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Objective To develop and validate new Juvenile Arthritis Disease Activity Score 10 (JADAS10) and clinical JADAS10 (cJADAS10) cutoffs to separate the states of inactive disease (ID), minimal disease activity (MiDA), moderate disease activity (MoDA), and high disease activity (HDA) in children with oligoarthritis and with rheumatoid factor-negative polyarthritis, based on subjective disease assessment by the treating pediatric rheumatologist. Methods The cutoffs definition cohort was composed of 1,936 patients included in the multinational Epidemiology, Treatment and Outcome of Childhood Arthritis (EPOCA) study. Using the subjective physician rating as an external criterion, 4 methods were applied to identify the cutoffs: mapping, Youden index, 90% specificity, and maximum agreement. The validation cohort included 4,014 EPOCA patients, patients from 2 randomized trials, and 88 patients from the PharmaChild registry. Cutoff validation was conducted by assessing discriminative and predictive ability. Results The JADAS10 cutoffs were 1.4, 4, and 13, respectively, for oligoarthritis and 2.7, 6, and 17, respectively, for polyarthritis. The cJADAS10 cutoffs were 1.1, 4, and 12, respectively, for oligoarthritis and 2.5, 5, and 16, respectively, for polyarthritis. The cutoffs discriminated strongly among different levels of pain and morning stiffness, between patients who were and those who were not prescribed a new medication, and between different levels of improvement in clinical trials. Achievement of ID and MiDA according to the new JADAS cutoffs at least twice in the first year of disease predicted better outcome at 2 years. Conclusion The 2021 JADAS and cJADAS cutoffs revealed good metrologic properties in both definition and validation samples, and are therefore suitable for use in clinical trials and routine practice.

We describe a family with a novel, inherited AXIN2 mutation (c.1989G>A) segregating in an autosomal dominant pattern with oligodontia and variable other findings including colonic polyposis, gastric polyps, a mild ectodermal dysplasia phenotype with sparse hair and eyebrows, and early onset colorectal and breast cancers. This novel mutation predicts p.Tyr663X, which is a truncated protein that is missing the last three exons, including the DIX (Disheveled and AXIN interacting) domain. This nonsense mutation is predicted to destroy the inhibitory action of AXIN2 on WNT signaling. Previous authors have described an unrelated family with autosomal dominant oligodontia and a variable colorectal phenotype segregating with a nonsense mutation of AXIN2, as well as a frameshift AXIN2 mutation in an unrelated individual with oligodontia. Our report provides additional evidence supporting an autosomal dominant AXIN2-associated ectodermal dysplasia and neoplastic syndrome.

GaN/AlN based heterostructures made of stacked GaN QDs have been studied by means of cathodoluminescence (CL) and micro-Raman techniques. The number of stacked layers varied between 2 and 85 in the studied samples. The QDs emission has been revealed by cross sectional CL spectra, confirmed by the high blue shift with increasing injection conditions. In-plane depth resolved CL has shown the contribution from aggregates of QDs with different average size along the growth direction. Micro-Raman data allowed to obtain information about the strain fields present in the layers. Relaxation process seems to occur, depending on the structural characteristics of the samples. The complementary information given by the two optical techniques allowed a better understanding of the contributions affecting the QDs emissions, such as the QD sizes, the strain levels or the effect of the internal electric fields.

GaN/AlN stacked quantum dots have been studied by means of Cathodoluminescence and Transmission Electron Microscopy. Assignment of the optical emissions was made on the basis of the structural parameters and power dependent cathodoluminescence studies. Quantum dot layer (depending on the nominal GaN coverage) and buffer layers emissions were distinguished. A quantum dot size dependent blue shift and a saturation trend was observed by increasing the power injection conditions. An important difference, both in structural and optical properties, among the isolated and the stacked quantum dots layers was also evidenced.

The supercritical carbon dioxide (SC-CO2) process involves pressurizing CO2 in a chamber which generates liquid phase of carbon dioxide. Pressurized liquid CO2 has a strong extraction capability of organic and inorganic compounds. The recent studies have also demonstrated that antimicrobial effect of SC-CO2 due extraction some cellular components of microorganisms. The efficacy of a supercritical carbon dioxide treatment on alfalfa seeds contaminated with Escherichia coli K12 was tested at 2000, 3000, and 4000 psi at 50C. Samples were treated for 15, 30, and 60 min at each pressure. After pummeling the seed samples in 0.1% peptone water, the initial and final Escherichia coli and total aerobic bacteria on the seeds were determined by plating on 3M Petri Films. After 48 h of incubation at 37 C, the colonies were enumerated. Treated seeds were evaluated in terms of germination characteristics. For aerobic plate count, the effect of pressure in the range of 2000–4000 psi was not statistically significant (p > 0.05) even though 85.6% inactivation was achieved at 4000 psi for 60 min. For E. coli, the reductions for 2000, 3000, and 4000 psi treatments for 15 min were 26.6, 68.1, and 81.3%, respectively. As the time was increased from 15 to 60 min at 4000 psi, the percent E. coli reduction increased from 81.3% to 92.8%. The percent germination for all treatments was over 90%. There was no significant difference (p > 0.05) in the germination rate of treated and untreated seeds. Supercritical carbon dioxide treatments demonstrated a reduction of E. coli K12 and total aerobic counts without affecting the germination characteristics of alfalfa seeds (p < 0.05). This study was a step in the direction of improving safety of alfalfa seeds used to produce fresh sprouts, which have been the cause of several outbreaks.

Episodic idiopathic systemic capillary leak syndrome is a rare disorder that presents with attacks of circulatory shock due to plasma leakage into the extravascular space. Reported here is the case of a girl who had recurrent circulatory shock. The diagnosis of episodic idiopathic systemic capillary leak syndrome was made following the fourth episode. The course was favorable following acute i.v. cristalloids and methylprednisolone.