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CONCLUSIONS The efficiency of diagnosis and treatment for suspected COVID-19 patients can be improved under MDT mode, particularly for complicated and refractory cases.BACKGROUND Mechanisms that facilitate early infection and inflammation in cystic fibrosis (CF) are unclear. We previously showed that young CF children with secondhand smoke exposure (SHSe) have increased susceptibility to respiratory infections. We aimed to define the impact of SHSe and other external factors upon the fecal bacteriome in early CF. METHODS Twenty CF infants and children were enrolled, clinical data recorded, and hair nicotine measured as an objective surrogate of SHSe. Fecal samples were collected at clinic visits and bacteriome 16S rRNA gene sequencing performed. RESULTS SHSe was associated with increased alpha diversity and increased relative abundance of Acinetobacter and Akkermansia, along with decreased Bifidobacterium and Lactobacillus. Recent antibiotic exposure predicted bacterial population structure in children less than 2 years of age and was associated with decreased Bacteroides relative abundance. Age was the strongest predictor of overall fecal bacterial composition and positively associated with Blautia and Parabacteroides. Weight for length was negatively associated with Staphylococcus relative abundance. CONCLUSIONS SHSe and other external factors such as antibiotics appear to alter fecal bacterial composition in young CF children, but the strongest predictor of overall composition was age. These findings have implications for understanding the intestinal microbiome in young CF children. © 2020 Wiley Periodicals, Inc.BACKGROUND Autism spectrum disorder (ASD) affects approximately one in 59 children. Variants in the activity-dependent neuroprotector homeobox ADNP (OMIM #611386) gene may be one of the most common single-gene causes of syndromic ASD. Most patients diagnosed with ADNP syndrome have ASD as a comorbidity, and all patients have mild-to-severe intellectual disability. METHODS/CASE REPORT We present a case report of a patient diagnosed with ADNP syndrome at 2.5 years of age. The patient has many of the key features of the syndrome, including ASD, global developmental delay, behavioral problems, congenital heart defect, early tooth eruption, and vision problems. The patient's initial presentation included congenital diaphragmatic hernia (CDH), which has not been previously reported in this condition. RESULTS The patient exhibited frequent behavioral outbursts and was initiated on antipsychotic medication with near-complete resolution of symptoms allowing her to engage more fully in early intervention therapies leading to progress in language acquisition. this website CONCLUSION This short report provides guidance for antipsychotic medication dosing to improve early intervention outcomes. This is the first report of CDH in this syndrome. © 2020 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.OBJECTIVE To investigate predictors of response, remission, low disease activity (LDA), damage and drug discontinuation in patients with systemic lupus erythematosus (SLE) treated with belimumab. METHODS We retrospectively analysed data of a multicentre cohort of SLE patients receiving intravenous belimumab. Proportion of patients achieving remission, LDA and SLE Responder Index-4 (SRI-4) were evaluated. SLICC damage index (SDI) was calculated yearly. Predictors of outcomes were investigated by multivariate logistic regression. RESULTS We included 466 active SLE patients from 24 Italian centres median (range) follow-up 18 (1-60) months. SRI-4 was achieved by 49.2%, 61.3%, 69.7%, 69.6% and 66.7% patients at 6, 12, 24, 36 and 48 months. Baseline predictors of response at 6 months were SLEDAI-2K≥10 (OR 3.14, 95%CI 2.033-4.860) and disease duration≤2 years (OR 1.94, 95%CI 1.078-3.473); at 12 months SLEDAI-2K≥10 (OR 3.48, 95%CI 2.004-6.025), SDI=0 (OR 1.74, 95%CI 1.036-2.923); at 24 months SLEDAI-2K≥10 (OR 4.25, 95%CI 2.018-8.940), disease duration ≤2 years (OR 3.79, 95%CI 1.039-13.52); at 36 months SLEDAI-2K≥10 (OR 14.59, 95%CI 3.54-59.79) and baseline smoking (OR 0.19, 95%CI 0.039-0.69). Patients spending≥25% follow-up in remission (42.9%) or≥50% in LDA (66.0%) accrued significantly less damage (p=0.046 and p=0.007). Baseline SDI=0 independently predicted LDA ≥50% and remission ≥25%; the lower the baseline damage, the higher the probability of remission ≥25%. Number of previous flares negatively predicted belimumab discontinuation due to inefficacy (p= 0.009) CONCLUSIONS The early use of belimumab in patients with active SLE and low baseline damage predicts favourable outcomes in a real-life setting. This article is protected by copyright. All rights reserved.The pandemic caused by severe acute respiratory syndrome coronavirus 2 heavily involves all those working in a laboratory. Samples from known infected patients or donors who are considered healthy can arrive, and a colleague might be asymptomatic but able to transmit the virus. Working in a clinical laboratory is posing several safety challenges. Few years ago, International Society for Advancement of Cytometry published guidelines to safely analyze and sort human samples that were revised in these days. We describe the procedures that we have been following since the first patient appeared in Italy, which have only slightly modified our standard one, being all human samples associated with risks. © 2020 International Society for Advancement of Cytometry.Bosch-Boonstra-Schaaf Optic Atrophy Syndrome (BBSOAS) is an autosomal dominant neurodevelopmental disorder caused by loss-of-function variants in NR2F1 and characterized by visual impairment, developmental delay, and intellectual disability. Here we report 18 new cases, provide additional clinical information for 9 previously reported individuals, and review an additional 27 published cases to present a total of 54 patients. Among these are 22 individuals with point mutations or in-frame deletions in the DNA-binding domain (DBD), and 32 individuals with other types of variants including whole-gene deletions, nonsense and frameshift variants, and point mutations outside the DBD. We corroborate previously described clinical characteristics including developmental delay, intellectual disability, autism spectrum disorder diagnoses/features thereof, cognitive/behavioral anomalies, hypotonia, feeding difficulties, abnormal brain MRI findings, and seizures. We also confirm a vision phenotype that includes optic nerve hypoplasia, optic atrophy, and cortical visual impairment. Additionally, we expand the vision phenotype to include alacrima and manifest latent nystagmus (fusional maldevelopment), and we broaden the behavioral phenotypic spectrum to include a love of music, an unusually good long-term memory, sleep difficulties, a high pain tolerance, and touch sensitivity. Furthermore, we provide additional evidence for genotype-phenotype correlations, specifically supporting a more severe phenotype associated with DBD variants. © 2020 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals, Inc.BACKGROUND Thyroid tumor size is an important prognostic factor. The aim of this study is to examine the histological subtypes and management of thyroid cancer based on tumor size (≤4 cm vs >4 cm). METHODS Retrospective cohort study utilizing the National Cancer Database, 2004-2014. RESULTS A total of 152 387 patients were included, 13 614 (8.9%) of whom had a tumor size >4 cm. Histological subtypes of tumors >4 cm were 69.6% papillary thyroid carcinoma, 17.5% FTC, 7.9% HCC, and 2.8% medullary thyroid carcinoma (MTC). High-volume hospitals for thyroid surgery were less likely to perform two-stage thyroidectomy, particularly for tumors ≤4 cm. Low-volume hospitals had a higher risk of staged thyroidectomy for MTC ≤4 cm (19.8%) compared with high-volume hospitals (8.7%) (P less then  .001). CONCLUSIONS This study describes the prevalence of thyroid cancer subtypes. In the era of a conservative approach to differentiated thyroid carcinoma, there could be a potential increase in the risk of staged thyroidectomy. © 2020 Wiley Periodicals, Inc.Newborn screening (NBS) is a well-established state-run public health program which has targeted the early identification of treatable diseases like classic galactosemia (CG) for over a decade. We describe the case of a symptomatic newborn with CG and an abnormal screen report, including positive DNA-based test, who still managed to fall through the cracks in a sub-optimally functioning NBS program, despite decades of screening experience. While much attention is paid to testing technology, this case illustrates basic minimum requirements a newborn screening program must fulfill to reliably identify and treat all affected individuals including minimum reporting requirements, case surveillance and a dedicated short-term follow-up program. In newborn screening, success is systematic. © 2020 Wiley Periodicals, Inc.BACKGROUND Children with cancer experience a wide range of conditions that require urgent evaluation in the emergency department (ED), yet variation in admission rates is poorly documented. PROCEDURE We performed a retrospective cohort study using the Pediatric Health Information System of ED encounters by children with cancer between July 2012 and June 2015. We compared demographics for admitted versus discharged using univariate statistics, and calculated admission rates by hospital, diagnosis, day of the week, and weekend versus weekday. We assessed the degree of interhospital admission rates using the index of dispersion (ID). RESULTS Children with cancer had 60 054 ED encounters at 37 hospitals. Overall, 62.5% were admitted (range 43.2%-92.1%, ID 2.6) indicating overdispersed admission rates with high variability. Children with cancer that visited the ED for a primary diagnosis of fever experienced the largest amount of variability in admission with rates ranging from 10.4% to 74.1% (ID 8.1). Less variability existed among hospital admission rates for both neutropenia (range 60%-100%, ID 1.0) and febrile neutropenia (FN) (range 66.7%-100%, ID 0.83). Admission rates by day of the week did not demonstrate significant variability for any of the scenarios examined (overall P = 0.91). There were no differences by weekend versus weekday either (overall P = 0.52). CONCLUSION The percentage of children with cancer admitted through the ED varies widely by institution and diagnosis. Standardization of best practices for children with cancer admitted through the ED should be an area of continued improvement. © 2020 Wiley Periodicals, Inc.Model erythro, phenolic and non-phenolic lignin β -O-4 dimer compounds were reacted with copper oxide and H 2 O 2 at the electronic field maximum position of a single-mode 2.45-GHz microwave system equipped with a cavity resonator. The reaction products obtained via microwave heating and an oil bath heating in the same reaction vessel and same temperature profile were quantitatively compared. Dimer degradation proceeded through consecutive elementary reactions. The phenolic dimer was dehydroxylated and followed by the spontaneous cleavage of C α -C β and C-O-C bonds to produce guaiacol, vanillin, and vanillic acid. The reaction of the non-phenolic dimer produced veratric acid, veratraldehyde, and guaiacol. Microwave irradiation accelerated cleavage of the side chain and the oxidation of vanillin to vanillic acid. However, no acceleration of veratraldehyde oxidation to veratric acid or aromatic ring cleavage to produce dicarboxylic acids was observed. The selective acceleration of elementary reactions during the degradation of model lignin compounds indicated that microwaves interacted with reaction intermediates that were sensitive to electromagnetic wave.
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