[show abstract][hide abstract] ABSTRACT: -There is limited data on the prognostic value of cardiovascular magnetic resonance (CMR) measurements in idiopathic pulmonary arterial hypertension, with no studies investigating the impact of correction of CMR indices for age and gender on prognostic value.
-Consecutive patients with idiopathic pulmonary arterial hypertension (IPAH) underwent CMR imaging at 1.5T. Steady state free precession (SSFP) cardiac volumes and mass measurements were corrected for age, gender and BSA according to reference data and prognostic significance assessed. 80 patients with IPAH were identified, 23 patients died during the mean follow-up of 32 +/- 14 months. Corrected for age, gender and BSA, RV end-systolic volume (p=0.004) strongly predicted mortality, independent of WHO functional class, mean right atrial pressure (mRAP), cardiac index (CI) and mixed venous oxygen saturations (mVO2).
-Consideration should be given to correcting CMR measures, for age, gender and BSA, particularly given the changing demographics of patients with IPAH. Corrected right ventricular end-systolic volume is a strong prognostic marker in IPAH, independent of invasively derived measurements, mRAP, CI and mVO2.
[show abstract][hide abstract] ABSTRACT: An antithrombin III variant was identified in the plasma of a female patient with a history of recurrent thromboses. The variant was shown to have normal antigenic levels but reduced heparin and progressive inhibitory activity consistent with an abnormality affecting function at the reactive centre. Polymerase chain reaction amplification of exon 6 of the gene with direct sequencing showed a point mutation resulting in the substitution of a proline for alanine at position 384. This substitution will predictably alter the conformation of the peptide loop containing the reactive centre of the molecule.
FEBS Letters 09/1989; 254(1-2):174-6.
Martina Daly added a full-text to this article and 3 others.
[show abstract][hide abstract] ABSTRACT: The proinflammatory cytokine interleukin-1beta (IL-1beta) is a secreted protein that lacks a signal peptide and does not follow currently known pathways of secretion. Its efficient release from activated immune cells requires a secondary stimulus such as extracellular ATP acting on P2X(7) receptors. We show that human THP-1 monocytes shed microvesicles from their plasma membrane within 2-5 s of activation of P2X(7) receptors. Two minutes after such stimulation, the released microvesicles contained bioactive IL-1beta, which only later appeared in the vesicle-free supernatant. We conclude that microvesicle shedding is a major secretory pathway for rapid IL-1beta release from activated monocytes and may represent a more general mechanism for secretion of similar leaderless secretory proteins.