Idiopathic Capillary Leak Syndrome Complicated by Massive Rhabdomyolysis

Idiopathic systemic capillary leak syndrome (Clarkson's disease) is characterized by recurring attacks of increased capillary permeability, resulting in severe hypovolemic shock due to plasma extravasation. Additional laboratory features include association with a monoclonal gammopathy, extreme...

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Veröffentlicht in:Chest 1993-07, Vol.104 (1), p.123-126
Hauptverfasser: Dolberg-Stolik, Orit C., Putterman, Chaim, Rubinow, Alan, Rivkind, Avraham I., Sprung, Charles L.
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container_end_page 126
container_issue 1
container_start_page 123
container_title Chest
container_volume 104
creator Dolberg-Stolik, Orit C.
Putterman, Chaim
Rubinow, Alan
Rivkind, Avraham I.
Sprung, Charles L.
description Idiopathic systemic capillary leak syndrome (Clarkson's disease) is characterized by recurring attacks of increased capillary permeability, resulting in severe hypovolemic shock due to plasma extravasation. Additional laboratory features include association with a monoclonal gammopathy, extreme hemoconcentration, and hypoalbuminemia. Rare manifestations of this syndrome are renal damage and rhabdomyolysis due to increased compartment pressure and ischemic myonecrosis. We present the findings in two patients with capillary leak syndrome complicated by severe rhabdomyolysis, in one case leading to acute renal failure. We review therapeutic aspects of this rare syndrome and emphasize the importance of early diagnosis and of prompt and aggressive fluid replacement.
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Vascular system</topic><topic>Compartment Syndromes - etiology</topic><topic>Diseases of the peripheral vessels. Diseases of the vena cava. 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Additional laboratory features include association with a monoclonal gammopathy, extreme hemoconcentration, and hypoalbuminemia. Rare manifestations of this syndrome are renal damage and rhabdomyolysis due to increased compartment pressure and ischemic myonecrosis. We present the findings in two patients with capillary leak syndrome complicated by severe rhabdomyolysis, in one case leading to acute renal failure. We review therapeutic aspects of this rare syndrome and emphasize the importance of early diagnosis and of prompt and aggressive fluid replacement.</abstract><cop>Northbrook, IL</cop><pub>Elsevier Inc</pub><pmid>8325053</pmid><doi>10.1378/chest.104.1.123</doi><tpages>4</tpages></addata></record>
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subjects Acute Kidney Injury - etiology
Adolescent
Adult
Biological and medical sciences
Blood and lymphatic vessels
Capillary Permeability - physiology
Cardiology. Vascular system
Compartment Syndromes - etiology
Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous
Edema - complications
Humans
Male
Medical sciences
Plasma
Rhabdomyolysis - etiology
Shock - etiology
Shock - physiopathology
Syndrome
title Idiopathic Capillary Leak Syndrome Complicated by Massive Rhabdomyolysis
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