Terminated NCT02641782 Phase 2
4 DT2219ARL for Relapsed or Refractory CD19 (+), CD 22 (+) B-Lineage Leukemia Or Lymphoma Completed NCT00889408 Phase 1
5 Human Leukocyte Antigen (HLA) Class I Haplotype Mismatched Natural Killer Cell Infusions Completed NCT00660166 Phase 1
6 Donor Lymphocyte Infusion (DLI) for Relapsed (Post Transplant) Leukemia Completed NCT00167167
7 Studies in the Pathogenesis of Systemic Capillary Leak Syndrome Recruiting NCT00936325
8 Characterization of Endothelial Dysfunction as a Function of Hyperaemia of the Brachial Artery During Cardiac Surgery Recruiting NCT02853682
9 Evaluation of Endothelial Dysfunction During Aortic Valvular Replacement by Bioprosthesis With and Without Extracorporeal Circulation Recruiting NCT03135496
10 Evaluation of the Association Between Pre-existing Endothelial Dysfunction and the Onset of Vasoplegia During Cardiac Surgery With Cardiopulmonary Bypass Not yet recruiting NCT02965339

Search NIH Clinical Center for Systemic Capillary Leak Syndrome

Genetic Tests for Systemic Capillary Leak Syndrome

Anatomical Context for Systemic Capillary Leak Syndrome

MalaCards organs/tissues related to Systemic Capillary Leak Syndrome:

39
Endothelial, Bone, Breast, B Cells, Kidney, Skeletal Muscle, Colon

Publications for Systemic Capillary Leak Syndrome

Articles related to Systemic Capillary Leak Syndrome:

(show top 50) (show all 134)
id Title Authors Year
1
A Systemic Capillary Leak Syndrome (Clarkson Syndrome) in a Patient with Chronic Lymphocytic Leukemia: A Case Report in an Out-of-Hospital Setting. ( 27069700 )
2016
2
Subcutaneous Immunoglobulin Therapy Prevents Systemic Capillary Leak Syndrome Attack. ( 27059380 )
2016
3
Idiopathic Systemic Capillary Leak Syndrome: A Case Report. ( 27195144 )
2016
4
Sudden cardiac arrest in a 73-year-old woman caused by systemic capillary leak syndrome. ( 27056382 )
2016
5
A Case of Refractory Systemic Capillary Leak Syndrome (Clarkson's Disease) during Pregnancy. ( 28003676 )
2016
6
Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome. ( 27688917 )
2016
7
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome. ( 27622772 )
2016
8
Idiopathic systemic capillary leak syndrome (Clarkson disease). ( 28012935 )
2016
9
Systemic capillary leak syndrome and autoimmune diseases: A case series. ( 27637319 )
2016
10
Isoform D of vascular endothelial growth factor in systemic capillary leak syndrome: a case report. ( 27386947 )
2016
11
Severe cerebral involvement due to idiopathic systemic capillary leak syndrome. ( 27099745 )
2016
12
97: PEDIATRIC SYSTEMIC CAPILLARY LEAK SYNDROME ENDOTHELIAL CELLS ARE HYPERSENSITIVE TO TNF-I9 AND LPS. ( 26569758 )
2015
13
Recurrent undifferentiated shock: Idiopathic Systemic Capillary Leak Syndrome. ( 26273434 )
2015
14
Systemic capillary leak syndrome in a patient receiving adjuvant oxaliplatin for locally advanced colon cancer. ( 26071595 )
2015
15
Endothelial Expression of Endothelin Receptor A in the Systemic Capillary Leak Syndrome. ( 26176954 )
2015
16
Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome. ( 26310790 )
2015
17
Multiglandular hormone deficiency in a patient with systemic capillary leak syndrome. ( 25685157 )
2015
18
1197: ACUTE THERAPY WITH IVIG IN A PEDIATRIC PATIENT WITH IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME. ( 26570858 )
2015
19
1222: SUCCESSFUL MANAGEMENT OF RECURRENT IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME IN A 4-YEAR OLD GIRL. ( 26570883 )
2015
20
Clinical and pathological findings of a fatal systemic capillary leak syndrome (Clarkson disease): a case report. ( 25738482 )
2015
21
Influenza A virus infection is associated with systemic capillary leak syndrome: case report and systematic review of the literature. ( 26330157 )
2015
22
Spinal Anesthesia in a Patient With a History of Systemic Capillary Leak Syndrome (Clarkson Disease). ( 26237002 )
2015
23
A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report. ( 25928289 )
2015
24
Systemic Capillary Leak Syndrome after Influenza Vaccination in a Peritoneal Dialysis Patient. ( 26703855 )
2015
25
An uncommon cause of distributive shock: Lessons from two consecutive cases of idiopathic systemic capillary leak syndrome (Clarkson's disease). ( 26300309 )
2015
26
Idiopathic systemic capillary leak syndrome in children. ( 25713284 )
2015
27
A case report of idiopathic systemic capillary leak syndrome that occurred during the postoperative period of abdominoperineal resection for colorectal cancer. ( 25594640 )
2015
28
Correction: Endothelial Expression of Endothelin Receptor A in the Systemic Capillary Leak Syndrome. ( 26325591 )
2015
29
Systemic capillary leak syndrome: a case-report. ( 26117513 )
2015
30
Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature. ( 24490827 )
2014
31
Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report. ( 24467750 )
2014
32
Systemic capillary leak syndrome revealing a diffuse large B-cell lymphoma. ( 24942976 )
2014
33
Invasive pulmonary aspergillosis in a patient presenting with idiopathic systemic capillary leak syndrome. ( 24859554 )
2014
34
Systemic capillary leak syndrome under general anesthesia: a case report. ( 25006371 )
2014
35
Systemic capillary leak syndrome associated with a rare abdominal and four-limb compartment syndrome: a case report. ( 24934689 )
2014
36
High dose intravenous immunoglobulin therapy of the Systemic Capillary Leak Syndrome (Clarkson disease). ( 25193271 )
2014
37
10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report. ( 24885961 )
2014
38
Two Episodes of Systemic Capillary Leak Syndrome in an 8-year-old Boy, Following Influenza A Virus Infection. ( 23995592 )
2014
39
Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). ( 25405070 )
2014
40
Systemic capillary leak syndrome: is methylene blue the silver bullet? ( 25544902 )
2014
41
Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease. ( 24787070 )
2014
42
Systemic capillary leak syndrome in an 85-year-old man (Clarkson's syndrome). ( 25287734 )
2014
43
Clarkson disease - systemic capillary leak syndrome in a 6-year-old girl: case report. ( 25311880 )
2014
44
Malignant hemispheric cerebral infarction associated with idiopathic systemic capillary leak syndrome. ( 24163674 )
2013
45
Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression. ( 23576661 )
2013
46
Genome-wide SNP analysis of the Systemic Capillary Leak Syndrome (Clarkson disease). ( 24808988 )
2013
47
Systemic capillary leak syndrome: recognition prevents morbidity and mortality. ( 24134172 )
2013
48
Episodic idiopathic systemic capillary leak syndrome in a girl. ( 23910812 )
2013
49
Trastuzumab-Induced Systemic Capillary Leak Syndrome in a Breast Cancer Patient. ( 24163290 )
2013
50
Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma. ( 22953081 )
2012

Variations for Systemic Capillary Leak Syndrome

Expression for Systemic Capillary Leak Syndrome

Search GEO for disease gene expression data for Systemic Capillary Leak Syndrome.

Pathways for Systemic Capillary Leak Syndrome

GO Terms for Systemic Capillary Leak Syndrome

Sources for Systemic Capillary Leak Syndrome

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60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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