Rare Diseases Symptoms Automatic Extraction

Systemic capillary leak syndrome.

[systemic capillary leak syndrome]

Systemic capillary leak syndrome is a rare, severe disorder with a high mortality rate. It consists of the shift of fluid and proteins from the intravascular to the extravascular compartment with subsequent hypovolemic shock. We describe a 34-year-old-woman who had several episodes of generalized edema that evolved to hypovolemic shock. During the acute phase, laboratory investigations revealed marked hypoproteinemia, leukocytosis, and high levels of hematocrit and hemoglobin. A paraprotein IgG kappa chain was detected. Although different therapeutic trials were used, the patient continued to have similar episodes and she died during an acute episode 2 1/2 years after the first symptom of this disorder. The cause of systemic capillary leak syndrome is unknown. The presence of a paraprotein IgG is frequent in this group of patients.

Diseases presenting "acute phase" symptom

  • allergic bronchopulmonary aspergillosis
  • benign recurrent intrahepatic cholestasis
  • focal myositis
  • hydrocephalus with stenosis of the aqueduct of sylvius
  • locked-in syndrome
  • neuralgic amyotrophy
  • scrub typhus
  • severe combined immunodeficiency
  • systemic capillary leak syndrome

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