Influence of the wind profile on the initiation of convection in mountainous terrain

A number of days with small precipitating convective cells were investigated using weather radars during the COPS (Convective and Orographically-induced Precipitation Study) field campaign in the region of the Vosges and the Rhine Valley in Central Europe. Depending on the weather situation, two distinct mechanisms could be identified for the initiation of convection.On some days, cells were initiated over the ridge of theVosges, whereas on other days cells were initiated in the lee of the Vosges. The initiation of convection appeared to be concentrated in a few favourable locations. Using the Froude number, it was possible to describe the two distinct mechanisms. When the Froude number was low, the flow was diverted around the Vosges and thermally driven convergence at the ridge initiated convection, whereas when the Froude number was high, the flow passed through mountain gaps and then converged on the lee side with the flow in the Rhine Valley. The convergence on the lee side was enhanced at locations where the outflows through valleys converged. Low Froude numbers were accompanied by weak winds varying with height,whereas high Froude numbers were observed during situations with stronger southwesterly winds increasing with height

Somatostatin and substance P analogues: applications in autoimmune and haematological diseases

Somatostatin has been extensively studied in relation to the endocrine and nervous systems. Many reports on the role of somatostatin receptor imaging and somatostatin treatment of neuroendocrine tumours have been published. The relation between somatostatin and other neuropeptides and the immune system is less explored. The aim of this study was to investigate the diagnostic applications of somatostatin and substance P analogues in autoimmune and haematological diseases. Both in vivo and in vitro studies were performed, using peptide receptor scintigraphy in patients and rats, peptide receptor autoradiography on tissue biopsies, ligand binding assays on cell homogenates, and polymerase chain reactions on lymphoid cell lines. Moreover, based on the results of these studies, speculations were made about the therapeutical applications of somatostatin and substance P analogues in autoimmune and haematological diseases

Do synovial biopsies help to support evidence for involvement of innate immunity in the immunopathology of Behçet's disease?

Behçet's disease is a complex vasculitis of unknown etiology. Abundant neutrophils suggest the involvement of innate immunity. Cytokines are skewed to the T-helper-1 pattern. Few sterile organs are easily accessible for analysis in Behçet's disease. Cañete and coworkers identify inflamed joints as a feasible model and suggest the involvement of innate immunity in Behçet's disease

Do synovial biopsies help to support evidence for involvement of innate immunity in the immunopathology of Behçet's disease?

Behçet's disease is a complex vasculitis of unknown etiology. Abundant neutrophils suggest the involvement of innate immunity. Cytokines are skewed to the T-helper-1 pattern. Few sterile organs are easily accessible for analysis in Behçet's disease. Cañete and coworkers identify inflamed joints as a feasible model and suggest the involvement of innate immunity in Behçet's disease

Does competition improve hospital performance:a DEA based evaluation from the Netherlands

Many countries have introduced competition among hospitals aiming to improve their performance. We evaluate the introduction of competition among hospitals in the Netherlands over the years 2008–2015. The analysis is based on a unique longitudinal data set covering all Dutch hospitals and health insurers, as well as demographic and geographic data. We measure hospital performance using Data Envelopment Analysis and distinguish three components of competition: the fraction of freely negotiated services, market power of hospitals, and insurer bargaining power. We present new methods to define variables for each of these components which are more accurate than previously developed measures. In a multivariate regression analysis, the variables explain more than half of the variance in hospital efficiency. The results indicate that competition between hospitals and the relative fraction of freely negotiable health services are positively related to hospital efficiency. At the same time, the policy measure to steadily increase the fraction of health services contracted in competition may well have resulted in a decrease in hospital efficiency. The models show no significant association between insurer bargaining power and hospital efficiency. Altogether, the results offer little evidence that the introduction of competition for hospital care in the Netherlands has been effective

Reviewing primary Sjögren’s syndrome: Beyond the dryness - From pathophysiology to diagnosis and treatment

Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx and/or vagina. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, pulmonary, renal, hematological and neurological involvement. The pathogenesis of pSS is currently not well understood, but increased activation of B cells followed by immune complex formation and autoantibody production are thought to play important roles. pSS is diagnosed using the American-European consensus group (AECG) classification criteria which include subjective symptoms and objective tests such as histopathology and serology. The treatment of pSS warrants an organ based approach,

An inflammatory condition with different faces: Immunoglobulin G4-Related disease

__Background:__ Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with involvement of different organs. The pathophysiological mechanism is unclear, but fibrosis is the hallmark of this disease. Early recognition is critical to avoid irreversible organ damage. Recently improved histological testing boosts the diagnostic yield. We present three cases of patients with IgG4-RD to emphasise the broad clinical presentation of this disease. __Case descriptions:__ Patient A, a 63-year-old male with bilateral orbital swelling due to IgG4-RD, was shown to suffer from IgG4-RD in a multifocal pattern as demonstrated by PET scanning. Patient B, a 53-year-old male with a long-standing abdominal mass of unknown origin, eventually proved to have IgG4-RD. Patient C was a 32-year-old male admitted with pleural effusion and pericardial tamponade. Histological diagnosis after pericardiectomy confirmed IgG4-RD. __Discussion:__ IgG4-RD has many faces and may mimic other conditions, such as malignancy and infectious diseases. Knowledge of this disease is needed to avoid unnecessary diagnostics and delay in treatment. IgG4-RD may be suspected based on specific clinical findings such as elevated serum IgG4 levels, but the diagnosis can only be established histologically. Although corticosteroids are an effective first choice of therapy, the relapse rate after this treatment remains high. The role of disease-modifying antirheumatic drugs in the treatment of IgG4-RD has not been outlined yet, but there is increasing evidence that rituximab might be an effective second-line therapy. __Conclusion:__ IgG4-RD is a disease with many faces requiring early recognition and therapy to avoid permanent damage of the organs

The 11q Terminal Deletion Disorder Jacobsen Syndrome is a Syndromic Primary Immunodeficiency

Background: Jacobsen syndrome (JS) is a rare contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. Clinical features include physical and mental growth retardation, facial dysmorphism, thrombocytopenia, impaired platelet function and pancytopenia. In case reports, recurrent infections and impaired immune cell function compatible with immunodeficiency were described. However, Jacobsen syndrome has not been recognized as an established syndromic primary immunodeficiency. Goal: To evaluate the presence of immunodeficiency in a series of 6 patients with JS. Methods: Medical history of 6 patients with JS was evaluated for recurrent infections. IgG, IgA, IgM and specific antibodies against S. pneumoniae were measured. Response to immunization with a polysaccharide vaccine (Pneumovax) was measured and B and T lymphocyte subset analyses were performed using flowcytometry. Results: Five out of 6 patients suffered from recurrent infections. These patients had low IgG levels and impaired response to S. pneumoniae polysaccharide vaccination. Moreover, we also found a significant decrease in the absolute number of memory B cells, suggesting a defective germinal center function. In a number of patients, low numbers of T lymphocytes and NK cells were found. Conclusions: Most patients with JS suffer from combined immunodeficiency in the presence of recurrent infections. Therefore, we consider JS a syndromic primary immunodeficiency. Early detection of immunodeficiency may reduce the frequency and severity of infections. All JS patients should therefore undergo immunological evaluation. Future studies in a larger cohort of patients will more precisely define the pathophysiology of the immunodeficiency in JS