Abstract

Idiopathic systemic capillary leak syndrome in childhood: A Literature Review

Highlights

  • IntroductionSystemic capillary leak syndrome (SCLS), known as Clarkson’s disease[1], is a rare and severe condition characterized by a recurrent stereotypical triad: hypovolemic shock, generalized edema, paradoxical hemoconcentration and hypoalbuminemia[2]

  • Systemic capillary leak syndrome (SCLS), is a rare condition characterized by a recurrent stereotypical triad: hypovolemic shock, generalized edema, paradoxical hemoconcentration and hypoalbuminemia

  • Systemic capillary leak syndrome (SCLS), known as Clarkson’s disease[1], is a rare and severe condition characterized by a recurrent stereotypical triad: hypovolemic shock, generalized edema, paradoxical hemoconcentration and hypoalbuminemia[2]

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Summary

Introduction

Systemic capillary leak syndrome (SCLS), known as Clarkson’s disease[1], is a rare and severe condition characterized by a recurrent stereotypical triad: hypovolemic shock, generalized edema, paradoxical hemoconcentration and hypoalbuminemia[2]. It is caused by massive fluid extravasation into the interstitial space. Mortality may result from hemodynamic failure in the acute phase or cardiac failure due to reflex circulatory overload in the subacute phase[3]. The physiopathology of this disease is still debated. We review all the 21 published cases of pediatric idiopathic SCLS

Review of the literature
21 Moreira22 F 5 y
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