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8% for complete and partial responses, respectively. Acitretin was more effective as first-line than as a subsequent agent (p=0.008). A trend towards better response was observed in the combination arm compared to patients receiving acitretin alone (p=0.056). Median time to best response was 6.9 months (IQR 4.4-9.4); median duration of response was 23.7 months (IQR 11.9-35.4). Overall, the mean length of all treatment patterns was 569 days (SD 718.8). Therapy was discontinued in 5 (3.9%) cases due to drug intolerance. Adverse effects were recorded in 62 (48.4%) cases with dyslipidemia (n=31; 24.2%), xerosis (n=24; 18.6%), and hair loss (n=10; 7.8%) being the most commonly recorded. CONCLUSIONS Acitretin, either alone or as adjuvant, showed a stable long-term effectiveness in this cohort, especially when used in the first-line setting. This RAR-selective agonist may serve as an attractive option for treatment of MF and should be further evaluated. This article is protected by copyright. https://www.selleckchem.com/products/gdc-0068.html All rights reserved.Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and poorly described subtype of CIDP. We aimed to study their clinical and electrophysiological characteristics and response to treatment. From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis. Patients were considered to have pure motor CIDP (PM-CIDP) if sensory conductions were normal or to have motor predominant CIDP (MPred-CIDP) if ≥2 sensory nerve action potential amplitudes were abnormal. https://www.selleckchem.com/products/gdc-0068.html Among the 700 patients with CIDP, 17 (2%) were included (PM-CIDP n = 7, MPred-CIDP n = 10); 71% were male, median age at onset was 48 years (range13-76 years), 47% had an associated inflammatory or infectious disease or neoplasia. At the more severe disease stage, 94% of patients had upper and lower limb weakness, with distal and proximal weakness in 4 limbs for 56% of them. Three-quarters (75%) responded to intravenous immunoglobulins (IVIg) and 4/5 patients to corticosteroids including 3/3 patients with MPred-CIDP. The most frequent conduction abnormalities were conduction blocks (CB, 82%) and F-wave abnormalities (88%). During follow-up, 4/10 MPred-CIDP patients developed mild sensory symptoms; none with PM-CIDP did so. Patients with PM-CIDP had poorer outcome (median ONLS4, range2-5) compared to MPred-CIDP (2, range0-4;P = 0.03) at last follow-up. The present study found a progressive clinical course in the majority of patients with motor CIDP as well as frequent associated diseases, CB, and F-wave abnormalities. Corticosteroids might be considered as a therapeutic option in resistant IVIg patients with MPred-CIDP. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.AIMS COVID-19 is now a global pandemic. At the time of survey, fewer than 150 children in Australia and New Zealand had documented infection. The aim of this study was to assess attitudes, readiness and confidence in the early stages of the COVID-19 pandemic through an online survey of paediatric physicians and sub-specialists across Australia and New Zealand. METHODS Multiple email list groups were used to contact paediatric physicians to undertake an online Likert scale survey between 17 and 24 March. Respondents' specialty, experience and work setting were recorded. Ordinal logistic regression was used to determine respondent factors. RESULTS There were 542 respondents from across Australia and New Zealand an estimated 11% of the paediatric physician workforce. A minority (36.6%) agreed that their national response had been well coordinated; the majority (92.7%) agreed that senior-level hospital administrators were taking the situation seriously. Most reported a good understanding of the natural history of COVID-19 in children, and knowledge of where to find local information. A large proportion of physicians (86.1%) were worried about becoming infected through their work; few (5.8%) reported that they would not come to work to avoid infection. Closure of school and childcares would reduce the ability to continue work at current capacity for 23.6% of respondents. CONCLUSION Despite limited experience in pandemics, most paediatric physicians felt informed. Concern about exposure at work is common; most were willing to work regardless. The closure of schools and daycares may have an impact on staffing. Coordination and leadership will be critical. © 2020 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).Because of their rigidity, polycyclic aromatic hydrocarbons (PAHs) have become a significant building block in molecular materials chemistry. Fusion or doping of boron into PAHs is known to improve the optoelectronic properties by reducing the LUMO energy level. Herein, we report a comprehensive study on the syntheses, structures, and photophysical properties of a new class of fused  N -heterocyclic boranes (NHBs), pyrene- and benzene-linked in a "Janus-type" fashion ( 2-4 ,  6-9,  and 11 ). Remarkably, these examples of fused NHBs display fluorescent properties, and collectively their emission spans the visible spectrum. The  pyrene-fused NHBs all display blue fluorescence, as the excitations are dominated by the pyrene core. In notable contrast, the emission properties of the benzene-fused analogues are highly tunable and is dependent on the electronics of the NHB fragments (i.e., the functional group directly bound to the boron atoms).  Pyrene-fused  2 - 4 and  11 represent the only molecules in which the K-region of pyrene is functionalized with NHB units , and while they exhibit distorted (twisted or stair-stepped) pyrene cores, benzene-fused 6 - 9 are planar.  The electronic structure and optical properties of these materials were probed by computational studies, including an evaluation of  aromaticity, electronic transitions, and molecular orbitals. © 2020 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.Evidence-based standards are fundamental to the practice, funding, and governance of modern medicine. These standards are developed using hierarchies of evidence yet it is often not appreciated that different hierarchies exist and there is a risk that inconsistent standards may be developed depending upon the hierarchy that is used. In this paper, we present four factors, independent of study design, that have led to differences amongst hierarchies. These factors are establishment of professional jurisdiction, practical concerns, methodological quality, and the importance of different questions within medicine. We demonstrate that each of these factors has led to the upgrading of expert opinion and/or the downgrading of randomized controlled trials and meta-analyses within different hierarchies. Our aim is to raise awareness of factors that have influenced the development of hierarchies. This may make the reader more critical of the processes that are used to develop evidence based standards. © 2020 John Wiley & Sons, Ltd.