Short Root Anomaly and Transverse Hemimelia: A Rare Case Presentation.

A case of Short Root Anomaly (SRA), hypodontia and taurodontism with the associated finding of transverse hemimelia, is presented. Although there is no documented evidence of a link between SRA and hemimelia, the shared genetic, environmental and metabolic factors suggest the possibility of a common underlying mechanism.

Efficacy and Safety of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis With and Without Fever at Baseline: Results From an Open-Label, Active-Treatment Extension Study.

Objective: To evaluate the long-term efficacy and safety of canakinumab and explore prediction of response in patients with systemic juvenile idiopathic arthritis (JIA) with or without fever at treatment initiation.

Methods: At enrollment, patients with active systemic JIA (ages 2 to <20 years) started open-label canakinumab (4 mg/kg every 4 weeks subcutaneously). Efficacy measures included the adapted American College of Rheumatology (ACR) Pediatric 50/70/90 criteria, the Juvenile Arthritis Disease Activity Score (JADAS), and clinically inactive disease and clinical remission on medication, evaluated by either the JADAS or ACR criteria.

Mother/child organophosphate and pyrethroid distributions.

The present study reports one of the few cases in which organophosphate (OP) and pyrethroid (PYR) pesticide human exposure is evaluated in family contexts by the analysis of mother/child pair samples. Urinary concentrations of 6 organic metabolites of organophosphates and 2 pyrethroids were measured in mothers and their 7-to 8-year-old children (n = 168) in a general population from the central area of Slovenia. The results were adjusted for specific gravity and creatinine.

Surface characterization of monolithic zirconia submitted to different surface treatments applying optical interferometry and raman spectrometry.

This study evaluated roughness parameters and phase transformation of monolithic zirconia ceramics after various mechanical and laser thermal treatments. Fully sintered monolithic zirconia cylinder specimens were divided to five groups, according to the applied surface treatment: CL: control, GB: grit-blasted with glass particles. AL50: grit-blasted with 50 μm alumina particles, AL90: grit-blasted with 90 μm dyed-alumina particles and FEML: subjected to femto laser thermal treatment.

Safety and Effectiveness of Adalimumab in Patients With Polyarticular Course of Juvenile Idiopathic Arthritis: STRIVE Registry Seven-Year Interim Results.

Objective: To evaluate safety and effectiveness of adalimumab (ADA) in polyarticular-course juvenile idiopathic arthritis (JIA) in the STRIVE registry.

Methods: STRIVE enrolled patients with polyarticular-course JIA into 2 arms based on treatment with methotrexate (MTX) alone or ADA with/without MTX (ADA ± MTX). Adverse events (AEs) per 100 patient-years of observation time were analyzed by registry arm.

Importance of the relationship between symptoms and self-reported physical activity level in stable COPD based on the results from the SPACE study.

Background: The burden of symptoms and risk of exacerbations are the main drivers of the overall assessment of the Chronic Obstructive Pulmonary Disease (COPD) and the adequate treatment approaches per current Global Initiative for Chronic Obstructive Lung Disease (GOLD). Physical activity has emerged as both functional outcome and non-pharmacological intervention in COPD patients, despite the lack of standardized measures or guidelines in clinical practice. This study aimed to explore in more depth the 24-h respiratory symptoms, the physical activity level (PAL) and the relationship between these two determinants in stable COPD patients.

Circulating anti-phospholipase A2 receptor antibodies as a diagnostic and prognostic marker in Greek patients with idiopathic membranous nephropathy - a retrospective cohort study.

Introduction: Circulating autoantibodies against phospholipase A2 receptor (anti-PLA2R) are recognized as key elements in the pathogenesis of idiopathic membranous nephropathy. In current clinical practice, they are increasingly gaining attention as novel tools for diagnosis and disease monitoring. We investigated the diagnostic and prognostic utility of anti-PLA2R antibody measurements in Greek patients with biopsy-proven membranous nephropathy.

Phenotypic variability and disparities in treatment and outcomes of childhood arthritis throughout the world: an observational cohort study.

Background: To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status.

Methods: In this multinational, cross-sectional, observational cohort study, we asked international paediatric rheumatologists from specialised centres to enrol children with a diagnosis of juvenile idiopathic arthritis, according to International League of Associations for Rheumatology criteria, who were seen consecutively for a period of 6 months.

Pharmacovigilance in juvenile idiopathic arthritis patients treated with biologic or synthetic drugs: combined data of more than 15,000 patients from Pharmachild and national registries.

Background: The availability of methotrexate and the introduction of multiple biological agents have revolutionized the treatment of juvenile idiopathic arthritis (JIA). Several international and national drug registries have been implemented to accurately monitor the long-term safety/efficacy of these agents. This report aims to present the combined data coming from Pharmachild/PRINTO registry and the national registries from Germany (BiKeR) and Sweden.

Hemorrhagic pneumonia in neonatal minks in Greece concomitant with Leismania infantum detection.

In the present study a severe outbreak of hemorrhagic pneumonia (HP) in neonatal minks concomitant with Leismania infantum (L. infantum) detection is reported. The outbreak took place on a Greek mink farm and affected 1,362 mink kits, with 524 dying.

The clinical and genetic heterogeneity of paroxysmal dyskinesias.

Paroxysmal dyskinesia can be subdivided into three clinical syndromes: paroxysmal kinesigenic dyskinesia or choreoathetosis, paroxysmal exercise-induced dyskinesia, and paroxysmal non-kinesigenic dyskinesia. Each subtype is associated with the known causative genes PRRT2, SLC2A1 and PNKD, respectively. Although separate screening studies have been carried out on each of the paroxysmal dyskinesia genes, to date there has been no large study across all genes in these disorders and little is known about the pathogenic mechanisms.

The PROTAGORAS study to evaluate the performance of the Endurant stent graft for patients with pararenal pathologic processes treated by the chimney/snorkel endovascular technique.

Objective: The chimney/snorkel endovascular aortic repair (ch-EVAR) is gaining ever-greater acceptance in the treatment of pararenal pathologic processes. However, the published experience includes mainly short-term clinical results with combinations of several abdominal devices and types of chimney grafts. The aim of this study was the midterm evaluation of the Endurant stent graft (Medtronic, Santa Rosa, Calif) as a standard abdominal device for ch-EVAR.

Impact of capsaicin on mast cell inflammation.

Mast cells are inflammatory cells, and they are prominent in inflammatory diseases such as allergy and asthma. Mast cells possess high-affinity receptors for IgE (FcERI) and the cross-linking of these receptors is essential to trigger the secretion of granules containing arachidonic acid metabolism (such as prostaglandin (PG) D2, leukotriene (LT) B4, and LTC4), histamine, cytokines, chemokines, and proteases, including mast cell-specific chymases and tryptases. Activation of mast cells provokes the secretion of cytokines and mediators that are responsible for the pathologic reaction of immediate hypersensitivity.

Treatment of intravenous drug users with chronic hepatitis C: treatment response, compliance and side effects.

Background: Although intravenous drug users (IVDUs) comprise the majority of patients with chronic hepatitis C, most of them are excluded from treatment because of concerns about adherence to treatment and side effects.

Material And Methods: In this study we retrospectively evaluated safety, compliance to treatment and efficacy of treatment in IVDUs with HCV infection in 163 former IVDUs with chronic hepatitis C, who were not in methadone substitution and were attending our clinics the period 1997-2004. All subjects were HCVRNA (+), had ALT levels>x1.

Long-term signal detection, segmentation and summarization using wavelets and fractal dimension: a bioacoustics application in gastrointestinal-motility monitoring.

The current paper describes a wavelet-based method for long-term processing and analysis of gastrointestinal sounds (GIS). Windowing techniques are used to select sequential blocks of the prolonged multi-channel recordings and proceed to various wavelet-domain processing stages. De-noising, significant-activity detection, automated segmentation and extraction of summary curves are applied in an integrated mode, allowing for enhanced content manipulation and analysis.

Long-term results of triple arthrodesis: 42 cases followed for 25 years.

We reviewed 42 patients who had had triple arthrodesis 25 years after surgery. The patients' age averaged 20 years. All patients had deformities due to poliomyelitis.