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Capillary leak syndrome

MedGen UID:
137987
Concept ID:
C0343084
Disease or Syndrome
Synonyms: Capillary Leak Syndrome; Capillary Leak Syndromes; Clarkson Disease; Systemic Capillary Leak Syndrome
SNOMED CT: Capillary leak syndrome (87730004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0001956
Orphanet: ORPHA188

Definition

Systemic capillary leak syndrome (SCLS) is a severe systemic disease due to increased capillary permeability, characterized by episodes of hypotension, edema and hypovolemia. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Capillary leak syndrome
Follow this link to review classifications for Capillary leak syndrome in Orphanet.

Professional guidelines

PubMed

Yang L, Xie X, Tu Z, Fu J, Xu D, Zhou Y
Signal Transduct Target Ther 2021 Jul 7;6(1):255. doi: 10.1038/s41392-021-00679-0. PMID: 34234112Free PMC Article
Baloch NU, Bikak M, Rehman A, Rahman O
Expert Rev Cardiovasc Ther 2018 May;16(5):331-340. Epub 2018 Apr 2 doi: 10.1080/14779072.2018.1456920. PMID: 29564922
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633

Recent clinical studies

Etiology

Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, Rosenblat TL, Brooks CL, Habboubi N, Mughal TI, Kantarjian H, Konopleva M, Lane AA
J Clin Oncol 2022 Sep 10;40(26):3032-3036. Epub 2022 Jul 12 doi: 10.1200/JCO.22.00034. PMID: 35820082Free PMC Article
Ladenstein R, Pötschger U, Valteau-Couanet D, Luksch R, Castel V, Yaniv I, Laureys G, Brock P, Michon JM, Owens C, Trahair T, Chan GCF, Ruud E, Schroeder H, Beck Popovic M, Schreier G, Loibner H, Ambros P, Holmes K, Castellani MR, Gaze MN, Garaventa A, Pearson ADJ, Lode HN
Lancet Oncol 2018 Dec;19(12):1617-1629. Epub 2018 Nov 12 doi: 10.1016/S1470-2045(18)30578-3. PMID: 30442501
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Cornell RF, Hari P, Drobyski WR
Biol Blood Marrow Transplant 2015 Dec;21(12):2061-2068. Epub 2015 Aug 29 doi: 10.1016/j.bbmt.2015.08.030. PMID: 26327628Free PMC Article
Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, Smith M, Anderson B, Villablanca JG, Matthay KK, Shimada H, Grupp SA, Seeger R, Reynolds CP, Buxton A, Reisfeld RA, Gillies SD, Cohn SL, Maris JM, Sondel PM; Children's Oncology Group
N Engl J Med 2010 Sep 30;363(14):1324-34. doi: 10.1056/NEJMoa0911123. PMID: 20879881Free PMC Article

Diagnosis

Mudhol RR, Bhise R
J Assoc Physicians India 2022 Oct;70(10):11-12. doi: 10.5005/japi-11001-0106. PMID: 37355874
Bichon A, Bourenne J, Gainnier M, Carvelli J
Rev Med Interne 2021 Nov;42(11):789-796. Epub 2021 Jun 4 doi: 10.1016/j.revmed.2021.05.012. PMID: 34099313
De Feo G, Parente R, Triggiani M
Curr Opin Allergy Clin Immunol 2018 Oct;18(5):382-386. doi: 10.1097/ACI.0000000000000468. PMID: 30028728
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Druey KM, Parikh SM
J Allergy Clin Immunol 2017 Sep;140(3):663-670. Epub 2016 Dec 22 doi: 10.1016/j.jaci.2016.10.042. PMID: 28012935Free PMC Article

Therapy

Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, Rosenblat TL, Brooks CL, Habboubi N, Mughal TI, Kantarjian H, Konopleva M, Lane AA
J Clin Oncol 2022 Sep 10;40(26):3032-3036. Epub 2022 Jul 12 doi: 10.1200/JCO.22.00034. PMID: 35820082Free PMC Article
Freyer CW, Porter DL
J Allergy Clin Immunol 2020 Nov;146(5):940-948. Epub 2020 Aug 6 doi: 10.1016/j.jaci.2020.07.025. PMID: 32771558
Pemmaraju N, Lane AA, Sweet KL, Stein AS, Vasu S, Blum W, Rizzieri DA, Wang ES, Duvic M, Sloan JM, Spence S, Shemesh S, Brooks CL, Balser J, Bergstein I, Lancet JE, Kantarjian HM, Konopleva M
N Engl J Med 2019 Apr 25;380(17):1628-1637. doi: 10.1056/NEJMoa1815105. PMID: 31018069
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, Smith M, Anderson B, Villablanca JG, Matthay KK, Shimada H, Grupp SA, Seeger R, Reynolds CP, Buxton A, Reisfeld RA, Gillies SD, Cohn SL, Maris JM, Sondel PM; Children's Oncology Group
N Engl J Med 2010 Sep 30;363(14):1324-34. doi: 10.1056/NEJMoa0911123. PMID: 20879881Free PMC Article

Prognosis

Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, Rosenblat TL, Brooks CL, Habboubi N, Mughal TI, Kantarjian H, Konopleva M, Lane AA
J Clin Oncol 2022 Sep 10;40(26):3032-3036. Epub 2022 Jul 12 doi: 10.1200/JCO.22.00034. PMID: 35820082Free PMC Article
Yang L, Xie X, Tu Z, Fu J, Xu D, Zhou Y
Signal Transduct Target Ther 2021 Jul 7;6(1):255. doi: 10.1038/s41392-021-00679-0. PMID: 34234112Free PMC Article
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Druey KM, Parikh SM
J Allergy Clin Immunol 2017 Sep;140(3):663-670. Epub 2016 Dec 22 doi: 10.1016/j.jaci.2016.10.042. PMID: 28012935Free PMC Article
Rothe MJ, Bialy TL, Grant-Kels JM
Dermatol Clin 2000 Jul;18(3):405-15. doi: 10.1016/s0733-8635(05)70189-3. PMID: 10943536

Clinical prediction guides

Jordan SC, Ammerman N, Huang E, Vo A
Am J Transplant 2022 Dec;22 Suppl 4:28-37. doi: 10.1111/ajt.17207. PMID: 36453709
Pemmaraju N, Sweet KL, Stein AS, Wang ES, Rizzieri DA, Vasu S, Rosenblat TL, Brooks CL, Habboubi N, Mughal TI, Kantarjian H, Konopleva M, Lane AA
J Clin Oncol 2022 Sep 10;40(26):3032-3036. Epub 2022 Jul 12 doi: 10.1200/JCO.22.00034. PMID: 35820082Free PMC Article
Siddall E, Khatri M, Radhakrishnan J
Kidney Int 2017 Jul;92(1):37-46. Epub 2017 Mar 17 doi: 10.1016/j.kint.2016.11.029. PMID: 28318633
Yu AL, Gilman AL, Ozkaynak MF, London WB, Kreissman SG, Chen HX, Smith M, Anderson B, Villablanca JG, Matthay KK, Shimada H, Grupp SA, Seeger R, Reynolds CP, Buxton A, Reisfeld RA, Gillies SD, Cohn SL, Maris JM, Sondel PM; Children's Oncology Group
N Engl J Med 2010 Sep 30;363(14):1324-34. doi: 10.1056/NEJMoa0911123. PMID: 20879881Free PMC Article
Escutenaire S, Pastoret PP
Rev Sci Tech 2000 Apr;19(1):64-78. doi: 10.20506/rst.19.1.1209. PMID: 11189727

Recent systematic reviews

Wong So J, Bouibede F, Jonville-Béra AP, Maillot F, Barbier F, Largeau B
J Intern Med 2023 Jul;294(1):58-68. Epub 2023 Apr 20 doi: 10.1111/joim.13641. PMID: 37038359
Orlando C, Milani GP, Simonetti GD, Goeggel Simonetti B, Lava SAG, Wyttenbach R, Bianchetti MG, Cristallo Lacalamita M
Pediatr Nephrol 2022 Apr;37(4):833-841. Epub 2021 Sep 21 doi: 10.1007/s00467-021-05244-z. PMID: 34546419Free PMC Article
Bozzini MA, Milani GP, Bianchetti MG, Fossali EF, Lava SAG
Eur J Pediatr 2018 Aug;177(8):1149-1154. Epub 2018 Jun 23 doi: 10.1007/s00431-018-3189-8. PMID: 29936591
Eo TS, Chun KJ, Hong SJ, Kim JY, Lee IR, Lee KH, Eisenhut M, Kronbichler A, Shin JI
J Allergy Clin Immunol Pract 2018 Mar-Apr;6(2):609-618. Epub 2017 Sep 19 doi: 10.1016/j.jaip.2017.07.021. PMID: 28939140
Rajagopala S, Thabah MM, Ariga KK, Gopalakrishnan M
QJM 2015 Sep;108(9):719-28. Epub 2015 Jan 27 doi: 10.1093/qjmed/hcv011. PMID: 25630907

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