Published in Best practice & research. Clinical rheumatology
Over the past two decades, there have been several advances in the assessment and management of connective tissue disease-related pulmonary arterial hypertension (CTD-PAH) that improved outcomes of the treatment of this lethal disease, and this will be the focus of this study. Systemic sclerosis is the leading cause of CTD-PAH, followed by systemic...
Published in Pediatric Surgery International
PurposeSphingolipids play a crucial role in pulmonary development. The sphingosine kinase 1 (SphK1) modulates the synthesis of sphingolipid sphingosine-1-phosphate (S1P). S1P regulates cell proliferation and angiogenesis via different receptors, S1P1, S1P2 and S1P3, which all influence the expression of Ras-related C3 botulinum toxin substrate 1 (R...
Published in Pediatric Cardiology
Pulmonary hypertension (PHT) in the preterm infant is frequently due to chronic lung disease. Rarely, PHT can be caused by pulmonary vein (PV) stenosis that has been described to be associated with prematurity. This study is a retrospective analysis of all premature infants
Published in Best practice & research. Clinical rheumatology
The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features inclu...
Published in Interactive cardiovascular and thoracic surgery
Pre- and postoperative ECMO bridging in patients undergoing PEA is a feasible option to stabilize patients in a critical pre- and/or postoperative situation and to improve outcome in these patients who would otherwise probably not survive the procedure.
Published in Respiration
Background: The insertion/deletion (I/D) polymorphism in the angiotensin-converting enzyme gene (ACE) and the C825T polymorphism in the G-protein β3 subunit gene (GNB3) are associated with the efficacy of phosphodiesterase-5 inhibitor (PDE-5I) in erectile dysfunction. In addition, GNB3 genotypes could be associated with clinical worsening in pulmon...
Published in BMC Pulmonary Medicine
BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease. For patients with operable CTEPH, there is a clear recommendation for surgical removal of persistent thrombi by pulmonary endarterectomy (PEA). However, without the presence of PH, therapeutic management of chronic thromboembolic disease (CTED) is challenging ...
Published in Journal of Biomedical Science
BackgroundIncreased circulating levels of endoglin+ endothelial microparticles (EMPs) have been identified in several cardiovascular disorders, related to severity. Endoglin is an auxilary receptor for transforming growth factor β (TGF-β) important in the regulation of vascular structure.ResultsWe quantified the number of microparticles in plasma o...
Published in Neonatology
Background: Pulmonary hypertension (PH) in infants with bronchopulmonary dysplasia (BPD) is associated with increased morbidity and mortality. Elevated levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) and decreased levels of amino acid precursors of nitric oxide (NO) have been associated with PH, but have not been studied in infants ...
Published in Journal of Cardiothoracic Surgery
BackgroundCardiac surgery patients at very high risk are difficult to stratify with the existing risk scores. The objective of this study is to assess the clinical performance of two existing risk stratification scores (EuroSCORE II and ACEF score) in the setting of very high-risk patients undergoing cardiac surgery, and to identify a possible stra...
