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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NIH Rare Diseases:42 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, and has symptoms including edema of the legs/lower limbs, hyperleukocytosis/leukocytosis and nasal congestion/sinusitis/rhinitis/rhinorrhea. 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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Sources:
42NIH Rare Diseases, 48Orphanet, 62UMLS, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Systemic Capillary Leak Syndrome, Aliases & Descriptions:

Name: Systemic Capillary Leak Syndrome 42 48 62
Capillary Leak Syndrome 42 48 62
Capillary Leak Syndrome with Monoclonal Gammopathy 42 62
Clarkson Disease 42 48
 
Scls 42 48
Periodic Systemic Capillary Leak Syndrome 42
Capillary Hyperpermeability Syndrome 48
Idiopathic Capillary Leak Syndrome 48


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
systemic capillary leak syndrome:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Adulthood; Age of death: Adult


External Ids:

MESH via Orphanet35 D019559
ICD10 via Orphanet26 I78.8
UMLS via Orphanet63 C0343084

Related Diseases for Systemic Capillary Leak Syndrome

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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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Symptoms:

48 (show all 26)
  • edema of the legs/lower limbs
  • 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
  • myocardium anomalies/myocarditis
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • 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

HPO human phenotypes related to Systemic Capillary Leak Syndrome:

(show all 22)
id Description Frequency HPO Source Accession
1 leukocytosis hallmark (90%) HP:0001974
2 edema of the lower limbs hallmark (90%) HP:0010741
3 sinusitis typical (50%) HP:0000246
4 pancreatitis typical (50%) HP:0001733
5 weight loss typical (50%) HP:0001824
6 diarrhea typical (50%) HP:0002014
7 abdominal pain typical (50%) HP:0002027
8 hypotension typical (50%) HP:0002615
9 myalgia typical (50%) HP:0003326
10 abnormal immunoglobulin level typical (50%) HP:0010701
11 impaired temperature sensation typical (50%) HP:0010829
12 pulmonary edema typical (50%) HP:0100598
13 renal insufficiency occasional (7.5%) HP:0000083
14 abnormality of the renal tubule occasional (7.5%) HP:0000091
15 seizures occasional (7.5%) HP:0001250
16 abnormality of the myocardium occasional (7.5%) HP:0001637
17 sudden cardiac death occasional (7.5%) HP:0001645
18 abnormality of the pericardium occasional (7.5%) HP:0001697
19 abnormality of the pleura occasional (7.5%) HP:0002103
20 abnormality of temperature regulation occasional (7.5%) HP:0004370
21 thrombophlebitis occasional (7.5%) HP:0004418
22 multiple myeloma occasional (7.5%) HP:0006775

Drugs & Therapeutics for Systemic Capillary Leak Syndrome

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Drug clinical trials:

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Search NIH Clinical Center for Systemic Capillary Leak Syndrome

Genetic Tests for Systemic Capillary Leak Syndrome

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Anatomical Context for Systemic Capillary Leak Syndrome

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MalaCards organs/tissues related to Systemic Capillary Leak Syndrome:

32
Bone, Endothelial, Breast, Skeletal muscle, Kidney, Lung, B cells

Animal Models for Systemic Capillary Leak Syndrome or affiliated genes

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Publications for Systemic Capillary Leak Syndrome

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Articles related to Systemic Capillary Leak Syndrome:

(show top 50)    (show all 103)
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
Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report. (24467750)
2014
7
Two Episodes of Systemic Capillary Leak Syndrome in an 8-year-old Boy, Following Influenza A Virus Infection. (23995592)
2014
8
Genome-wide SNP analysis of the Systemic Capillary Leak Syndrome (Clarkson disease). (24808988)
2013
9
Episodic idiopathic systemic capillary leak syndrome in a girl. (23910812)
2013
10
Systemic capillary leak syndrome: recognition prevents morbidity and mortality. (24134172)
2013
11
Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma. (22953081)
2012
12
The systemic capillary leak syndrome: a scarcely known nephrological entity. (22135035)
2012
13
Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). (22411873)
2012
14
The systemic capillary leak syndrome: a case series of 28 patients from a European registry. (21464348)
2011
15
Idiopathic systemic capillary leak syndrome preceding diagnosis of infiltrating lobular carcinoma of the breast with quiescence during neoadjuvant chemotherapy. (21364118)
2011
16
A case of idiopathic systemic capillary leak syndrome with high serum levels of G-CSF on exacerbation. (21422685)
2011
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
The systemic capillary leak syndrome. (21135304)
2010
21
Four-limb compartment syndrome associated with the systemic capillary leak syndrome. (21119178)
2010
22
Immunoglobulins for treatment of systemic capillary leak syndrome. (20569743)
2010
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
Vascular endothelial growth factor in systemic capillary leak syndrome. (19486705)
2009
26
Idiopathic systemic capillary leak syndrome: novel therapy for acute attacks. (19703708)
2009
27
High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome. (18552679)
2008
28
Systemic capillary leak syndrome associated with compartment syndrome. (17878647)
2007
29
Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis. (16802499)
2006
30
Systemic capillary leak syndrome presenting as recurrent shock. (16738498)
2006
31
Systemic capillary leak syndrome after granulocyte colony-stimulating factor (G-CSF). (12692521)
2003
32
An unusual evolution of the systemic capillary leak syndrome. (11865098)
2002
33
Systemic capillary leak syndrome. (12487153)
2002
34
Systemic capillary leak syndrome. (11929183)
2002
35
Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome. (11591575)
2001
36
Systemic capillary leak syndrome preceding plasma cell leukaemia. (11713377)
2001
37
Systemic capillary leak syndrome]. (10682651)
2000
38
Systemic capillary leak syndrome: where does the fluid go? (9799931)
1998
39
Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment. (9428835)
1997
40
A case of rapidly fatal systemic capillary leak syndrome in a kidney transplant recipient. (9489137)
1997
41
Systemic capillary leak syndrome in a child. (7472826)
1995
42
Fulminating systemic capillary leak syndrome with lymphocytosis and hypogammaglobulinemia. (8570875)
1995
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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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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Sources for Systemic 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