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Case Reports
. 2014 May 1;189(9):1145-7.
doi: 10.1164/rccm.201310-1746LE.

Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease

Zhihui Xie  1 , Chandra C Ghosh, Samir M Parikh, Kirk M Druey
Affiliations
Case Reports

Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease

Zhihui Xie et al. Am J Respir Crit Care Med. .
No abstract available

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Figures

Figure 1.
Figure 1.
Molecular classification of systemic capillary leak syndrome (SCLS) using transendothelial electrical resistance (TER) assays. Human microvascular endothelial cells (HMVEC) were plated to confluence on TER assay wells. Cells were serum starved in 1% fetal bovine serum followed by addition of sera at time zero, and resistance was measured over time. (A) Normalized resistance values (absolute resistance at each time point divided by resistance at time zero) after addition of patient 14’s basal (blue) or episodic (red) sera or sera pooled from eight healthy control subjects (green). Plot is from a single experiment representative of 3 to 5 replicates per condition. (B, C) The effect of intravenous immunoglobulin (IVIG) on HMVEC permeability induced by acute sera from patients with classical SCLS (n = 5) (B) or atypical patient 14 (C). Cells were pretreated with bovine serum albumin (BSA) or IVIG at a final concentration of 1.25 mg/ml for 1 hour followed by TER measurements. Bar graph shows end point resistance values at 2.5 hours after serum addition (mean ± SEM of 3–5 replicates per condition; *P = 0.01, t test). n.s. = not significant. (D) Anti-IL8 (5 μg/ml), infliximab (5 μg/ml), or equivalent concentrations of control IgG1 (Thermo Fisher Scientific, Waltham, MA) was applied to HMVECs 1 hour before addition of acute SCLS sera from patient 14. Bar graph shows end point resistance values at 2.5 hours after serum addition (mean ± SEM of 3–5 replicates per condition; **P = 0.003, ***P = 0.0005, t test). All patients in this study gave informed consent for the publication of this letter.
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References

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