systemic capillary leak syndrome

systemic capillary leak syndrome

a rare disorder of unknown cause presenting with episodic hypotension, hemoconcentration, and hypoalbuminemia; monoclonal gammopathy is often associated.

systemic capillary leak syndrome

a rare disorder of unknown cause presenting with episodic hypotension, hemoconcentration, and hypoalbuminemia; monoclonal gammopathy is often associated.
A condition in which the size of the pores in the capillaries are increased, leading to a leakage of 10+ litres of fluid into the interstitial space, resulting in haemoconcentration, hypoalbuminaemia, severe hypotension, generalised oedema and multiple organ failure— cardiopulmonary collapse, and acute renal failure—due to acute tubular necrosis following hypovolemia, rhabdomyolysis, due to reduced perfusion, causing progressive dyspnea and pericarditis, high fever, confusion, and disorientation
Aetiology Cytokine therapy with IL-2, GM-CSF, used to treat metastatic cancer
Prognosis The high mortality in early reports has been reduced by prophylactic therapy using terbutaline, theophyllin

sys·tem·ic cap·il·lar·y leak syn·drome

(sis-tem'ik kap'i-lar-ē lēk sin'drōm)
A rare disorder of unknown cause presenting with episodic hypotension, hemoconcentration, and hypoalbuminemia; monoclonal gammopathy is often associated.

systemic capillary leak syndrome

A rare disease whose hallmarks are episodes of hypotension associated with extravasation of plasma from the systemic circulation.
References in periodicals archive ?
Increased permeability and loss of the barrier function are two core characteristics of endothelial cells during sepsis, which are mainly attributed to morphological changes of endothelial cells, such as unbonding and protuberance of cell membrane, abnormal arrangement and obscured boundaries of endothelium, all above cause endothelium falling off from basal membrane, further translocation of circulatory substract, subendothelial edema, and systemic capillary leak syndrome, the latter results in plasma shifting from the blood vessel to interstitial spaces, decrease of central venous pressure and mean arterial pressure, and then hypoperfusion of organs, secondary systemic tissue injury and edema, and even MODS in severe cases.
Idiopathic hypoalbuminemia is different from systemic capillary leak syndrome (SCLS) which presents with recurrent acute episodes of vascular leakage manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema.
Idiopathic systemic capillary leak syndrome in children.
Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years.
Systemic capillary leak syndrome associated with compartment syndrome.
Treatment of the systemic capillary leak syndrome with terbutaline and theophylline.
Systemic capillary leak syndrome presenting as recurrent shock.
Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space.
Key Words: systemic capillary leak syndrome, compartment syndrome, rhabdomyolysis, polycythaemia
The systemic capillary leak syndrome (SCLS) is characterized by spontaneous recurrent episodes of hypotension, haemoconcentration, generalized oedema, and in most cases, the presence of a paraprotein.
In a series of eight patients, Tahirkheli and greipp from the mayo Clinic showed treatment with theophylline and terbutaline caused episodes of the systemic capillary leak syndrome to completely abate in some patients and decreased the incidence and severity of episodes in others (5).
Systemic capillary leak syndrome and monoclonal IgG gammapathy: studies in a sixth patient and review of the literature.