Cranial structure and condylar asymmetry of patients with juvenile idiopathic arthritis: a risky growth pattern

The aim of the study was to evaluate the cephalometric differences and condylar asymmetry between patients with juvenile idiopathic arthritis (JIA) and normal control group. Sixty-two JIA patients with a latero-lateral cephalogram and orthopantomography, seeking for orthodontic therapy, and 62 normal matched subjects were comprised in the study. Cephalometric analysis was used for the evaluation of facial morphology while the method of Habets et al. (J Oral Rehabil 15(5): 465-471, 1988) was used to compare the condyles in orthopantomography. The significance of between-group differences was assessed using the Mann-Whitney test, as appropriate. The results showed a prevalence of the upper maxilla with hypomandibulia (class II), hyperdivergency with short vertical ramus posterior and posterior rotation of the mandible in JIA children (SNB, ANB, NSL/ML, Fh/ML, NL/ML, ArGo, ML P < 0.0001, ML/Oc P < 0.004, ArGo/GoGn P = 0.02, no difference for SNA). The condyles of the JIA group resulted highly asymmetric (P < 0.0001). The growth pattern of JIA patients resulted clearly different from normal subjects. This serious impairment of the cranial growth may be considered as an indicator of the need for early and continuous orthognatodonthic therapy during the entire period of development for all JIA patients, independently from temporomandibular joint signs or symptoms. To this end, it is important that rheumatologists and orthognathodontists set up a multidisciplinary treatment planned to control the side effects of a deranged growing pattern, to strictly avoid any orthodontic therapies that may worsen function and growth, and to promote treatments improving the physiology and biology of the cranial development

Development of high-resolution infrared thermographic imaging method as a diagnostic tool for acute undifferentiated limp in young children

Acute limp is a common presenting condition in the paediatric emergency department. There are a number of causes of acute limp that include traumatic injury, infection and malignancy. These causes in young children are not easily distinguished. In this pilot study, an infrared thermographic imaging technique to diagnose acute undifferentiated limp in young children was developed. Following required ethics approval, 30 children (mean age = 5.2 years, standard deviation = 3.3 years) were recruited. The exposed lower limbs of participants were imaged using a high-resolution thermal camera. Using predefined regions of interest (ROI), any skin surface temperature difference between the healthy and affected legs was statistically analysed, with the aim of identifying limp. In all examined ROIs, the median skin surface temperature for the affected limb was higher than that of the healthy limb. The small sample size recruited for each group, however, meant that the statistical tests of significant difference need to be interpreted in this context. Thermal imaging showed potential in helping with the diagnosis of acute limp in children. Repeating a similar study with a larger sample size will be beneficial to establish reproducibility of the results

Anxiety and depression in keratotic oral lichen planus: a multicentric study from the SIPMO

Objectives: Oral lichen planus with exclusive keratotic reticular, papular, and/or plaque-like lesions (K-OLP) is a clinical pattern of OLP that may be associated with a complex symptomatology and psychological alteration. The aim of the study was to evaluate the prevalence of anxiety (A) and depression (D) in patients with K-OLP, analyzing the potential predictors which can affect mental health status. Methods: Three hundred K-OLP patients versus 300 healthy controls (HC) were recruited in 15 Italian universities. The Numeric Rating Scale (NRS), Total Pain Rating Index (T-PRI), and Hamilton Rating Scales for Depression and for Anxiety (HAM-D and HAM-A) were administered. Results: The K-OLP patients showed statistically higher scores in the NRS, T-PRI, HAM-D, and HAM-A compared with the HC (p-value < 0.001**). A and D were found in 158 (52.7%) and 148 (49.3%) K-OLP patients. Strong linear correlations were identified between HAM-A, HAM-D, NRS, T-PRI, and employment status and between HAM-D, HAM-A, NRS, T-PRI, employment status, and female gender. Multivariate logistic regression revealed that HAM-D and HAM-A showed the greatest increase in the R2 value for A and D in the K-OLP patients, respectively (DR2 = 55.5% p-value < 0.001**; DR2 = 56.5% p-value < 0.001**). Conclusions: The prevalence of A and D is higher in the K-OLP patients compared with the HC, also found in K-OLP subjects without pain, suggesting that the processing of pain may be in a certain way independent of the processing of mood. Clinical relevance: Mood disorders and pain assessment should be carefully performed in relation to K-OLP to obtain a complete analysis of the patients

The European Hematology Association Roadmap for European Hematology Research: a consensus document

The European Hematology Association (EHA) Roadmap for European Hematology Research highlights major achievements in diagnosis and treatment of blood disorders and identifies the greatest unmet clinical and scientific needs in those areas to enable better funded, more focused European hematology research. Initiated by the EHA, around 300 experts contributed to the consensus document, which will help European policy makers, research funders, research organizations, researchers, and patient groups make better informed decisions on hematology research. It also aims to raise public awareness of the burden of blood disorders on European society, which purely in economic terms is estimated at €23 billion per year, a level of cost that is not matched in current European hematology research funding. In recent decades, hematology research has improved our fundamental understanding of the biology of blood disorders, and has improved diagnostics and treatments, sometimes in revolutionary ways. This progress highlights the potential of focused basic research programs such as this EHA Roadmap. The EHA Roadmap identifies nine ‘sections’ in hematology: normal hematopoiesis, malignant lymphoid and myeloid diseases, anemias and related diseases, platelet disorders, blood coagulation and hemostatic disorders, transfusion medicine, infections in hematology, and hematopoietic stem cell transplantation. These sections span 60 smaller groups of diseases or disorders. The EHA Roadmap identifies priorities and needs across the field of hematology, including those to develop targeted therapies based on genomic profiling and chemical biology, to eradicate minimal residual malignant disease, and to develop cellular immunotherapies, combination treatments, gene therapies, hematopoietic stem cell treatments, and treatments that are better tolerated by elderly patients

Budget impact analysis of treatments for hepatitis C virus: what's new?

4-8 November 201

Updates on Novel Non-Replacement Drugs for Hemophilia

Over the last decade, the world of hemophilia has experienced an unprecedented therapeutic advance, thanks to the progress in bioengineering technologies, leading to the introduction of drugs with novel mechanisms of action based on restoring thrombin generation or coagulation factor VIII mimicking. Apart from the bispecific monoclonal antibody emicizumab, already approved for patients with severe hemophilia A with and without inhibitors, novel non-replacement drugs designed to reduce the treatment burden of patients with hemophilia A or B with or without inhibitors are undergoing evaluation in clinical trials. Thanks to their innovative mechanism of action and subcutaneous administration, these drugs promise to provide effective bleeding protection together with improved adherence and improve health-related quality of life for patients with hemophilia. On the other hand, rare thromboembolic events have been reported with some of these drugs and warrant continuous post-marketing surveillance and investigation of predisposing factors, although the overall safety profile of most of these drugs is good. Finally, new challenges need to be faced in the clinical and laboratory monitoring of the hemostatic status in patients treated with these innovative therapies. In this review, we provide an update on the available data on novel non-replacement drugs currently undergoing evaluation in clinical trials for patients with hemophilia

Myelo peroxidase as an Indicator of Oxidative Stress in Metabolic Syndrome

Background: Increased myeloperoxidase (MPO) activity would be the link between the rise of the inflammatory response and oxidative stress (OS) in metabolic syndrome (MS). Objective: The aim of this study was to determine the enzymatic activity of MPO associated with OS in animals with MS and establish their relationship with probable cardiovascular injury. Methods: Male Wistar rats were divided into two groups: Group A, control (n=12) and Group B, induced MS (n=12). Metabolic syndrome was produced by 6-week administration of 10% fructose diluted in the drinking water. Insulin (mU/ml), glucose (mg/dl), lipid panel (mg/dl), HOMA (homeostatic model assessment), MPO (IU/ml) and superoxide dismutase (SOD) activity (U/ml) were measured. Light microscopy was used for the histological study of the heart and thoracic aorta. Results: Group B showed significantly increased levels of plasma glucose (176±17.3 mg/dl), insulin (29.5±4.52 mU/ml), HOMA (11±1.3), total cholesterol (133±9.6 mg/dl) and triglycerides (75±12.9 mg/dl) compared with Group A: plasma glucose (115±1.1 mg/dl), insulin (4±0.82 mU/ml), HOMA (3±0.38), total cholesterol (69.7±1.6 mg/dl) and triglycerides (46.2±6 mg/dl), (p<0.001 for all variables). A significant decrease in HDL (28.3±1.14 mg/dl) in Group B vs. Group A (61±1.0 mg/dl) (p<0.001) validated the experimental MS model. Myeloperoxidase activity increased significantly in Group B (181.3±15.7 IU/ml) vs. Group A (116.07±4.2 IU/ml) (p<0.001). A similar behavior was seen with SOD antioxidant activity in Group B (181±6 U/ml) vs. Group A (138±3.6 U/ml) (p <0.01). Light microscopy of the heart and thoracic aorta revealed histopathological changes in animals with induced MS. Conclusion: Increased MPO and SOD in Group B would indicate the presence of OS in MS, with consequences at the vascular level.Introducción: Los incrementos en la actividad de la mieloperoxidasa (MPO) serían el nexo entre el aumento de la respuesta inflamatoria y el estrés oxidativo (EO) en el síndrome metabólico (SM). Objetivo: Determinar la actividad de la enzima MPO asociada con EO en animales con SM y establecer su relación con las probables lesiones cardiovasculares. Material y métodos: Se utilizaron ratas macho de la cepa Wistar, que se dividieron en: Grupo A, control (n = 12) y Grupo B, inducción de SM (n = 12). El SM se indujo con la administración de fructosa al 10% diluida en agua de bebida durante 6 semanas. Se cuantificaron insulina (mU/ml), glucemia (mg/dl), perfil lipídico (mg/dl), HOMA (homeostatic model assessment), MPO (UI/ml) y actividad de la superóxido dismutasa (SOD) (U/ml). Se estudió la histología de la aorta torácica y el corazón por microscopia óptica. Resultados: Se observaron niveles de glucemia (176 ± 17,3 mg/dl), insulina (29,5 ± 4,52 mU/ml), HOMA (11 ± 1,3), colesterol total (133 ± 9,6 mg/dl) y triglicéridos (75 ± 12,9 mg/dl) incrementados significativamente en el Grupo B en comparación con el Grupo A: glucemia (115 ± 1,1 mg/dl), insulina (4 ± 0,82 mU/ml), HOMA (3 ± 0,38), colesterol total (69,7 ± 1,6 mg/dl) y triglicéridos (46,2 ± 6 mg/dl) (p < 0,001 para todas las variables); se verificó disminución significativa de los valores de HDL (28,3 ± 1,14 mg/dl) en el Grupo B en comparación con el Grupo A (61 ± 1,01 mg/dl) (p < 0,001), validando así el modelo experimental de SM. La actividad de la MPO se incrementó significativamente en el Grupo B (181,3 ± 15,7 UI/ml) respecto del Grupo A (116,07 ± 4,2 UI/ml) (p < 0,001). Similar comportamiento presentó la actividad antioxidante de la SOD en el Grupo B (181 ± 6 U/ml) respecto del Grupo A (138 ± 3,6 U/ml) (p < 0,01). Las microscopias ópticas de corazón y de aorta torácica evidenciaron cambios histopatológicos en los animales con SM inducido. Conclusión: Los incrementos de la MPO y la SOD en el Grupo B demostrarían la presencia de EO, con repercusión a nivel vascular en el SM