Efficacy of non-pharmacological interventions to reduce pain in children with sickle cell disease: A systematic review.

Background: Pain is the clinical hallmark of sickle cell disease (SCD) leading to hospitalization, psychological sequelae and a decreased health-related quality of life. The aim of this systematic literature review is to evaluate the efficacy of non-pharmacological interventions in reducing sickle cell related pain in children with SCD.

Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, a comprehensive literature search up until October 2022 was performed to identify studies that investigated the efficacy of non-pharmacological interventions on (1) pain frequency and/or intensity, and (2) analgesic and health service use in children with SCD until the age of 21.

Measurement of post-transfusion red blood cell survival kinetics in sickle cell disease and β-Thalassemia: A biotin label approach.

Background: Red blood cell (RBC) transfusions are an important treatment modality for patients with sickle cell disease (SCD) and β-thalassemia. A subgroup of these patients relies on a chronic RBC transfusion regimen. Little is known about RBC survival (RCS) of the transfused allogeneic RBCs.

Extended phenotyping does not preclude the occurrence of delayed haemolytic transfusion reactions in sickle cell disease.

Delayed haemolytic transfusion reaction (DHTR) is a potentially life-threatening complication of red blood cell (RBC) transfusions in sickle cell disease (SCD) and is classically induced by reactivation of previously formed antibodies. Improved antigenic matching has reduced alloimmunization and may reduce DHTR risk. We conducted a retrospective cohort study to investigate the incidence rate of DHTR in SCD patients receiving extended matched units (ABO/RhDCcEe/K/Fy /Jk /S).

Retention and Transfer of Cognitive Bias Mitigation Interventions: A Systematic Literature Study.

Cognitive biases can adversely affect human judgment and decision making and should therefore preferably be mitigated, so that we can achieve our goals as effectively as possible. Hence, numerous bias mitigation interventions have been developed and evaluated. However, to be effective in practical situations beyond laboratory conditions, the bias mitigation effects of these interventions should be retained over time and should transfer across contexts.

Fluid overload due to intravenous fluid therapy for vaso-occlusive crisis in sickle cell disease: incidence and risk factors.

Intravenous fluid therapy (IV-FT) is routinely used in the treatment of vaso-occlusive crises (VOCs), as dehydration possibly promotes and sustains erythrocyte sickling. Patients with sickle cell disease (SCD) are at risk of developing diastolic dysfunction and fluid overload due to IV-FT. However, data on the adverse effects of IV-FT for VOC is sparse.

Back to base pairs: What is the genetic risk for red bloodcell alloimmunization?

Red blood cell (RBC) alloimmunization is a serious complication of blood transfusions, challenging selection of compatible units for future transfusions. Genetic characteristics may be associated with the risk of RBC alloimmunization and may therefore serve to identify high-risk patients. The aim of this systematic review was to summarize the available evidence on genetic risk factors for RBC alloimmunization.

Identification of genetic biomarkers for alloimmunization in sickle cell disease.

Most sickle cell disease (SCD) patients rely on blood transfusion as their main treatment strategy. However, frequent blood transfusion poses the risk of alloimmunization. On average, 30% of SCD patients will alloimmunize while other patient groups form antibodies less frequently.

Do Children and Adolescents With Inflammatory Bowel Disease Complete Clinical Disease Indices Similar to Physicians?

Objectives: The degree to which children and adolescents with inflammatory bowel disease (IBD) complete clinical disease activity indices in accordance with their physician is indefinite. Therefore, we investigated the agreement between patient- and physician-based clinical indices in children and adolescents with a previous diagnosis of IBD.

Methods: In this cross-sectional study, IBD patients (8-18 years) were included prospectively.

Isolated cortical vein thrombosis: systematic review of case reports and case series.

Background And Purpose: Isolated cortical vein thrombosis is a distinct subtype of cerebral venous and sinus thrombosis. Because of the rarity of isolated cortical vein thrombosis, limited knowledge on its clinical and radiological manifestations is available.

Methods: We performed a systematic review of published data.

Biorefining of wheat straw using an acetic and formic acid based organosolv fractionation process.

To assess the potential of acetic and formic acid organosolv fractionation of wheat straw as basis of an integral biorefinery concept, detailed knowledge on yield, composition and purity of the obtained streams is needed. Therefore, the process was performed, all fractions extensively characterized and the mass balance studied. Cellulose pulp yield was 48% of straw dry matter, while it was 21% and 27% for the lignin and hemicellulose-rich fractions.

Searching for microbial protein over-expression in a complex matrix using automated high throughput MS-based proteomics tools.

In the discovery of new enzymes genomic and cDNA expression libraries containing thousands of differential clones are generated to obtain biodiversity. These libraries need to be screened for the activity of interest. Removing so-called empty and redundant clones significantly reduces the size of these expression libraries and therefore speeds up new enzyme discovery.

Quantitative and qualitative analysis of haemoglobin variants using capillary zone electrophoresis.

Capillary zone electrophoresis (CZE) has been introduced into the clinical chemistry laboratory because of its range of potential applications. In this paper, we evaluate an alkaline CZE method for the quantification of HbA2 and HbF and also assess the combination of the alkaline CZE method with an acid CZE method for the determination of haemoglobin variants in an automated fashion. Correlation of the HbA2% determined between the HbA2-CZE method (alkaline conditions) and the Helena Sickle Thal Quick Column method was good (r = 0.

Production of inflammatory mediators and cytokine responsiveness of an SV40-transformed human proximal tubular epithelial cell line.

Proximal tubular epithelial cells (PTEC) play a central role in the physiology of the renal tubulointerstitium. To be able to study the relationship between tubular cells and inflammatory renal diseases the availability of cultured cells is of importance. This study describes an immortalized proximal tubular epithelial cell line which was generated using SV40 DNA.

Transforming growth factor-beta 1 regulates chemokine and complement production by human proximal tubular epithelial cells.

Previously it has been demonstrated that human proximal tubular epithelial cells (PTEC) are able to produce chemokines (such as IL-8 and MCP-1) and complement components (such as C2, C3, C4 and factor H), and that production of these proteins is regulated by pro-inflammatory cytokines such as interleukin-1 alpha (IL-1alpha), tumor necrosis factor-alpha (TNF-alpha) and interferon-gamma (IFN-gamma). Since TGF-beta is also expressed in the renal interstitium during inflammation, we investigated the effect of TGF-beta on the production of chemokines and complement components by PTEC in culture. Transforming growth factor-beta 1 up-regulated IL-8 production by an average of 4.

Expression of the IL-2 receptor on human renal proximal tubular epithelial cells.

The high-affinity receptor for interleukin (IL)-2, IL-2R, is composed of three chains, i.e., the alpha, beta, and gamma chains.

Interferon-gamma induces biosynthesis of complement components C2, C4 and factor H by human proximal tubular epithelial cells.

In a previous study the authors demonstrated that the production of complement component C4 by human kidney proximal tubular epithelial cells (PTEC) is upregulated by interferon gamma (IFN-gamma). In the present study the authors describe that PTEC in culture express both mRNA and protein of the IFN-gamma receptor complex, and that culture of PTEC with 1000 U/ml IFN-gamma for 72 h results in enhanced production not only of C4 (36.1 ng/10(6) cells), but also of C2 (10.

Possible role for CD40-CD40L in the regulation of interstitial infiltration in the kidney.

Interstitial infiltration by mononuclear cells is a hallmark of most inflammatory kidney diseases, and the degree of infiltration is associated with disease progression. It has been demonstrated that proximal tubular epithelial cells (PTEC) are an important source of different cytokines/chemokines and thereby play a central role in the regulation of the local inflammatory response. CD40 is a cell surface receptor involved in immune regulation for which the ligand is expressed on activated T cells.

Interleukin-1 alpha enhances the biosynthesis of complement C3 and factor B by human kidney proximal tubular epithelial cells in vitro.

Local production in tubular cells of complement has been shown to occur in several kidney diseases by in situ hybridization, but the regulation at the local site during an inflammation is still unknown. In the present study, we demonstrate that human proximal tubular epithelial cells (PTEC) are able to produce complement components C3 and Factor B under non-stimulated conditions in vitro. The basal production of both was increased by 0.

Increased urinary excretion of monocyte chemoattractant protein-1 during acute renal allograft rejection.

Background: Acute rejection is characterized histologically by infiltration of the interstitium by mononuclear cells. Monocyte chemoattractant protein 1 (MCP-1) has recently been identified as a monocyte chemotactic factor. This study examined the possible role of MCP-1 in renal transplantation.

Proteinase 3, the major autoantigen of Wegener's granulomatosis, enhances IL-8 production by endothelial cells in vitro.

Proteinase 3 is the major target antigen of antineutrophil cytoplasmic autoantibodies (ANCA) in Wegener's granulomatosis and is contained in the azurophilic granules of polymorphonuclear neutrophils, the dominant cell type in vascular lesions during the early stages of systemic vasculitis. This study questioned whether neutrophil lysosomal enzymes, once released at the site of inflammation, are able to potentiate the influx of additional neutrophils by enhancing the production of the chemotactic cytokine interleukin-8 (IL-8) by endothelial cells. Therefore, human umbilical vein endothelial cells in culture were incubated with varying concentrations of highly purified proteinase 3, human neutrophil elastase, and cathepsin G for different time periods.

Regulation and production of IL-8 by human proximal tubular epithelial cells in vitro.

A number of inflammatory kidney diseases are associated with interstitial nephritis and influx of leucocytes in the renal interstitium. Potentially the influx of neutrophils in the interstitium may be induced by the chemotactic cytokine IL-8. In the present study we have analysed the production of IL-8 by cultured human proximal tubular epithelial cells (PTEC) in response to a number of proinflammatory cytokines.

Problem-based learning for tuberculosis and leprosy supervisors.

A problem-based learning programme for tuberculosis and leprosy supervisors is reported from Nigeria. It proved stimulating, motivating and enjoyable, and encouraged independent learning. The quality of their subsequent field work was average to good and showed sustained motivation.

Production and cytokine-mediated regulation of monocyte chemoattractant protein-1 by human proximal tubular epithelial cells.

Impairment of renal function in various types of glomerular disease is associated with tubulointerstitial changes. The mechanism of mononuclear cell infiltration in the interstitium is not fully understood. Recently, monocyte chemoattractant protein-1 (MCP-1) has been identified as a monocyte-specific chemotactic factor.

Monocyte chemoattractant protein-1 in normal and diseased human kidneys: an immunohistochemical analysis.

We conducted an immunohistochemical analysis to investigate the presence of monocyte chemoattractant protein-1 (MCP-1) in normal and diseased human kidneys and the correlation with infiltration of macrophages. A total of 50 renal biopsies were classified according to pathologic diagnosis. The distribution as well as the intensity of MCP-1 staining, and infiltration by CD68+ macrophages were evaluated in diseased versus normal kidneys.