Literature DB >> 16738498

Systemic capillary leak syndrome presenting as recurrent shock.

Christos Karatzios1, France Gauvin, E Patricia Egerszegi, Bruce Tapiero, Chantal Buteau, Georges Etienne Rivard, Philippe Ovetchkine.   

Abstract

OBJECTIVE: To report a case of systemic capillary leak syndrome (SCLS) in a child.
DESIGN: Case report.
SETTING: Pediatric intensive care unit. PATIENT: A 6-yr-old girl was admitted twice to the pediatric intensive care unit, at a 10-month interval, in severe shock with important edema.
RESULTS: The patient presented with acute symptoms of abdominal pain, vomiting, and syncope in the hour preceding the shock. During both episodes necessary management included aggressive intravenous fluid rehydration, mechanical ventilation, and use of inotropes/vasopressors. Suspicion of a lower limb fasciitis necessitated surgical exploration, but pathology reports were negative on both occasions revealing only subcutaneous tissue edema. The patient recovered within 24 hrs on both episodes. Investigation ruled out cardiogenic shock and septic shock due to bacterial etiology. On the first episode, a nasopharyngeal aspirate was positive for influenza A (H3N2) by both viral immunofluorescence and culture. The presumed diagnosis was toxic shock syndrome associated with influenza virus. On the second episode, all bacterial and virology cultures remained negative. Hypovolemic shock was suspected, but there was no history of dehydration, bleeding, or gastrointestinal losses (persistent vomiting or diarrhea). Noninfectious causes of hypovolemic shock with edema were ruled out, leading us to believe that she suffered from SCLS.
CONCLUSIONS: Although well described in the adult literature, there have been few reports of SCLS in pediatric patients. SCLS should be considered in the differential diagnosis of recurrent hypovolemic shock without identifiable cause. The only therapeutic intervention is to obtain vascular access when initial manifestations occur and give aggressive fluid reanimation.

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Year:  2006        PMID: 16738498     DOI: 10.1097/01.PCC.0000227120.61949.FB

Source DB:  PubMed          Journal:  Pediatr Crit Care Med        ISSN: 1529-7535            Impact factor:   3.624


  7 in total

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2.  Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase.

Authors:  Anna Sofi E Asmundsson; Ashley R Bjorklund; Gwenyth A Fisher
Journal:  J Pediatr Intensive Care       Date:  2017-10-23

3.  Idiopathic systemic capillary leak syndrome in children.

Authors:  Peter Hsu; Zhihui Xie; Katie Frith; Melanie Wong; Alyson Kakakios; Kelly D Stone; Kirk M Druey
Journal:  Pediatrics       Date:  2015-03       Impact factor: 7.124

4.  High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome.

Authors:  Manuel Zipponi; Roland Eugster; Tanja Birrenbach
Journal:  BMJ Case Rep       Date:  2011-05-03

5.  Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience.

Authors:  Prashant Kapoor; Patricia T Greipp; Eric W Schaefer; Sumithra J Mandrekar; Arif H Kamal; Natalia C Gonzalez-Paz; Shaji Kumar; Philip R Greipp
Journal:  Mayo Clin Proc       Date:  2010-07-15       Impact factor: 7.616

6.  Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.

Authors:  Marie-Ange Bozzini; Gregorio P Milani; Mario G Bianchetti; Emilio F Fossali; Sebastiano A G Lava
Journal:  Eur J Pediatr       Date:  2018-06-23       Impact factor: 3.183

7.  10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report.

Authors:  Tadashi Iwasa; Hiroyuki Ohashi; Kentaro Kihira; Yuhki Koike; Kohei Otake; Mikihiro Inoue; Hirofumi Sawada; Hidemi Toyoda; Yoshihiro Komada
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  7 in total

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