Studies in the Pathogenesis of Systemic Capillary Leak Syndrome

Inclusion Criteria

• •Subjects with SCLS under 8 years of age are only eligible for components of this protocol that can occur Off Site and samples can be sent to the NIH. All SCLS subjects must be at least 8 years old to participate in any other aspects of this protocol at the NIH.
• •Subjects with SCLS may enter study while pregnant and remain on study after becoming pregnant. At the discretion of the investigator, some components of this protocol will not occur while subject is pregnant.
• •Diagnosis or suspected diagnosis of Systemic Capillary Leak Syndrome by a physician or documented history of capillary leak as characterized by one or more of the following:
• •1. Hypotension (systolic blood pressure typically less than 90 mm Hg or diastolic blood pressure less than 60 mm Hg) or associated symptoms of hypotension (e.g., hypotonia \[collapse\], lightheadedness or syncope, incontinence, increased thirst)
• •2. Laboratory evidence of hemoconcentration (e.g., hemoglobin levels above the normal range \[typically greater than17 g/dL\], elevated leukocyte counts).
• •3. Clinical evidence of fluid extravasation (e.g. edema) and/or laboratory evidence of protein extravasation such as serum hypoalbuminemia (less than 3.5 g/dL) either chronically or during an acute hypotensive episode.
• •Letter of referral, with copies of pertinent medical history and laboratory studies, from prospective study subject s referring physician. Patients may self-refer, but a letter of referral from primary physician is still required.
• •Willing to donate blood for sample storage to be used for future research.
• •Willing to donate blood for sample storage to be used for future research.
• •Relatives must be at least 3 years old.
• •Healthy volunteers must be at least 18 years old.
• •Relatives may be pregnant or become pregnant while participating in this protocol. At the discretion of the investigator, some components of this protocol will not occur while subject is pregnant. No skin biopsies or intradermal testing will be done on pregnant relatives. Bloodwork volumes may be decreased at the discretion of the investigator.
• •Healthy volunteers may not be pregnant.
• •Able to give informed consent.