SCLS
MCID: SYS007
MIFTS: 37

Systemic Capillary Leak Syndrome (SCLS) malady

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

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Sources:
43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 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: 8/30/2012

MalaCards: Systemic Capillary Leak Syndrome, also known as capillary leak syndrome, is related to capillary leak syndrome and compartment syndrome, and has symptoms including cardiac rhythm disorder/arrhythmia, collapse/sudden death/cardiac arrest/cardiorespiratory arrest and venous thrombosis/phlebitis/thrombophlebitis. An important gene associated with Systemic Capillary Leak Syndrome is ALB (albumin). Affiliated tissues include bone, endothelial and breast.

Aliases & Classifications for Systemic Capillary Leak Syndrome

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43NIH Rare Diseases, 49Orphanet, 62UMLS, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

49
capillary leak syndrome:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Adulthood; Age of death: Adult


Aliases & Descriptions:

systemic capillary leak syndrome 43 49
capillary leak syndrome 43 49 62
capillary leak syndrome with monoclonal gammopathy 43 62
clarkson disease 43 49
scls 43 49
periodic systemic capillary leak syndrome 43
capillary hyperpermeability syndrome 49
idiopathic capillary leak syndrome 49


External Ids:

MESH via Orphanet36 D019559
ICD10 via Orphanet26 I78.8
SNOMED-CT via Orphanet59 87730004
UMLS via Orphanet63 C0343084

Related Diseases for Systemic Capillary Leak Syndrome

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Systemic Capillary Leak Syndrome:



Diseases related to systemic capillary leak syndrome

Symptoms for Systemic Capillary Leak Syndrome

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49Orphanet
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Symptoms:

49 (show all 26)
  • cardiac rhythm disorder/arrhythmia
  • collapse/sudden death/cardiac arrest/cardiorespiratory arrest
  • venous thrombosis/phlebitis/thrombophlebitis
  • renal tubular defect/tubulopathy
  • renal failure
  • pollakiuria/polyuria/dysuria/anuria/acute urine retention/oliguria
  • seizures/epilepsy/absences/spasms/status epilepticus
  • multiple myeloma
  • fever/chilling
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • myocardium anomalies/myocarditis
  • hyperleukocytosis/leukocytosis
  • nasal congestion/sinusitis/rhinitis/rhinorrhea
  • acute abdominal pain/colic
  • acute diarrhea
  • pancreatitis
  • pulmonary edema
  • hypotension
  • myalgia/muscular pain
  • monoclonal immunoglobulins/gammapathy/dysglobulinemia
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • asthenia/fatigue/weakness
  • hot flushes/sensation of cold
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • cough
  • edema of the legs/lower limbs

Drugs & Therapeutics for Systemic Capillary Leak Syndrome

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Sources:
42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

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Genetic Tests for Systemic Capillary Leak Syndrome

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Anatomical Context for Systemic Capillary Leak Syndrome

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33MalaCards
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MalaCards organs/tissues related to Systemic Capillary Leak Syndrome:

33
Bone, Endothelial, Breast, Lung, Kidney, Skeletal muscle

Animal Models for Systemic Capillary Leak Syndrome or affiliated genes

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Publications for Systemic Capillary Leak Syndrome

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52PubMed
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Articles related to Systemic Capillary Leak Syndrome:

(show top 50)    (show all 91)
idTitleAuthorsYear
1
Two Episodes of Systemic Capillary Leak Syndrome in an 8-year-old Boy, Following Influenza A Virus Infection. (23995592)
2014
2
Episodic idiopathic systemic capillary leak syndrome in a girl. (23910812)
2013
3
Systemic capillary leak syndrome: recognition prevents morbidity and mortality. (24134172)
2013
4
Trastuzumab-Induced Systemic Capillary Leak Syndrome in a Breast Cancer Patient. (24163290)
2013
5
Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma. (22953081)
2012
6
The systemic capillary leak syndrome: a scarcely known nephrological entity. (22135035)
2012
7
Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). (22411873)
2012
8
Myocardial involvement in systemic capillary leak syndrome: first demonstration by pathologic findings. (24062914)
2012
9
The systemic capillary leak syndrome: a case series of 28 patients from a European registry. (21464348)
2011
10
Idiopathic systemic capillary leak syndrome preceding diagnosis of infiltrating lobular carcinoma of the breast with quiescence during neoadjuvant chemotherapy. (21364118)
2011
11
A case of idiopathic systemic capillary leak syndrome with high serum levels of G-CSF on exacerbation. (21422685)
2011
12
Systemic capillary leak syndrome caused by granulocyte colony-stimulating factor. (21963759)
2011
13
High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome. (22696704)
2011
14
Neurologic involvement in a child with systemic capillary leak syndrome. (20123768)
2010
15
Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience. (20634497)
2010
16
A case report of systemic capillary leak syndrome (Clarkson's disease). (20148770)
2010
17
The systemic capillary leak syndrome. (21135304)
2010
18
Four-limb compartment syndrome associated with the systemic capillary leak syndrome. (21119178)
2010
19
Immunoglobulins for treatment of systemic capillary leak syndrome. (20569743)
2010
20
Systemic capillary leak syndrome or Clarkson's disease: a case report. (19526356)
2009
21
Chronic systemic capillary leak syndrome: a case responding to prednisolone treatment. (20166237)
2009
22
Vascular endothelial growth factor in systemic capillary leak syndrome. (19486705)
2009
23
Idiopathic systemic capillary leak syndrome: novel therapy for acute attacks. (19703708)
2009
24
High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome. (18552679)
2008
25
Systemic capillary leak syndrome associated with compartment syndrome. (17878647)
2007
26
Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis. (16802499)
2006
27
Systemic capillary leak syndrome presenting as recurrent shock. (16738498)
2006
28
Idiopathic systemic capillary leak syndrome: cutaneous involvement can be misleading. (15539891)
2004
29
Systemic capillary leak syndrome after granulocyte colony-stimulating factor (G-CSF). (12692521)
2003
30
An unusual evolution of the systemic capillary leak syndrome. (11865098)
2002
31
Systemic capillary leak syndrome. (12487153)
2002
32
Systemic capillary leak syndrome. (11929183)
2002
33
Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome. (11591575)
2001
34
Systemic capillary leak syndrome preceding plasma cell leukaemia. (11713377)
2001
35
Systemic capillary leak syndrome]. (10682651)
2000
36
Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. A case series. (10375339)
1999
37
Systemic capillary leak syndrome: where does the fluid go? (9799931)
1998
38
Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment. (9428835)
1997
39
A case of rapidly fatal systemic capillary leak syndrome in a kidney transplant recipient. (9489137)
1997
40
Systemic capillary leak syndrome in a child. (7472826)
1995
41
Fulminating systemic capillary leak syndrome with lymphocytosis and hypogammaglobulinemia. (8570875)
1995
42
Systemic capillary leak syndrome. (8222853)
1993
43
Control of systemic capillary leak syndrome with aminophylline and terbutaline. (1580299)
1992
44
Muscle biopsy findings in systemic capillary leak syndrome. (2179110)
1990
45
Systemic capillary leak syndrome. (2563486)
1989
46
Treatment of systemic capillary leak syndrome. (2904614)
1988
47
Anterior tibial compartment syndrome secondary to systemic capillary leak syndrome. (3625226)
1987
48
Ultrastructure of the microvessels in skeletal muscle in a case of systemic capillary leak syndrome. (525443)
1979
49
Systemic capillary leak syndrome. Evaluation of single protein fractions in serum and in interstitial fluid. (496875)
1979
50
Systemic capillary leak syndrome and monoclonal IgG gammopathy; studies in a sixth patient and a review of the literature. (870792)
1977

Variations for Systemic Capillary Leak Syndrome

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Expression for genes affiliated with Systemic Capillary Leak Syndrome

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Systemic Capillary Leak Syndrome

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Pathways for genes affiliated with Systemic Capillary Leak Syndrome

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Compounds for genes affiliated with Systemic Capillary Leak Syndrome

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GO Terms for genes affiliated with Systemic Capillary Leak Syndrome

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Products for genes affiliated with Systemic Capillary Leak Syndrome

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  • Antibodies
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  • Antibodies

Sources for Systemic Capillary Leak Syndrome

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet