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PedAM

Pediatric Disease Annotations & Medicines



   capillary leak syndrome
  

Disease ID 701
Disease capillary leak syndrome
Definition
A condition characterized by recurring episodes of fluid leaking from capillaries into extra-vascular compartments causing hematocrit to rise precipitously. If not treated, generalized vascular leak can lead to generalized EDEMA; SHOCK; cardiovascular collapse; and MULTIPLE ORGAN FAILURE.
Synonym
acute vascular leak syndrome
avls
capillary leak syndrome (disorder)
capillary leak syndrome [disease/finding]
capillary leak syndromes
capillary leaking syndrome
clarkson disease
systemic capillary leak syndrome
Orphanet
DOID
UMLS
C0343084
MeSH
SNOMED-CT
Comorbidity
UMLS | Disease | Sentences' Count(Total Sentences:14)
C0021400  |  influenza  |  2
C0034063  |  pulmonary edema  |  2
C0006309  |  brucellosis  |  1
C0024302  |  large cell lymphoma  |  1
C0007785  |  cerebral infarction  |  1
C0009492  |  compartment syndrome  |  1
C0035078  |  renal failure  |  1
C0019100  |  dengue hemorrhagic fever  |  1
C0026691  |  kawasaki disease  |  1
C0376545  |  hematological malignancies  |  1
C0022660  |  acute renal failure  |  1
C0007785  |  cerebral infarct  |  1
C0206180  |  anaplastic large cell lymphoma  |  1
C0042769  |  virus infection  |  1
Curated Gene(Waiting for update.)
Inferring Gene(Waiting for update.)
Text Mined Gene
Entrez_id | Symbol | Score | Resource(Total Genes:58)
3385  |  ICAM3  |  DISEASES
3560  |  IL2RB  |  DISEASES
3002  |  GZMB  |  DISEASES
51804  |  SIX4  |  DISEASES
1440  |  CSF3  |  DISEASES
3558  |  IL2  |  DISEASES
3458  |  IFNG  |  DISEASES
5341  |  PLEK  |  DISEASES
6402  |  SELL  |  DISEASES
4695  |  NDUFA2  |  DISEASES
1131  |  CHRM3  |  DISEASES
3569  |  IL6  |  DISEASES
26525  |  IL36RN  |  DISEASES
9172  |  MYOM2  |  DISEASES
4591  |  TRIM37  |  DISEASES
3553  |  IL1B  |  DISEASES
1991  |  ELANE  |  DISEASES
3589  |  IL11  |  DISEASES
3383  |  ICAM1  |  DISEASES
3827  |  KNG1  |  DISEASES
6717  |  SRI  |  DISEASES
51608  |  GET4  |  DISEASES
3439  |  IFNA1  |  DISEASES
710  |  SERPING1  |  DISEASES
715  |  C1R  |  DISEASES
2215  |  FCGR3B  |  DISEASES
213  |  ALB  |  DISEASES
1437  |  CSF2  |  DISEASES
26585  |  GREM1  |  DISEASES
1632  |  ECI1  |  DISEASES
3627  |  CXCL10  |  DISEASES
27289  |  RND1  |  DISEASES
358  |  AQP1  |  DISEASES
2629  |  GBA  |  DISEASES
285  |  ANGPT2  |  DISEASES
4684  |  NCAM1  |  DISEASES
716  |  C1S  |  DISEASES
23762  |  OSBP2  |  DISEASES
7171  |  TPM4  |  DISEASES
6238  |  RRBP1  |  DISEASES
1378  |  CR1  |  DISEASES
55811  |  ADCY10  |  DISEASES
2214  |  FCGR3A  |  DISEASES
11093  |  ADAMTS13  |  DISEASES
7422  |  VEGFA  |  DISEASES
4038  |  LRP4  |  DISEASES
3440  |  IFNA2  |  DISEASES
3456  |  IFNB1  |  DISEASES
833  |  CARS  |  DISEASES
238  |  ALK  |  DISEASES
2524  |  FUT2  |  DISEASES
10622  |  POLR3G  |  DISEASES
720  |  C4A  |  DISEASES
7124  |  TNF  |  DISEASES
3586  |  IL10  |  DISEASES
721  |  C4B  |  DISEASES
930  |  CD19  |  DISEASES
56963  |  RGMA  |  DISEASES
Locus(Waiting for update.)
Disease ID 701
Disease capillary leak syndrome
Integrated Phenotype(Waiting for update.)
Text Mined Phenotype
HPO | Name | Sentences' Count(Total Phenotypes:8)
HP:0002615  |  Low blood pressure  |  4
HP:0000969  |  Dropsy  |  3
HP:0100598  |  Pulmonary oedema  |  2
HP:0000083  |  Renal insufficiency  |  1
HP:0001919  |  Acute renal failure  |  1
HP:0002202  |  Pleural effusion  |  1
HP:0001945  |  Fever  |  1
HP:0012398  |  Peripheral edema  |  1
Disease ID 701
Disease capillary leak syndrome
Manually Symptom
UMLS  | Name(Total Manually Symptoms:3)
C1963101  |  encephalopathy
C1142110  |  abdominal compartment syndrome
C0948600  |  organ failure
Text Mined Symptom(Waiting for update.)
Manually Genotype(Total Text Mining Genotypes:0)
(Waiting for update.)
All Snps(Total Genotypes:0)
(Waiting for update.)
GWASdb Annotation(Total Genotypes:0)
(Waiting for update.)
GWASdb Snp Trait(Total Genotypes:0)
(Waiting for update.)
Mapped by lexical matching(Total Items:0)
(Waiting for update.)
Mapped by homologous gene(Total Items:0)
(Waiting for update.)
Chemical(Total Drugs:8)
CUI ChemicalName ChemicalID CasRN DiseaseName DiseaseID DirectEvidence PubMedIDs
C0343084acitretinD01725555079-83-9capillary leak syndromeMESH:D019559marker/mechanism14746632
C0343084aldesleukinC082598110942-02-4capillary leak syndromeMESH:D019559marker/mechanism11219485
C0343084bortezomibD000069286-capillary leak syndromeMESH:D019559marker/mechanism20150504
C0343084clofarabineC068329123318-82-1capillary leak syndromeMESH:D019559marker/mechanism20057324
C0343084cisplatinD00294515663-27-1capillary leak syndromeMESH:D019559marker/mechanism11822767
C0343084gemcitabineC056507103882-84-4capillary leak syndromeMESH:D019559marker/mechanism11822767
C0343084piroxicamD01089436322-90-4capillary leak syndromeMESH:D019559marker/mechanism14682198
C0343084sirolimusD02012353123-88-9capillary leak syndromeMESH:D019559marker/mechanism10328196
FDA approved drug and dosage information(Total Drugs:4)
DiseaseID Drug_name active_ingredients strength Dosage Form/Route Marketing Status TE code RLD RS
MESH:D019559clolarclofarabine20MG/20ML (1MG/ML)INJECTABLE;IV (INFUSION)PrescriptionAPYesYes
MESH:D019559rapamunesirolimus1MG/MLSOLUTION;ORALPrescriptionNoneYesYes
MESH:D019559rapamunesirolimus1MGTABLET;ORALPrescriptionABYesNo
MESH:D019559velcadebortezomib3.5MG/VIALINJECTABLE;INTRAVENOUS, SUBCUTANEOUSPrescriptionNoneYesYes
FDA labeling changes(Total Drugs:4)
DiseaseID Pediatric_Labeling_Date Trade_Name Generic_Name_or_Proper_Name Indications Studied Label Changes Summary Product Labeling BPCA(B) PREA(P) BPCA(B) and PREA(P) Pediatric Rule (R) Sponsor Pediatric Exclusivity Granted Date NNPS
MESH:D01955912/28/2004clolarclofarabineRelapsed or refractory acute lymphoblastic leukemia after at least two prior regimensLabeling for patients 1 to 21 years old. This use is based on the induction of complete responses Randomized trials demonstrating increased survival or other clinical benefit have not been conducted Information on dose, PK parameters, and AE profiheLabelingB---Genzyme07/14/2004FALSE'
MESH:D01955911/3/2005rapamunesirolimusProphylaxis of organ rejection in patients undergoing renal transplantsSafety and efficacy established in children 13 years or older judged to be at low to moderate immunologic risk Safety was assessed in a controlled clinical trial in pediatric (LabelingB---Wyeth11/17/2004FALSE'
MESH:D01955911/3/2005rapamunesirolimusProphylaxis of organ rejection in patients undergoing renal transplantsSafety and efficacy established in children 13 years or older judged to be at low to moderate immunologic risk Safety was assessed in a controlled clinical trial in pediatric (LabelingB---Wyeth11/17/2004FALSE'
MESH:D01955909/14/2015velcadebortezomibRelapsed Acute Lymphoblastic Leukemia (ALL) and Lymphoblastic Lymphoma (LL)Effectiveness in pediatric patients with relapsed pre-B ALL has not been established. The activity and safety of Velcade in combination with intensive reinduction chemotherapy was evaluated in pediatric and young adult patients with lymphoid malignancies. There were 140 patients with ALL or LL enrolled and evaluated for safety. No new safety concerns were observedLabelingB---Millennium Pharmaceuticals, Inc.-FALSE