Idiopathic systemic capillary leak syndrome in children
Peter Hsu1, Zhihui Xie2, Katie Frith3
1Department of Allergy and Immunology, The Children's Hospital at Westmead, Sydney, Australia;
Pediatrics
|February 26, 2015
Summary
Pediatric systemic capillary leak syndrome (SCLS) differs from adult SCLS, often triggered by infections and lacking monoclonal gammopathy. Prophylactic therapies like immunoglobulins are safe and effective for children with SCLS.
Area of Science:
- Pediatric Hematology
- Immunology
- Critical Care Medicine
Background:
- Systemic capillary leak syndrome (SCLS) is characterized by acute vascular leakage in adults.
- Pediatric SCLS presents similarly with hypotension, hypoalbuminemia, hemoconcentration, and edema.
Observation:
- A cohort of six children with SCLS was studied across multiple countries.
- Clinical features, serum cytokine profiles, and treatments were analyzed.
- Infectious triggers were identified in most pediatric SCLS episodes, unlike adult SCLS.
Findings:
- Children with SCLS showed elevated serum levels of chemokine (C-C motif) ligand 2 (CCL2), interleukin-8, and tumor necrosis factor α.
- Monoclonal gammopathy was absent in all pediatric SCLS cases.
- All pediatric patients were successfully managed with prophylactic therapies.
Implications:
- Pediatric SCLS has distinct triggers and lacks monoclonal gammopathy compared to adult SCLS.
- Prophylactic treatments, including high-dose immunoglobulins or theophylline/verapamil, are safe and effective for pediatric SCLS.
- Understanding these differences aids in diagnosing and managing pediatric SCLS.
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