Titre
Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.
Type
article
Institution
Externe
Périodique
Auteur(s)
Bozzini, M.A.
Auteure/Auteur
Milani, G.P.
Auteure/Auteur
Bianchetti, M.G.
Auteure/Auteur
Fossali, E.F.
Auteure/Auteur
Lava, SAG
Auteure/Auteur
Liens vers les personnes
ISSN
1432-1076
Statut éditorial
Publié
Date de publication
2018-08
Volume
177
Numéro
8
Première page
1149
Dernière page/numéro d’article
1154
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Systematic Review
Publication Status: ppublish
Publication Status: ppublish
Résumé
Approximately 500 cases of idiopathic systemic capillary leak syndrome (Clarkson syndrome) have been reported worldwide. This life-threatening condition is characterized by episodes of increase in vascular permeability with loss of fluid into the interstitium and presents with acute onset of edema, signs of tissue hypoperfusion, hemoconcentration, and low blood protein level. It has been diagnosed mainly in middle-aged adults with a monoclonal gammopathy. We performed a systematic review of the literature on Clarkson syndrome in subjects ≤ 18 years of age. We identified 24 reports, published since 1989, providing data on 32 otherwise healthy subjects, who experienced 67 well-documented episodes of Clarkson syndrome. The condition affected more frequently girls (21, 66%) than boys, presented throughout childhood, and was preceded by a mostly viral illness in 75% of cases. A monoclonal gammopathy was never reported. Uncompensated circulatory shock, muscle compartment syndrome, acute kidney injury, pulmonary edema, and either pleural or pericardial effusion were, in decreasing order of frequency, the most common complications. Four patients died.Conclusion: Clarkson syndrome develops not only in adulthood but also in childhood. In this age group, this condition is not linked to a monoclonal gammopathy. What is Known: • Clarkson syndrome is a rare condition that has been diagnosed mainly in middle-aged adults and is mostly linked to a monoclonal gammopathy. What is New: • In subjects ≤ 18 years of age, Clarkson syndrome is not linked to a monoclonal gammopathy.
PID Serval
serval:BIB_07CE981B6C1C
PMID
Date de création
2025-03-10T20:52:09.041Z
Date de création dans IRIS
2025-05-20T14:40:39Z