Rare Diseases Symptoms Automatic Extraction

Capillary leak syndrome in a 5-month-old infant associated with intractable diarrhoea.

[systemic capillary leak syndrome]

Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown pathophysiology, characterised by episodic life-threatening hypotension, haemoconcentration and hypo-albuminaemia. A 5-month-old child presented with episodes of relapsing diarrhoea, vomiting and hyponatraemia and developed generalised oedema during treatment. Clinical and laboratory findings were consistent with acute SCLS. Treatment with careful fluid replacement, fresh frozen plasma and aminophylline in the acute phase and teophylline and gingko biloba in the chronic phase led to sustained remission during follow-up for over 1 year. To our knowledge, this is the youngest case of SCLS in the literature and is unusual in that it presented with diarrhoea.