David V procedure and hemiarch replacement in a patient with Loeys-Dietz-Syndrome and beta thalassemia minor: a case report.

We report the case of a 36-year-old European female patient presenting with a sinus valsalva aneurysm of 47 mm with moderate aortic regurgitation. Additionally, an aneurysm of the brachiocephalic trunk and multiple aneurysms of the right internal mammary artery were identified. Previous medical history included Loeys-Dietz syndrome (LDS) Type RII due to a TGF-beta receptor mutation, and beta thalassemia minor with a baseline hemoglobin of 9,3 g/dL on admission.

Selection of Parameters for Optimized WAAM Structures for Civil Engineering Applications.

Using the CMT (Cold Metal Transfer, F. Fronius, Upper Austria) welding process, wire arc additive manufacturing (WAAM) enables companies to fabricate steel components in a resource-saving manner (additive vs. subtractive) by properly reinforcing existing steel components.

Reduction ascending aortoplasty: A retrospective analysis of outcomes and risk factors.

Objectives: Indication for Reduction of Ascending Aortoplasty (RAA) and long-term outcomes remain unclear. This study analyzed the outcomes after nonreinforced RAA in two Austrian centers.

Methods: Patients with RAA at two Austrian centers between 6/2,009 and 6/2,017 were retrospectively analyzed.

'We don't worry that much about language': street-level bureaucracy in the context of linguistic diversity.

The way we deal with diversity is crucial for social equity in the context of migration-related super-diversityand represents a challenge for all actors involved. The present article aims to contribute to the understanding of linguistic discrimination by contrasting the perceptions of institutional actors and mobile European citizens concerning language-related barriers when accessing labour market mediation services and benefits. The article draws exemplarily on empirical data (mainly qualitative interviews) relating to the provision of labour market-related services by the Austrian Employment Service and Hungarian migrants' experiences with this institution.

The role of Foxp3 and Tbet co-expressing Treg cells in lung carcinoma.

Despite the opposite roles of Tbet and Foxp3 in the immune system as well as in tumour biology, recent studies have demonstrated the presence of of CD4 T cells, expressing both, Tbet and Foxp3. Although TbetFoxp3 T cells are currently a subject of intense research, less is known about their biological function especially in cancer. Here we found a considerable accumulation of TbetFoxp3CD4 T cells, mediated by the immunosuppressive cytokine TGFβ in the lungs of tumour bearing mice.

Enhanced Acid Sphingomyelinase Activity Drives Immune Evasion and Tumor Growth in Non-Small Cell Lung Carcinoma.

The lipid hydrolase enzyme acid sphingomyelinase (ASM) is required for the conversion of the lipid cell membrane component sphingomyelin into ceramide. In cancer cells, ASM-mediated ceramide production is important for apoptosis, cell proliferation, and immune modulation, highlighting ASM as a potential multimodal therapeutic target. In this study, we demonstrate elevated ASM activity in the lung tumor environment and blood serum of patients with non-small cell lung cancer (NSCLC).

NFATc1 deficiency in T cells protects mice from experimental autoimmune encephalomyelitis.

NFATc1 is a member of the nuclear factor of activated T cells (NFAT) family of transcription factors. NFAT is activated upon T-cell receptor activation followed by intracytoplasmatic calcium influx where calmodulin, a calcium sensor protein, activates the phosphatase calcineurin that dephosphorylates NFAT proteins and results in NFAT nuclear import. Here, we show the analysis of conditional NFATc1-deficient mice bearing a deletion of NFATc1 in CD4(+) and CD8(+) T cells.

Short-term clinical outcomes for intermittent cold versus intermittent warm blood cardioplegia in 2200 adult cardiac surgery patients.

Background: Aim of the present study was to compare clinical outcome of intermittent cold (ICC) versus intermittent warm (IWC) blood cardioplegia in different cardiosurgical procedures.

Methods: Two thousand one hundred and eighty-eight patients were retrospectively divided into 5 groups: isolated coronary artery bypass surgery (CABG; N.=1203), isolated aortic valve surgery (AVR; N.

Type A dissection and chronic dilatation: tenascin-C as a key factor in destabilization of the aortic wall.

Objectives: Tenascin-C plays an important role in myocardial and vascular remodelling. We hypothesized that tenascin-C is a key factor in the development of degenerative disease of the ascending aorta, leading to chronic dilatation and acute aortic dissection.

Methods: Ascending aortic wall specimens were obtained during surgery for chronic dilatation (n=52) and acute Type A dissection (n=30).

Idiopathic pulmonary artery aneurysm.

A 60-year-old male being treated for chronic venous stasis ulcers presented with an asymptomatic pulmonary artery aneurysm involving the main pulmonary trunk and extending into both pulmonary arteries. He underwent successful resection of the aneurysm, and his chronic ulcerations recovered spontaneously. Surgical treatment of pulmonary artery aneurysm could be found in reports from Wilms and Sauerbruch dating from the early twenties of the last century.

Hypoplastic thyroid, growth hormone deficiency, corneal opacities, cataract and hyperkeratotic skin disease: a possible new ichthyosis syndrome associated with endocrinopathies.

A 56 year old man presented with ichthyosis vulgaris since early childhood, clinically characterised by fine scaling of the trunk and hyperkeratotic scales on the exterior surfaces of the upper and lower extremities. The patient also showed hypothyroidism due to hypoplastic thyroid, cataract, hypercholesterinemia with concommitant arcus cornealis and biliary concrements. Renal lithiasis caused by calcio-oxalate was additionally present.

[Therapy with somatostatin analogs in patient with orbitopathy and positive Octreoscan].

We prospectively evaluated 28 persons with active endocrine ophthalmopathy and positive sonographic criteria (A-mode) on extraocular eye muscles. To evaluate somatostatin-receptor status SPECT of the orbits was performed with a double-headed rotating gamma camera after application of 110 MBq 111-In-Pentreotide. 9 patients (12/56 eyes respectively) showed a marked uptake ratio (> 2 in circular ROIs by semiquantitative calculation) and were selected for lanreotide (30 mg i.

Pre- and early postoperative risk factors for death after cardiac transplantation: a single center analysis.

Due to the limited number of donor organs, death on the waiting list and waiting time for cardiac transplantation have markedly increased. A pressing need of appropriate selection criteria for patients who would benefit most from transplantation is apparent. The purpose of this study is to identify pre- and early postoperative risk factors that influence long term survival after cardiac transplantation.

Are T cells from healthy heart really only passengers? Characterization of cardiac tissue T cells.

Numerous studies have dealt with occurrence of dendritic cells in various nonlymphoid organs such as kidney, liver or heart, whereas lymphocyte patterns in these organs have not been analyzed in detail. In the present study, leukocytes were quantified as cells/mm2 in the perivascular, interstitial and parenchymal tissue sections of normal heart. We measured an overall mean leukocyte count in normal heart tissue of 17.

Phenotypic patterns of mononuclear cells in dilated cardiomyopathy.

Background: Immunological factors in the pathogenesis of idiopathic dilated cardiomyopathy (IDC) were suggested previously on the basis of the demonstration of mononuclear cell infiltrates and autoantibodies against the myocardium. The present study investigated whether tissue leukocyte subpopulations isolated from hearts with IDC (n = 6) differ in phenotype from those of tissues without IDC (n = 7).

Methods And Results: Leukocytes were quantified as reactive cells per square millimeter in perivascular, interstitial, and parenchymal tissue sections.

Treatment of non-healing skin ulcers with autologous activated mononuclear cells.

The aim of this study was to investigate whether cultured autologous mononuclear cells (MNC) effectively initiate, accelerate and improve granulation and epithelialisation of skin ulcers. Thirty-three patients with chronic arterial occlusive disease (CAOD; n = 21) or venous post-thrombotic syndrome (PTS; n = 12) were treated with autologous MNC and compared with a control group of 30 patients who received tissue culture medium alone. Previous treatments had been unsuccessful for a mean of 9.

Effects of interleukin-1, -2, -4, -6, interferon-gamma and granulocyte/macrophage colony stimulating factor on human vascular endothelial cells.

Human vascular endothelial cells (HUVEC) exhibit various immunological functions, i.e. expression of HLA class-II antigens after incubation with IFN-gamma or antigen presenting function.

[The coronary sinus as a source of activated T-lymphocytes in patients after orthotopic heart transplantation].

Mononuclear cells are the component of blood responsible for allograft recognition, rejection and acceptance. Shifts in the patterns of various mononuclear cell subpopulations were often used as a diagnostic tool in detection of heart rejection. The specificity of mononuclear cell monitoring has remained a controversial point until today, because infections led to similar changes as organ rejection.

Lyme borreliosis as a cause of myocarditis and heart muscle disease.

Lyme borreliosis (LB) is a multisystem disorder that may cause self-limiting or chronic diseases of the skin, the nervous system, the joints, heart and other organs. The aetiological agent is the recently discovered Borrelia burgdorferi. In 1980, cardiac manifestations of LB were first described, including acute conduction disorders, atrioventricular block, transient left ventricular dysfunction and even cardiomegaly.

Monitoring of mononuclear cell subsets isolated from the coronary sinus and the right atrium in patients after heart allograft transplantation.

The rejection of a transplanted heart leads to an accumulation of mononuclear cells in the cardiac tissue and to reactions of the antigen-recognizing cells with the foreign tissue. Consequently, during rejections immunologic changes, such as the number of mononuclear cells and the patterns of mononuclear cell subpopulations, should be detectable by analysis of mononuclear cells from the coronary sinus of transplanted hearts. Seventy-nine endomyocardial biopsies were performed in 37 patients.

Quality of life after orthotopic heart transplantation.

During the last decade heart transplantation has become the chosen method to treat terminally ill patients suffering from severe cardiac illness. It was the aim of our study to retrospectively survey life quality of donor organ recipients who underwent heart transplantation during the first years of transplantation at our center (1984 through 1987). Thirty-five patients were asked to evaluate their postoperative improvement or deterioration and their satisfaction with the level reached on visual scales.

[Unilateral lung transplantation as an effective therapy in primary lung emphysema].

Unilateral lung transplantation is the treatment of choice for terminal restrictive lung disease. We report about three patients with end-stage pulmonary emphysema treated by single lung transplantation. All patients are alive 3, 6 and 7 months after the operation with good quality of life.