Adrenomedullin surges are linked to acute episodes of the systemic capillary leak syndrome (Clarkson disease).

ABSTRACT:

Background

Systemic Capillary Leak Syndrome (SCLS) is an extremely rare and life-threatening vascular disorder of unknown etiology. SCLS is characterized by abrupt and transient episodes of hypotensive shock and edema due to plasma leakage into peripheral tissues. The disorder has garnered attention recently because its initial presentation resembles more common vascular disorders including systemic anaphylaxis, sepsis, and acute infections with the Ebola/Marburg family of filoviruses. Although approximately 70-85% of patients with SCLS have a concurrent monoclonal gammopathy of unknown significance (MGUS), any contribution of the paraprotein to acute flares is unknown.

Procedure

To identify circulating factors that might trigger acute SCLS crises, we profiled transcriptomes

SUBMITTER: Xie Z 

PROVIDER: S-EPMC6312640 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature