Subscribe to RSS
DOI: 10.1055/s-0037-1607342
Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase
Publication History
20 January 2017
13 September 2017
Publication Date:
23 October 2017 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00029029/201802/lookinside/thumbnails/10-1055-s-0037-1607342_1700005-1.jpg)
Abstract
Systemic capillary leak syndrome (SCLS) is a potentially life-threatening disorder characterized by distributive shock, hypoalbuminemia, and hemoconcentration. It is exceedingly rare in children with less than 20 cases reported to date. The underlying cause for this syndrome remains largely unknown and acute treatment has remained mainly supportive. Prophylaxis with intravenous immunoglobulin (IVIG) has been shown to successfully prevent further episodes in both adults and children. We present a case of a 2-year-old previously healthy male admitted to the pediatric intensive care unit with a clinical course consistent with SCLS. His shock was refractory to aggressive fluid and vasopressor support. Reversal of SCLS with IVIG given in the acute phase had been described in three adult subjects, and for this reason, the decision was made to administer IVIG. Within an hour of administration, hemodynamics stabilized and vasopressor support could be weaned. He has had no further episodes on prophylactic infusions of IVIG. Although the exact mechanism of IVIG in SCLS is unknown, it has proven to be an effective and safe prophylactic therapy, and in our patient, it drastically reversed the acute capillary leak. We suggest that IVIG should be considered as acute therapy in pediatric patients with refractory shock and a clinical course suggestive of SCLS.
Keywords
systemic capillary leak syndrome - intravenous immunoglobulin - hemoconcentration - hypoalbuminemiaNote
The views expressed in this article are those of the authors and do not reflect the official policy of the Navy, Department of Defense, or U.S. Government.
-
References
- 1 Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. Am J Med 1960; 29: 193-216
- 2 Druey KM, Greipp PR. Narrative review: the systemic capillary leak syndrome. Ann Intern Med 2010; 153 (02) 90-98
- 3 Hsu P, Xie Z, Frith K. , et al. Idiopathic systemic capillary leak syndrome in children. Pediatrics 2015; 135 (03) e730-e735
- 4 Perme T, Pokorn M, Markelj G. , et al. Two episodes of systemic capillary leak syndrome in an 8-year-old boy, following influenza A virus infection. Pediatr Infect Dis J 2014; 33 (02) 222-224
- 5 Pierce R, Khokha M, Kluger M, Lavik JP, Pober J. Pediatric systemic capillary leak syndrome endothelial cells are hypersensitive to TNF-[alpha] and LPS. Crit Care Med 2015; 43 (12) 25-26
- 6 Gousseff M, Arnaud L, Lambert M. , et al; Capillary Leak Syndrome Registry. The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med 2011; 154 (07) 464-471
- 7 Xie Z, Chan EC, Long LM, Nelson C, Druey KM. High-dose intravenous immunoglobulin therapy for systemic capillary leak syndrome (Clarkson disease). Am J Med 2015; 128 (01) 91-95
- 8 Lambert M, Launay D, Hachulla E. , et al. High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome. Crit Care Med 2008; 36 (07) 2184-2187
- 9 Kerketta J, Lodh M, Mandal K. Clarkson disease—systemic capillary leak syndrome in a 6-year-old girl: case report. Paediatr Int Child Health 2015; 35 (02) 160-163
- 10 Iwasa T, Ohashi H, Kihira K. , et al. 10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report. BMC Pediatr 2014; 14: 137
- 11 Karatzios C, Gauvin F, Egerszegi EP. , et al. Systemic capillary leak syndrome presenting as recurrent shock. Pediatr Crit Care Med 2006; 7 (04) 377-379
- 12 Kesik V, Atas E, Korkmazer N, Babacan O. Clofarabine associated capillary leak syndrome in a child with lymphoma successfully treated with intravenous immunoglobulin. J Cancer Res Ther 2015; 11 (03) 653