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We offer our opinions on the most promising directions of research and how various aspects of each area might be unified towards common goals.

Diagnosis of childhood polyarteritis nodosa (PAN) has become challenging after the definition of deficiency of adenosine deaminase 2 (DADA2). We aimed to define the differential features of pediatric PAN and DADA2 patients in our center and in the literature.

The charts of pediatric PAN and DADA2 patients followed at the Pediatric Rheumatology Unit of Hacettepe University between 2010-2020 were analyzed. A systematic literature review was conducted for articles regarding pediatric PAN or DADA2.

Thirty-four pediatric PAN and 18 pediatric DADA2 patients were included. The age at onset was younger, parental consanguinity, livedo reticularis, neurologic involvement (especially strokes), lymphopenia, and hypogammaglobulinemia were more frequent, while thrombocytosis and panniculitis were less frequent in DADA2 patients. The primary treatment was anti-tumor necrosis factor (anti-TNF) in DADA2. For induction treatment, all systemic PAN patients received corticosteroids, and cyclophosphamide (n=11) or mycophenoic DADA2. Cyclophosphamide could be switched to MMF when remission is confirmed with PVAS in severe PAN.

The aim of this study was to investigate whether peripheral arthritis together with disease activity independently contribute to functional impairment over time in patients with axSpA and to evaluate if there are contextual factors modifying this relationship.

Patients with axial spondyloarthritis from the ESPAXIA cohort were followed-up annually over a mean of 3.7 years. Physical function was assessed by the self-reported questionnaire BASFI, disease activity by ASDAS and peripheral arthritis was also recorded. Generalized estimating equations (GEE) were used to investigate longitudinal association between peripheral arthritis, ASDAS and BASFI as the outcome. Autoregressive models (adjusted for BASFI 1 year earlier) were run to allow for a truly longitudinal interpretation. Interactions between each of ASDAS and peripheral arthritis with contextual factors (age, gender, educational level, smoking, job type) were tested.

185 patients (77 % male, mean (SD) age 42 (13) years old and mean disease duration (SD) of 9.4 (9.6) years) were included. ASDAS and peripheral arthritis independently contributed to explaning BASFI over time. Contextual factors did not modify either of the relationships. A true longitudinal relation was proven with the autoregressive GEE model, showing that, adjusted for age, gender, spinal mobility and use of NSAIDs, an increase of one ASDAS unit led to a BASFI 0.48 units higher (ß 0.48 [95%CI 0.39, 0.57]), and the presence of peripheral arthritis, to a BASFI 0.44 units higher (ß 0.44 [95%CI 0.08, 0.8]).

Peripheral arthritis and higher disease activity independently lead to more functional impairment in axSpA over time. Contextual factors do not modify these relationships.

Peripheral arthritis and higher disease activity independently lead to more functional impairment in axSpA over time. Contextual factors do not modify these relationships.Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that is characterized by a high heterogeneity in patients' disease course. Patients with bulbar onset of symptoms (b-ALS) have a poorer prognosis than patients with limb onset (l-ALS). However, neuroimaging correlates of the assumed biological difference between b-ALS and l-ALS may have been obfuscated by patients' diversity in the disease course. We conducted Voxel-Based-Morphometry (VBM) and Tract-Based-Spatial-Statistics (TBSS) in a group of 76 ALS patients without clinically relevant cognitive deficits. The subgroups of 26 b-ALS and 52 l-ALS patients did not differ in terms of disease Phase or disease aggressiveness according to the D50 progression model. VBM analyses showed widespread ALS-related changes in grey and white matter, that were more pronounced for b-ALS. TBSS analyses revealed that b-ALS was predominantly characterized by frontal fractional anisotropy decreases. This demonstrates a higher degree of neurodegenerative burden for the group of b-ALS patients in comparison to l-ALS. Correspondingly, higher bulbar symptom burden was associated with right-temporal and inferior-frontal grey matter density decreases as well as fractional anisotropy decreases in inter-hemispheric and long association tracts. Contrasts between patients in Phase I and Phase II further revealed that b-ALS was characterized by an early cortical pathology and showed a spread only outside primary motor regions to frontal and temporal areas. In contrast, l-ALS showed ongoing structural integrity loss within primary motor-regions until Phase II. We therefore provide a strong rationale to treat both onset types of disease separately in ALS studies.

We sought to determine the feasibility of the Practical Alternative to Hospitalization (PATH) program, an intervention that offers ED clinicians an outpatient care pathway for patients initially designated for inpatient admission or observation.

We evaluated a novel care delivery model that was piloted at a tertiary academic medical center in December 2019. An advanced practice provider screened patients designated for inpatient admission or observation and identified eligible participants. Outpatient services were customized for each patient but primarily included care coordination and monitoring through telemedicine and home health services. The primary feasibility outcome was the proportion of eligible patients who were enrolled in the program, as well as patient outcomes after discharge including return ED visits and averted ED boarding time.

A total of 199 patients were designated for inpatient admission or observation during PATH program hours. Of 52 eligible patients, 30 (58%) were enrolled. The mean participant age was 62.5 years (SD 17.5), and 25 (83%) had non-Hispanic Black race/ethnicity. The most common disease conditions were chest pain, heart failure, and hyperglycemia. 4 (13%) enrolled patients returned to an ED within 30 days. We estimate that ED boarding time was reduced by 8.2h (SD 8.1) per patient.

Emergency physicians and patients were willing to use a novel service that provided an alternative disposition to hospitalization.

alternative payment models that seek to reduce hospital utilization and cost may consider strengthening systems to monitor and coordinate care for patients after ED discharge.

alternative payment models that seek to reduce hospital utilization and cost may consider strengthening systems to monitor and coordinate care for patients after ED discharge.Temporal encephaloceles (TEs) are one of the cause of refractory temporal lobe epilepsy (TLE). We reviewed the neuroimaging and video-electroencephalography (EEG) records of epilepsy patients who underwent temporal lobectomy in our center to investigate frequency of TEs. We retrospectively reevaluated 294 patients who underwent epilepsy surgery in our tertiary epilepsy centre between January 2010 and March 2019 and included 159 patients (78 females, 49 %; 81 males) who had temporal lobectomy. Preoperatively, TEs were reported in 3 of 159 patients (1 female, 2 males). After reevaluation 4 more patients with TEs (1 female, 3 males) were added. The ratio of TE in patients who underwent temporal lobectomy increased from 1.8 % (n=3) to 4,4 % (n=7). The median ages were 18 (range 16-22) versus 10 years (range 5-17) at habitual seizure onset and the median of epilepsy duration was 5 (range 3-15) versus 175 (range 11-25) years between patients with and without TE. Habitual seizure onset age was significantly higher (p =, 007) in the patients with encephalocele and epilepsy duration was shorter (p =, 003) than patients without encephalocele. The ictal EEG records of all patients TE rhythmic delta activity which is suggested neocortical temporal lobe onset seizures. 4 of 7 patients' PET imaging showed temporal lobe hypometabolism compatible with ipsilateral to the TEs. The three patients underwent anterior temporal lobectomy without amygdalohippocampectomy and others had anterior temporal lobectomy with amygdalohippocampectomy. We suggested that there might be some clues for temporal encephalocele, an easily overlooked cause in patients with nonlesional temporal lobe epilepsy.TLE patients with TE had relatively late onset of epilepsy and rhythmic delta activity on ictal EEG. Also, temporal hypometabolism on PET may be a useful key to suspicion of TE.Red mud and phosphogypsum are voluminous industrial by-products worldwide. They have long been disposed of in landfills or open storage, leading to a waste of resource and environmental pollution. This study provides a novel approach to recycle these industrial by-products as sustainable red mud-phosphogypsum-Portland cement (RPPC) binders for stabilization/solidification (S/S) of multimetal-contaminated soil. The physical strength, metal leachability and microstructure of S/S soil were investigated after 7-day and 28-day curing, as well as freezing-thawing (F-T) cycle and wetting-drying (W-D) cycle. The results show that the strength of soil treated by all binders fulfilled the uniaxial compressive strength requirement (350 kPa) of S/S waste in landfills. Microstructural analyses show that the main hydration products of the RPPC S/S soil are ilmenite, ettringite, anhydrite and hydrated calcium silicate. The 10% and 15% RPPC binders have a competitive metal immobilization ability compared with 10% PC, but the immobilization priority is different Pb > Zn > Cd in RPPC system and Zn > Cd > Pb in PC system, respectively, probably due to the precipiataion of Pb2+ with the abundant SO42- in phosphogypsum in RPPC system. The strength of RPPC and PC treated soil was still higher than 350 kPa except for RPPC7.5 after 10 freeze-thaw or 10 wetting-drying cycles. The RPPC binder performed worse than PC binder after both freeze-thaw and wetting-drying cycles, especially at a lower dosage. Only the metal leaching concentrations of samples treated by RPPC15 and PC10 could fulfil the Chinese standards for hazardous wastes.Chemosensory perception is crucial for fish reproduction and survival. Direct contact of olfactory neuroepithelium to the surrounding environment makes it vulnerable to contaminants in aquatic ecosystems. Copper nanoparticles (CuNPs), which are increasingly used in commercial and domestic applications due their exceptional properties, can impair fish olfactory function. However, the molecular events underlying olfactory toxicity of CuNPs are largely unexplored. Vorapaxar Our results suggested that CuNPs were bioavailable to olfactory mucosal cells. Using RNA-seq, we compared the effect of CuNPs and copper ions (Cu2+) on gene transcript profiles of rainbow trout (Oncorhynchus mykiss) olfactory mucosa. The narrow overlap in differential gene expression between the CuNP- and Cu2+-exposed fish revealed that these two contaminants exert their effects through distinct mechanisms. We propose a transcript-based conceptual model that shows that olfactory signal transduction, calcium homeostasis, and synaptic vesicular signaling were affected by CuNPs in the olfactory sensory neurons (OSNs).