[Systemic capillary leak syndrome].

[systemic capillary leak syndrome]

A man aged 61 had recurrent attacks of severe shock . The episodes were preceded by symptoms such as a runny nose , epigastric discomfort with nausea , vertigo , orthostatism and sometimes light fever . During the attacks there were marked hypotension , a strong rise of the haematocrit , a decrease of the protein and albumin concentrations in the blood and prerenal kidney failure . In addition , there was a paraprotein , type IgG-kappa . The shock every time responded rapidly to intravenous administration of fluid and was followed by a period of substantial polyuria . The pattern was characteristic of systemic capillary leak syndrome , a rare but frequently fatal disease characterized by episodes of unexplained extravasation of plasma . The aetiology and pathogenesis are unknown . Attacks are suppressed by supportive therapy (administration of fluids , inotropics ) and future attacks may be prevented by the intake of terbutaline and theophylline . The systemic capillary leak syndrome should be considered in the differential diagnosis of idiopathic and anaphylactic shock .

Diseases
Validation

Diseases presenting "polyuria" symptom

congenital toxoplasmosis

erdheim-chester disease

familial hypocalciuric hypercalcemia

primary hyperoxaluria type 1

systemic capillary leak syndrome

typhoid

This symptom has already been validated