SCLS
MCID: SYS007
MIFTS: 25

Systemic Capillary Leak Syndrome (SCLS) malady

Bone diseases category

Summaries for Systemic Capillary Leak Syndrome

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42NIH Rare Diseases, 32MalaCards
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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: 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 myocardium anomalies/myocarditis, pericardium anomalies/pericarditis/absence/cysts/pericardial effusion and cardiac rhythm disorder/arrhythmia. An important gene associated with Systemic Capillary Leak Syndrome is ALB (albumin). Affiliated tissues include bone.

Aliases & Classifications for Systemic Capillary Leak Syndrome

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Sources:
42NIH Rare Diseases, 48Orphanet, 60UMLS, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Bone diseases


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

systemic capillary leak syndrome 42 48
capillary leak syndrome 42 48 60
capillary leak syndrome with monoclonal gammopathy 42 60
clarkson disease 42 48
scls 42 48
periodic systemic capillary leak syndrome 42
capillary hyperpermeability syndrome 48
idiopathic capillary leak syndrome 48


External Ids:

MESH via Orphanet35 D019559
ICD10 via Orphanet26 I78.8
SNOMED-CT via Orphanet57 87730004
UMLS via Orphanet61 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

Clinical Features for Systemic Capillary Leak Syndrome

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48Orphanet
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Symptoms:

48 (show all 26)
  • 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
  • cough
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • hot flushes/sensation of cold
  • 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
  • edema of the legs/lower limbs

Drugs & Therapeutics for Systemic Capillary Leak Syndrome

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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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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Sources:
32MalaCards
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MalaCards organs/tissues related to Systemic Capillary Leak Syndrome:

32
Bone

Animal Models for Systemic Capillary Leak Syndrome or affiliated genes

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Publications for Systemic Capillary Leak Syndrome

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Genetic Variations for Systemic Capillary Leak Syndrome

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Expression for genes affiliated with Systemic Capillary Leak Syndrome

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Systemic Capillary Leak Syndrome

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

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
  • Proteins
  • Lysates
  • Antibodies

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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet