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Episodic idiopathic systemic capillary leak syndrome in a girl

Gregorio Paolo Milani1, Rosa Maria Dellepiane, Massimo Luca Castellazzi

  • 1Pediatric Emergency Unit, Clinica De Marchi, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. yoyobiancorosso@hotmail.com

Pediatrics International : Official Journal of the Japan Pediatric Society
|August 6, 2013
PubMed

Insights

Episodic idiopathic systemic capillary leak syndrome (ISCLS) is a rare condition causing shock from plasma leakage. This case report highlights a girl

Area of Science:

  • Internal Medicine
  • Pediatrics
  • Hematology

Background:

  • Episodic idiopathic systemic capillary leak syndrome (ISCLS) is a rare, life-threatening disorder characterized by recurrent episodes of circulatory shock.
  • The pathophysiology involves transient, unexplained increases in vascular permeability, leading to plasma extravasation into interstitial spaces.

Observation:

  • A pediatric case of recurrent circulatory shock is presented.
  • The patient experienced multiple episodes of shock before a definitive diagnosis was established.
  • Diagnosis was confirmed as episodic idiopathic systemic capillary leak syndrome after the fourth episode.

Findings:

  • The diagnosis of episodic idiopathic systemic capillary leak syndrome was challenging due to its rarity and episodic nature.
  • Prompt treatment with acute intravenous (IV) crystalloids and methylprednisolone was initiated.
  • The patient's clinical course showed a favorable response to the administered treatment.

Implications:

  • This case underscores the importance of recognizing ISCLS in patients with recurrent unexplained shock.
  • Early diagnosis and prompt management with IV crystalloids and corticosteroids can lead to favorable outcomes.
  • Further research into the underlying mechanisms and optimal treatment strategies for ISCLS is warranted.

Keywords:
circulatory shockepisodic idiopathic systemic capillary leak syndrome

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