High accuracy 234U(n,f) cross section in the resonance energy region

New results are presented of the 234U neutron-induced fission cross section, obtained with high accuracy in the resonance region by means of two methods using the 235U(n,f) as reference. The recent evaluation of the 235U(n,f) obtained with SAMMY by L. C. Leal et al. (these Proceedings), based on previous n-TOF data [1], has been used to calculate the 234U(n,f) cross section through the 234U/235U ratio, being here compared with the results obtained by using the n-TOF neutron flux

Expanding the clinical and genetic spectrum of ALPK3 variants: Phenotypes identified in pediatric cardiomyopathy patients and adults with heterozygous variants

Introduction: Biallelic damaging variants in ALPK3, encoding alpha-protein kinase 3, cause pediatric-onset cardiomyopathy with manifestations that are incompletely defined. Methods and Results: We analyzed clinical manifestations of damaging biallelic ALPK3 variants in 19 pediatric patients, including nine previously published cases. Among these, 11 loss-of-function (LoF) variants, seven compound LoF and deleterious missense variants, and one homozygous deleterious missense variant were identified. Among 18 live-born patients, 8 exhibited neonatal dilated cardiomyopathy (44.4%; 95% CI: 21.5%-69.2%) that subsequently transitioned into ventricular hypertrophy. The majority of patients had extracardiac phenotypes, including contractures, scoliosis, cleft palate, and facial dysmorphisms. We observed no association between variant type or location, disease severity, and/or extracardiac manifestations. Myocardial histopathology showed focal cardiomyocyte hypertrophy, subendocardial fibroelastosis in patients under 4 years of age, and myofibrillar disarray in adults. Rare heterozygous ALPK3 variants were also assessed in adult-onset cardiomyopathy patients. Among 1548 Dutch patients referred for initial genetic analyses, we identified 39 individuals with rare heterozygous ALPK3 variants (2.5%; 95% CI: 1.8%-3.4%), including 26 missense and 10 LoF variants. Among 149 U.S. patients without pathogenic variants in 83 cardiomyopathy-related genes, we identified six missense and nine LoF ALPK3 variants (10.1%; 95% CI: 5.7%-16.1%). LoF ALPK3 variants were increased in comparison to matched controls (Dutch cohort, P = 1.6×10−5; U.S. cohort, P = 2.2×10−13). Conclusion: Biallelic damaging ALPK3 variants cause pediatric cardiomyopathy manifested by DCM transitioning to hypertrophy, often with poor contractile function. Additional extracardiac features occur in most patients, including musculoskeletal abnormalities and cleft palate. Heterozygous LoF ALPK3 variants are enriched in adults with cardiomyopathy and may contribute to their cardiomyopathy. Adults with ALPK3 LoF variants therefore warrant evaluations for cardiomyopathy

Long-term effects of transabdominal electrical stimulation in treating children with slow-transit constipation

Aims: Transcutaneous electrical stimulation (TES) was used to treat children with slow-transit constipation (STC) for 1 to 2 months in a randomized controlled trial during 2006 to 2008. We aimed to determine long-term outcomes, hypothesizing that TES produced sustained improvement. Methods: Physiotherapists administered 1 to 2 months of TES to 39 children (20 minutes, 3 times a week). Fifteen continued to self-administer TES (30 minutes daily for more than 2 months). Mean long-term follow-up of 30 of 39 patients was conducted using questionnaire review 3.5 years (range 1.9-4.7 years) later. Outcomes were evaluated by confidence intervals or paired t test. Results: Seventy-three percent of patients perceived improvement, lasting more than 2 years in 33% and less than 6 months in 25% to 33%. Defecation frequency improved in 30%. Stools got wetter in 62% after stimulation and then drier again. Soiling improved in 75% and abdominal pain in 59%. Laxative use stopped in 52%, and 43% with appendicostomies stopped washouts. Soiling/Holschneider continence score improved in 81% (P = .0002). Timed sits switched to urge-initiated defecations in 80% patients. Eighty percent of relapsed patients elected to have home stimulation. Conclusion: TES holds promise for STC children. Improvement occurred in two thirds of children, lasting more than 2 years in one third, whereas symptoms recurred after 6 months in one third of children. (C) 2011 Elsevier Inc. All rights reserved

Group B Streptococcus infections caused by improper sourcing and handling of fish for raw consumption, Singapore, 2015–2016

10.3201/eid2312.170596Emerging Infectious Diseases23121982-199

Role of soil rhizobacteria in phytoremediation of heavy metal contaminated soils

Heavy metal pollution of soil is a significant environmental problem and has its negative impact on human health and agriculture. Rhizosphere, as an important interface of soil and plant, plays a significant role in phytoremediation of contaminated soil by heavy metals, in which, microbial populations are known to affect heavy metal mobility and availability to the plant through release of chelating agents, acidification, phosphate solubilization and redox changes, and therefore, have potential to enhance phytoremediation processes. Phytoremediation strategies with appropriate heavy metal-adapted rhizobacteria have received more and more attention. This article paper reviews some recent advances in effect and significance of rhizobacteria in phytoremediation of heavy metal contaminated soils. There is also a need to improve our understanding of the mechanisms involved in the transfer and mobilization of heavy metals by rhizobacteria and to conduct research on the selection of microbial isolates from rhizosphere of plants growing on heavy metal contaminated soils for specific restoration programmes