Ionic Bent-Core Pillar[]arenes: From Liquid Crystals to Nanoaggregates and Functional Applications.

Herein, we report the first examples of supramolecular systems from bent-core-based pillar[]arenes through ionic bonds. These ionic materials have been prepared by the interaction of an amino-ended pillar[5]arene (P5N10) and three different carboxylic acids, including bent-core moieties. The bent-core units are based on ester, biphenyl, and azobenzene structures bearing two different flexible spacers between the carboxyl group and the central bent-core aromatic units.

Medication Optimization Using Pharmacogenomic Testing in a Complex Mental Health Population Prescribed Psychiatric Polypharmacy.

The use of polypharmacy has become significantly more common over the past two decades, increasing the risk of drug-drug interactions and adverse drug reactions. Pharmacogenomic (PGx) assays have the purported benefit of being able to predict an individual's response to a specific medication based on genetic markers, which may facilitate the development of optimized medication regimens for patients prescribed polypharmacy. This 12-week pilot study examined the impact of the PGx results on the clinical management of Veterans who were prescribed psychiatric polypharmacy.

Suitability of strontium and cobalt-free perovskite cathodes with LaSiAlO apatite electrolyte for intermediate temperature solid oxide fuel cells.

Aluminium-doped lanthanum silicate (LSAO) apatite-type compounds have been considered as promising candidates for substituting yttria-stabilized zirconia (YSZ) as electrolytes for intermediate temperature solid oxide fuel cells (IT-SOFC). Nevertheless, not many materials have been reported to work as cathodes in a LSAO apatite-based cell. In the present work, eight different strontium and cobalt-free compounds with a perovskite-type structure and the general composition LaM1-xNxO3-δ (where M = Fe, Cr, Mn; N = Cu, Ni; and x = 0.

Measuring Membrane Permeation Rates through the Optical Visualization of a Single Pore.

Membranes are a critical technology for energy-efficient separation processes. The routine method of evaluating membrane performance is a permeation measurement. However, such measurements can be limited in terms of their utility: membrane microstructure is often poorly characterized; membranes or sealants leak; and conditions in the gas phase are poorly controlled and frequently far-removed from the conditions employed in the majority of real processes.

Biallelic loss-of-function LACC1/FAMIN Mutations Presenting as Rheumatoid Factor-Negative Polyarticular Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) is a complex rheumatic disease with both autoimmune and autoinflammatory components. Recently, familial cases of systemic-onset JIA have been attributed to mutations in LACC1/FAMIN. We describe three affected siblings from a Moroccan consanguineous family with an early-onset chronic, symmetric and erosive arthritis previously diagnosed as rheumatoid factor (RF)-negative polyarticular JIA.

S100A12 and S100A8/9 proteins are biomarkers of articular disease activity in Blau syndrome.

Objective: To identify biomarkers of articular and ocular disease activity in patients with Blau syndrome (BS).

Methods: Multiplex plasma protein arrays were performed in five BS patients and eight normal healthy volunteers (NHVs). Plasma S100A12 and S100A8/9 were subsequently measured by ELISA at baseline and 1-year follow-up in all patients from a prospective multicentre cohort study.

Development and Initial Validation of the Macrophage Activation Syndrome/Primary Hemophagocytic Lymphohistiocytosis Score, a Diagnostic Tool that Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome.

Objective: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis.

Study Design: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample.

Efficacy and safety of canakinumab in cryopyrin-associated periodic syndromes: results from a Spanish cohort.

Objectives: Cryopyrin-associated periodic syndromes (CAPS) are dominantly-inherited autoinflammatory diseases. The uncontrolled IL-1β overproduction observed in these patients is the rational basis to treat them with anti-IL-1 drugs. The objective of this study was to evaluate the efficacy and safety of treatment with the long-lasting fully humanised anti-IL-1β monoclonal antibody canakinumab in a Spanish cohort of patients with CAPS.

Consensus of the Spanish society of pediatric rheumatology for transition management from pediatric to adult care in rheumatic patients with childhood onset.

To develop recommendations on the transition from pediatric care to adult care in patients with chronic inflammatory rheumatic diseases with childhood onset based. Recommendations were generated following nominal group methodology and Delphi technique. A panel of 16 experts was established.

Clinical management algorithm of uveitis associated with juvenile idiopathic arthritis: interdisciplinary panel consensus.

Uveitis associated with juvenile idiopathic arthritis (JIA) typically involves the anterior chamber segment, follows an indolent chronic course, and presents a high rate of uveitic complications and a worse outcome as compared to other aetiologies of uveitis. Disease assessment, treatment, and outcome measures have not been standardized. Collaboration between pediatric rheumatologists and ophthalmologists is critical for effective management and prevention of morbidity, impaired vision, and irreparable visual loss.

Blau syndrome: cross-sectional data from a multicentre study of clinical, radiological and functional outcomes.

Objective: To report baseline articular, functional and ocular findings of the first international prospective cohort study of Blau syndrome (BS).

Methods: Three-year, multicentre, observational study on articular, functional (HAQ, Childhood HAQ and VAS global and pain), ophthalmological, therapeutic and radiological data in BS patients.

Results: Baseline data on the first 31 recruited patients (12 females and 19 males) from 18 centres in 11 countries are presented.

Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients.

Objective: To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA).

Methods: In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected retrospectively into a web-based database.

Results: A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries.

Detection of synovitis by ultrasonography in clinically inactive juvenile idiopathic arthritis on and off medication.

Objectives: To determine the prevalence of abnormalities detected by ultrasonography (US) in children with juvenile idiopathic arthritis (JIA) showing clinically inactive disease (ID) on medication and off medication.

Inclusion Criteria: 1) JIA patients, 2) clinician-determined ID, 3) JIA drugs withdrawal or stably dosed modified anti-rheumatic drugs (DMARDs) therapy for at least 6 months prior to inclusion, 4) biologics naïve patients. Clinical and US assessments were performed on 44 joints, which were scored for grey-scale (GS) synovitis and Power Doppler (PD) signal.

Adalimumab therapy for refractory childhood uveitis.

Purpose: To report the results of adalimumab therapy in a cohort of children with refractory noninfectious uveitis.

Methods: The medical records of patients diagnosed with uveitis and treated with biweekly adalimumab injections for a period of at least 3 months at the University Hospital of La Paz from 2007-2012 were retrospectively reviewed. Improvement in inflammatory activity was graded according to grading schema of the Standardization of Uveitis Nomenclature Working Group.

Sedation for intra-articular corticosteroid injections in juvenile idiopathic arthritis: the views of patients and their parents.

Unlabelled: Intra-articular corticosteroid injections (IACI) are one of the mainstays of treatment for children with juvenile idiopathic arthritis. The most important disadvantage of IACI is the pain associated with the procedure. Little is known about the children or parents' perception of this pain.

Reduced joint assessment vs comprehensive assessment for ultrasound detection of synovitis in juvenile idiopathic arthritis.

Objective: To propose a reduced joint power Doppler US (PDUS) assessment and provide preliminary evidence of its validity, feasibility, reliability and sensitivity to change compared with a comprehensive (i.e. 44 joints) PDUS assessment in evaluating synovitis in JIA.

Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis.

Background: Systemic juvenile idiopathic arthritis (JIA) is the most severe subtype of JIA; treatment options are limited. Interleukin-6 plays a pathogenic role in systemic JIA.

Methods: We randomly assigned 112 children, 2 to 17 years of age, with active systemic JIA (duration of ≥6 months and inadequate responses to nonsteroidal antiinflammatory drugs and glucocorticoids) to the anti-interleukin-6 receptor antibody tocilizumab (at a dose of 8 mg per kilogram of body weight if the weight was ≥30 kg or 12 mg per kilogram if the weight was <30 kg) or placebo given intravenously every 2 weeks during the 12-week, double-blind phase.

Interacting plasmon and phonon polaritons in aligned nano- and microwires.

The availability of macroscopic, nearly periodic structures known as eutectics opens a new path for controlling light at wavelength scales determined by the geometrical parameters of these materials and the intrinsic properties of their component phases. Here, we analyze the optical waveguiding properties of eutectic mixtures of alkali halides, formed by close-packed arrangements of aligned cylindrical inclusions. The wavelengths of phonon polaritons in these constituents are conveniently situated in the infrared and are slightly larger than the diameter and separation of the inclusions, typically consisting on single-crystal wires down to submicrometer diameter.

High incidence of NLRP3 somatic mosaicism in patients with chronic infantile neurologic, cutaneous, articular syndrome: results of an International Multicenter Collaborative Study.

Objective: Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome, also known as neonatal-onset multisystem inflammatory disease (NOMID), is a dominantly inherited systemic autoinflammatory disease. Although heterozygous germline gain-of-function NLRP3 mutations are a known cause of this disease, conventional genetic analyses fail to detect disease-causing mutations in ∼40% of patients. Since somatic NLRP3 mosaicism has been detected in several mutation-negative NOMID/CINCA syndrome patients, we undertook this study to determine the precise contribution of somatic NLRP3 mosaicism to the etiology of NOMID/CINCA syndrome.

Broadband laser tunability of Nd3+ ions in 0.8CaSiO3-0.2Ca3(PO4)2 eutectic glass.

Site-selective spectroscopy and stimulated emission experiments performed in the (4)F(3/2)-->(4)I(11/2) laser transition of Nd(3+)-doped 0.8CaSiO(3-) 0.2Ca(3)(PO(4))(2) eutectic glass are presented.