Clarkson disease — systemic capillary leak syndrome in a 6-year-old girl: case report

Abstract

Idiopathic systemic capillary leak syndrome is a rare and life-threatening disorder of unknown pathology. It results from leakage of plasma from the intravascular into the interstitial space and is characterised by recurrent episodes of shock, weight gain and generalised oedema. Recurrent episodes of this syndrome are reported in a 6-year-old girl who presented with the characteristic symptoms of shock, oedema and hypoalbuminaemia. She was treated effectively with fluid resuscitation, ionotropes and intravenous immunoglobin. Since systemic capillary leak syndrome is rare and the diagnosis is often missed, further research regarding its pathogenesis and treatment in children is warranted.

Keywords:

• Shock
• Hypoalbuminaemia
• Oedema
• Haemoconcentration
• Immunoglobulin