title: Idiopathic systemic capillary leak syndrome in children.
abstract: Adult subjects with systemic capillary leak syndrome (SCLS) present with acute and recurrent episodes of vascular leak manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema. We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented SCLS. Six children with SCLS were recruited from the United States, Australia, Canada, and Italy. Serum cytokines from SCLS subjects and a group of 10 healthy children were analyzed. Children with SCLS (aged 5-11 years old) presented with at least 1 acute, severe episode of hypotension, hypoalbuminemia, and hemoconcentration in the absence of underlying causes for these abnormalities. In contrast to what is observed in adult SCLS, identifiable infectious triggers precipitated most episodes in these children, and none of them had a monoclonal gammopathy. We found elevated levels of chemokine (C-C motif) ligand 2 (CCL2), interleukin-8, and tumor necrosis factor α in baseline SCLS sera compared with the control group. All patients are alive and well on prophylactic therapy, with 4 patients receiving intravenous or subcutaneous immunoglobulins at regular intervals. The clinical manifestations of pediatric and adult SCLS are similar, with the notable exceptions of frequent association with infections and the lack of monoclonal gammopathy. Prophylactic medication, including high dose immunoglobulins or theophylline plus verapamil, appears to be safe and efficacious therapy for SCLS in children.
authors.affiliation: Department of Allergy and Immunology, The Children's Hospital at Westmead, Sydney, Australia;
authors.forename: Peter
authors.initials: P
authors.lastname: Hsu
authors.affiliation: Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland; and.
authors.forename: Zhihui
authors.initials: Z
authors.lastname: Xie
authors.affiliation: Department of Immunology and Infectious Diseases, Sydney Children's Hospital, Sydney, Australia.
authors.forename: Katie
authors.initials: K
authors.lastname: Frith
authors.affiliation: Department of Allergy and Immunology, The Children's Hospital at Westmead, Sydney, Australia;
authors.forename: Melanie
authors.initials: M
authors.lastname: Wong
authors.affiliation: Department of Allergy and Immunology, The Children's Hospital at Westmead, Sydney, Australia;
authors.forename: Alyson
authors.initials: A
authors.lastname: Kakakios
authors.affiliation: Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland; and.
authors.forename: Kelly D
authors.initials: KD
authors.lastname: Stone
authors.affiliation: Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland; and kdruey@niaid.nih.gov.
authors.forename: Kirk M
authors.initials: KM
authors.lastname: Druey
mesh_terms: D019559:Capillary Leak Syndrome / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D004487:Edema / Q000097:blood / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D007223:Infant; D016209:Interleukin-8 / Q000097:blood; D008297:Male; D013806:Theophylline / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D016209:Interleukin-8; D014665:Vasodilator Agents; D013806:Theophylline
keywords: inflammatory cytokines; intravenous immunoglobulin; systemic capillary leak syndrome; vascular hyperpermeability