Looking for Clarkson syndrome

Ever heard of Clarkson syndrome? No, nor me. It’s an acute condition that our adult colleagues are familiar with, but according to a literature review, can indeed occur in children, although rarely (Bozzini MA et al. Eur J Peds 2018. doi.org/10.1007/s00431-018-3189-8). Also known as idiopathic capillary leak syndrome, it causes acute life-threatening circulatory shutdown in previously healthy people. This review found 67 reported episodes in the world literature, affecting 32 children of all ages (newborn to 18 years). None had underlying conditions. Most had a preceding viral illness. All presented with oedema, hypoperfusion, haemoconcentration and low plasma protein levels. The most frequent complications were uncompensated circulatory shock (57%), and rhabdomyolysis (27%), leading to anterior tibial compartment syndrome in some (13%). Kidney injury, effusions and coagulopathies were also seen. Most were treated with colloid infusions and inotropic support, and 28% required ventilation. Many other drugs were used, including antimicrobials and steroids. Most recovered over a couple of weeks, but four died in the acute phase. In 69% the episodes were recurrent (up to six times), and various strategies were used to prevent recurrence, such as polyclonal immunoglobulin infusions and methylxanthines. In adults, it is often associated with …