title: 10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report.
abstract: BACKGROUND
  Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder, characterized by episodic life-threatening hypotension, hypoalbuminemia, and hemoconcentration.
  
  
  METHODS
  A 10-year-old girl presented with abdominal pain, vomiting, diarrhea, fever and developed generalized edema a day after admission. Clinical and laboratory findings were consistent with ISCLS. She received aggressive fluid replacement, methylprednisolone pulse (30 mg/kg/day), high-dose intravenous immunoglobulin (IVIG, 2 g/kg/day) and plasma exchange in acute phase. She received fasciotomy of bilateral lower extremities as she developed complications of compartment syndrome. Since there were two episodes of ISCLS attacks, theophylline and terbutaline were initiated for prevention of attacks and then the remission is currently maintained. Because of high fatality rate in ISCLS, prompt diagnosis and intervention are very important.
  
  
  CONCLUSIONS
  We describe here, a rare case of pediatric ISCLS. ISCLS should be considered as a differential diagnosis, when the patient presents with unexplained or sudden hypovolemic shock. Reports on pediatrics ISCLS are very few, and accumulation of similar case reports is needed.
authors.affiliation: Department of Pediatrics, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu City, Mie Prefecture 514-8507, Japan. masaru@clin.medic.mie-u.ac.jp.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Iwasa
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ohashi
authors.forename: Kentaro
authors.initials: K
authors.lastname: Kihira
authors.forename: Yuhki
authors.initials: Y
authors.lastname: Koike
authors.forename: Kohei
authors.initials: K
authors.lastname: Otake
authors.forename: Mikihiro
authors.initials: M
authors.lastname: Inoue
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Sawada
authors.forename: Hidemi
authors.initials: H
authors.lastname: Toyoda
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Komada
mesh_terms: D058666:Adrenergic beta-2 Receptor Agonists / Q000627:therapeutic use; D019559:Capillary Leak Syndrome / Q000175:diagnosis / Q000628:therapy*; D002648:Child; D003161:Compartment Syndromes / Q000209:etiology / Q000601:surgery; D000071938:Fasciotomy; D005260:Female; D005440:Fluid Therapy; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D010951:Plasma Exchange; D013726:Terbutaline / Q000627:therapeutic use; D013806:Theophylline / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D058666:Adrenergic beta-2 Receptor Agonists; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D014665:Vasodilator Agents; D013806:Theophylline; D013726:Terbutaline; D008775:Methylprednisolone