Two Episodes of Systemic Capillary Leak Syndrome in an 8-year-old Boy, Following Influenza A Virus Infection

In 1960, Dr. Bayard Clarkson described a woman experiencing sporadic, recurrent episodes of shock and anasarca. Plasma from an acute attack induced a “shock”-like syndrome when injected into rats. The enigmatic “Systemic Capillary Leak Syndrome” (SCLS) named for Dr. Clarkson is characterized by transient and severe, but reversible, hemoconcentration and hypoalbuminemia due to leakage of fluids and macromolecules into tissues. Although < 500 cases of SCLS have been reported in the literature since 1960, the condition is probably under-diagnosed due to lack of awareness and a high mortality without treatment. Allergists should be vigilant of this diagnosis since its presentation can resemble more common plasma leakage syndromes including angioedema or systemic anaphylaxis. Although the precise molecular etiology of SCLS remains unknown, substantial advances over the last five years have increased our understanding of SCLS pathogenesis.

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“…SCLS has also been reported in fourteen children during the past six years. 5, 17, 21, 25, 28, 35, 50, 52 …”

Section: Incidence Of Idiopathic Sclsmentioning

confidence: 99%

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

130

153

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“…14,15 In contrast, we found elevated levels of IL-8, TNF-a, and CCL2 in pediatric SCLS sera compared with sera from healthy control children, whereas concentrations of VEGF, Ang2, or CXCL10 were not different from controls (Fig 2). However, the absolute levels of all cytokines were much lower in the Sion-Sarid et al 2010 9 Gousseff et al 2011 3 Piastra et al 2012 10 Perme et al 2013 11 Iwasa et al 2014 12 children tested than those seen in adults.…”

Section: Discussionmentioning

confidence: 99%

“…3 Only 9 cases of clear-cut, idiopathic SCLS have been reported previously in children. 3,[5][6][7][8][9][10][11][12] Here we describe 6 children, aged between 5 months and 11 years, who presented with recurrent episodes of systemic capillary leak. Children were given prophylactic therapy successfully, which included theophylline plus verapamil or intravenous or subcutaneous IgG.…”

mentioning

confidence: 99%

Idiopathic Systemic Capillary Leak Syndrome in Children

et al. 2015

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“…Only 10 cases of ISCLS in children have been reported to date [3-11]. These attacks occurred at ages ranging from 5 months to 8 years.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2014

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BackgroundIdiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder, characterized by episodic life-threatening hypotension, hypoalbuminemia, and hemoconcentration.Case presentationA 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.ConclusionWe 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.

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Two Episodes of Systemic Capillary Leak Syndrome in an 8-year-old Boy, Following Influenza A Virus Infection

Cited by 17 publications

References 8 publications

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Idiopathic Systemic Capillary Leak Syndrome in Children

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

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