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Objectives: Odevixibat, a reversible ileal bile acid transport inhibitor, has been shown to reduce serum bile acids (sBA) and pruritus mostly in children with progressive familial intrahepatic cholestasis (PFIC) 1 and 2 in clinical trials and case reports. There are currently no published case reports or series describing its use in rare variants of cholestatic liver disease., Methods: We describe three children with progressive cholestatic liver disease who developed refractory pruritus, who had a genotypic diagnosis of AKR1D1 , ABCB4 variant, and PKHD1 and PKHD2 variants; all being variants of unknown significance as per the American College of Medical Genetics and Genomics guidelines., Results: On Odevixibat there was a significant improvement in sBA (absolute change from baseline: -196 and -393 μmol/L) and pruritus in two children with heterozygous AKR1D1 and ABCB4 mutations. The child with ABCB4 variants was found to have features of sclerosing cholangitis along with a diagnosis of Crohn's disease, which represents the first reported usage of Odevixibat in such a case with good response. There was some reported improvement in the third child with PKHD1 and PKHD2 variants; however, we hypothesize that no sustained improvement could be due to severe and progressive nature of the disease. There were no side effects reported and it was well tolerated in all., Conclusion: We suggest that Odevixibat may be used as an adjunctive drug in refractory pruritus and could be started early in the course of disease if clinically and phenotypically indicated., Competing Interests: Dr. Tassos Grammatikopoulos: Consultancy‐Albireo. The remaining authors declare no conflict of interest., (© 2024 The Authors. JPGN Reports published by Wiley Periodicals LLC on behalf of The European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

The Amazon forest carbon sink is declining, mainly as a result of land-use and climate change 1-4 . Here we investigate how changes in law enforcement of environmental protection policies may have affected the Amazonian carbon balance between 2010 and 2018 compared with 2019 and 2020, based on atmospheric CO 2 vertical profiles 5,6 , deforestation 7 and fire data 8 , as well as infraction notices related to illegal deforestation 9 . We estimate that Amazonia carbon emissions increased from a mean of 0.24 ± 0.08 PgC year -1 in 2010-2018 to 0.44 ± 0.10 PgC year -1 in 2019 and 0.52 ± 0.10 PgC year -1 in 2020 (± uncertainty). The observed increases in deforestation were 82% and 77% (94% accuracy) and burned area were 14% and 42% in 2019 and 2020 compared with the 2010-2018 mean, respectively. We find that the numbers of notifications of infractions against flora decreased by 30% and 54% and fines paid by 74% and 89% in 2019 and 2020, respectively. Carbon losses during 2019-2020 were comparable with those of the record warm El Niño (2015-2016) without an extreme drought event. Statistical tests show that the observed differences between the 2010-2018 mean and 2019-2020 are unlikely to have arisen by chance. The changes in the carbon budget of Amazonia during 2019-2020 were mainly because of western Amazonia becoming a carbon source. Our results indicate that a decline in law enforcement led to increases in deforestation, biomass burning and forest degradation, which increased carbon emissions and enhanced drying and warming of the Amazon forests., (© 2023. The Author(s), under exclusive licence to Springer Nature Limited.)

Flavonoids are involved in citrus defense against phytopathogens. In this study, we applied in vitro biocatalysis assays using the flavanones glycosides hesperidin and naringin to explore the enzymatic activities involved in such interaction. The main enzymatic activity observed was the hydrolysis catalyzed by fungi naringinases and hesperidinases. Withing 7 days, the two citrus phytopathogenic fungi, Penicillium digitatum and Penicillium italicum, exhibited the highest hydrolyzing rate on the flavanones, reaching conversion values higher than 90%. In addition, Geothrichum citri-aurantii exhibited no enzymatic activity and Penicillium expansum only hydrolyzed hesperidin. In order to evaluate flavonoid biotransformation by the fungi in vivo, citrus fruits infected with P. digitatum were analyzed through molecular networking and Imaging Mass Spectrometry (IMS). In vivo assays revealed that citrus fruit in response to the infection is able to hydroxylate flavonoids, and novel flavonoid structures were associated to the citrus' defense. The data reported here present a new point of view in the relation between citrus flavonoids and phytopathogenic fungi and can be useful to understand the infection processes and host-pathogen interaction., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Huanglongbing (HLB) is a disease of worldwide incidence that affects orange trees, among other commercial varieties, implicating in great losses to the citrus industry. The disease is transmitted through Diaphorina citri vector, which inoculates Candidatus Liberibacter spp. in the plant sap. HLB disease lead to blotchy mottle and fruit deformation, among other characteristic symptoms, which induce fruit drop and affect negatively the juice quality. Nowadays, the disease is controlled by eradication of sick, symptomatic plants, coupled with psyllid control. Polymerase chain reaction (PCR) is the technique most used to diagnose the disease; however, this methodology involves high cost and extensive sample preparation. Mass spectrometry imaging (MSI) technique is a fast and easily handled sample analysis that, in the case of Huanglongbing allows the detection of increased concentration of metabolites associated to the disease, including quinic acid, phenylalanine, nobiletin and sucrose. The metabolites abieta-8,11,13-trien-18-oic acid, suggested by global natural product social molecular networking (GNPS) analysis, and 4-acetyl-1-methylcyclohexene showed a higher distribution in symptomatic leaves and have been directly associated to HLB disease. Desorption electrospray ionization coupled to mass spectrometry imaging (DESI-MSI) allows the rapid and efficient detection of biomarkers in sweet oranges infected with Candidatus Liberibacter asiaticus and can be developed into a real-time, fast-diagnostic technique.

Phytopathogens are responsible for great losses in agriculture, once they are able to subvert or elude the host defense mechanisms through virulence factors secretion for their dissemination. Herein, it is reviewed phytotoxins that act as virulence factors and are produced by bacterial phytopathogens ( Candidatus Liberibacter spp., Erwinia amylovora , Pseudomonas syringae pvs and Xanthomonas spp.) and fungi ( Alternaria alternata , Botrytis cinerea , Cochliobolus spp., Fusarium spp., Magnaporthe spp., and Penicillium spp.), which were selected in accordance to their worldwide importance due to the biochemical and economical aspects. In the current review, it is sought to understand the role of virulence factors in the pathogen-host interactions that result in plant diseases.