Publications

BibTex format

@article{Newnham:2019:10.1183/13993003.01805-2018,
author = {Newnham, M and South, K and Bleda, M and Auger, WR and Barberà, JA and Bogaard, H and Bunclark, K and Cannon, JE and Delcroix, M and Hadinnapola, C and Howard, LS and Jenkins, D and Mayer, E and Ng, C and Rhodes, CJ and Screaton, N and Sheares, K and Simpson, MA and Southwood, M and Su, L and Taboada, D and Traylor, M and Trembath, RC and Villar, SS and Wilkins, MR and Wharton, J and Gräf, S and Pepke-Zaba, J and Laffan, M and Lane, DA and Morrell, NW and Toshner, M},
doi = {10.1183/13993003.01805-2018},
journal = {European Respiratory Journal},
title = {The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension},
url = {http://dx.doi.org/10.1183/13993003.01805-2018},
volume = {53},
year = {2019}
}

Download

RIS format (EndNote, RefMan)

TY  - JOUR
AB  - Chronic thromboembolic pulmonary hypertension (CTEPH) is an important consequence of pulmonary embolism (PE) that is associated with abnormalities in haemostasis. We investigated the ADAMTS13-VWF axis in CTEPH, including its relationship to disease severity, inflammation, ABO groups and ADAMTS13 genetic variants.ADAMTS13 and VWF plasma antigen levels were measured in patients with CTEPH (n=208), chronic thromboembolic disease without pulmonary hypertension (CTED; n=35), resolved PE (n=28), idiopathic pulmonary arterial hypertension (n=30) and healthy controls (n=68). CTEPH genetic ABO associations and protein quantitative trait loci were investigated. ADAMTS-VWF axis abnormalities were assessed in CTEPH and healthy control subsets by measuring ADAMTS13 activity, D-dimers and VWF-multimeric size.CTEPH patients had decreased ADAMTS13 (adjusted β (95% CI)=−23.4 (−30.9– −15.1)%, p<0.001) and increased VWF levels (β=+75.5 (44.8–113)%, p<0.001) compared to healthy controls. ADAMTS13 levels remained low after reversal of pulmonary hypertension by pulmonary endarterectomy surgery and were equally reduced in CTED. We identify a genetic variant near the ADAMTS13 gene associated with ADAMTS13 protein that accounted for ∼8% of the variation in levels.The ADAMTS13-VWF axis is dysregulated in CTEPH. This is unrelated to pulmonary hypertension, disease severity or markers of systemic inflammation and implicates the ADAMTS13-VWF axis in CTEPH pathobiology.
AU  - Newnham,M
AU  - South,K
AU  - Bleda,M
AU  - Auger,WR
AU  - Barberà,JA
AU  - Bogaard,H
AU  - Bunclark,K
AU  - Cannon,JE
AU  - Delcroix,M
AU  - Hadinnapola,C
AU  - Howard,LS
AU  - Jenkins,D
AU  - Mayer,E
AU  - Ng,C
AU  - Rhodes,CJ
AU  - Screaton,N
AU  - Sheares,K
AU  - Simpson,MA
AU  - Southwood,M
AU  - Su,L
AU  - Taboada,D
AU  - Traylor,M
AU  - Trembath,RC
AU  - Villar,SS
AU  - Wilkins,MR
AU  - Wharton,J
AU  - Gräf,S
AU  - Pepke-Zaba,J
AU  - Laffan,M
AU  - Lane,DA
AU  - Morrell,NW
AU  - Toshner,M
DO  - 10.1183/13993003.01805-2018
PY  - 2019///
SN  - 0903-1936
TI  - The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension
T2  - European Respiratory Journal
UR  - http://dx.doi.org/10.1183/13993003.01805-2018
UR  - http://hdl.handle.net/10044/1/66846
VL  - 53
ER  -

Download

DrChristopherRhodes

Contact

Location

Summary

Citation

BibTex format

RIS format (EndNote, RefMan)