JPGN Journal Club: October 2024

October JPGN Journal Club, led by Dr Jake Mann !  As always, keep in mind ESPGHAN’s other educational offerings :  https://www.espghan.org/knowledge-center – in particular on X.25 a Monothematic Conference on Paediatric Gastric Disease ; on XI.11 the Winter School on Basic Science and Translational Research ; and on XI.15 a Masterclass on Transition from Paediatric to Adult Healthcare in Patients with GI or Liver Disease.  For today’s discussion Jake has chosen a review of a small series of patients – from J Pediatr Gastroenterol Nutr, by D’Arienzo et al., “Characteristics and outcomes of home parenteral nutrition among children with severe neurological impairment” – and from Hepatology, by Xiao et al., a molecular-biologic analysis of cells that take part in the inflammatory, fibrotic, and proliferative processes of extrahepatic biliary atresia, “Integrative single-cell and spatial transcriptomic analyses identify a pathogenic cholangiocyte niche and TNFRSF12A as therapeutic target for biliary atresia”.  We learn from the JPGN article that severe neurological impairment confers neither benefit nor risk upon treatment with home parenteral nutrition, possibly a comforting conclusion.  From the Hepatology article we learn that blockade or knock-out of tumor necrosis factor receptor superfamily member 12A, or TNFRSF12A, ablates the usual changes in portal tracts of a mouse model of biliary atresia, suggesting an approach to therapy.  The techniques used in this study are complex – we’re lucky to have Jake as our guide to understanding it all. Literature D’Arienzo D et al.  Characteristics and outcomes of home parenteral nutrition among children with severe neurological impairment.  J Pediatr Gastroenterol Nutr 2024 Sep 10.  DOI :  10.1002/jpn3.12369.  PMID :  39252541  Xiao MH et al.  Integrative single-cell and spatial transcriptomic analyses identify a pathogenic cholangiocyte niche and TNFRSF12A as therapeutic target for biliary atresia.  Hepatology 2024 Aug 23.  DOI :  10.1097/HEP.0000000000001064. PMID :  39178365   

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