ESPGHAN Podcast ESPGHAN

JPGN Journal Club reports from the Milan venue of ESPGHAN’s 2024 annual meeting, seizing the opportunity to learn from those rarely in Europe – as today with Prof Binita Kamath, in transit between Toronto and Philadelphia professionally, who along with Dr Jake Mann will tag-team her way down the field shooting for goal with two articles of interest (Winter et al.’s Biomarkers predicting the effect of anti‐TNF treatment in paediatric and adult inflammatory bowel disease, from J Pediatr Gastroenterol Nutr 2024 May 2 doi: 10.1002/jpn3.12221, and Islam et al.’s A novel model to study mechanisms of cholestasis in human cholangiocytes reveals a role for the SIPR2 pathway, from Hepatol Commun 2024 Feb 268[3]:e0389).  
In the study from Rotterdam (Prof J Escher, team leader), proteomics screening identified several species more abundant in material from persons who had developed resistance to anti-tumour necrosis factor inhibition than in persons without such resistance.  The numbers of subjects in these two cohorts ?  Small.  The p value of the increases in abundance seen ?  Slight.  Larger numbers called for, perhaps with different statistical handling – but a promising start and an interesting attack on the question of “What leads to treatment failure ? “
Prof Kamath stepped forward to explicate the work by Islam et al. – quite properly, since it is her team’s work.  Extrahepatic bile-duct cholangiocytes, grown as organoids, were challenged with taurocholic acid and other agents, and the cholangiocytes’ responses, categorised by pathways of involved molecules, were charted and analysed.  Of interest was that a hitherto little-studied molecule, sphingosine-1-phosphate receptor 2, was implicated as participating strongly :  New routes for investigation !    
The information, viewpoints, and recommendations shared in this podcast are solely those of the hosts and guests, and may evolve over time as the field advances.
 

O-makase is the word of the day, the phrase that in Japan tells your chef that your meal is both literally and metaphorically in her hands – “Choose for me,” it means. Most fine dining has an equivalent; the French say menu de dégustation, here in Milan / Mailand / Milano it’s called menù degustazione. That is: Non prevede scelte da parte del cliente! You, the diner, have no say. Either eat what’s set on the plate in front of you or stand up and leave the restaurant. O-makase, baby !  
We hope you don’t leave, that is, switch off this podcast, although equally to please all 5300-plus delegate-guests at the ESPGHAN Annual Meeting Restaurant this year would be a miracle.  That’s 104 different countries-cuisines !  With courses in interventional ultrasound and in inflammatory bowel disease, also on offer are working-group and special-interest group meetings, an endoscopy learning zone, and an allied health professional course, together with 1433 accepted abstracts, uhm, menu items.  Now what about working a miracle? You ask.
Well, there’s riches there, something for everybody – has our chef assembled from among this a tasting menu for everybody ?  We think so.  She’s reviewed the entire bill of fare and has selected a double armful of dishes to set before you ; meaty, piquant, provocative, and in every instance driving forward the intellectual, clinically relevant, and tasty art of paediatric gastroenterology, hepatology, and nutrition.  Here’s your table! Loosen your clothing unobtrusively, say to Dr Elena Cernat of Leeds, chef extraordinaire – now what was that phrase ? Right! O-makase! – and get ready to feast.  
Elena Cernat´s favourite Italian song: Lasciate mi cantare https://open.spotify.com/track/5pzNrUTk5nhOOlUJQZGukp?si=e48dc20cde2743d3
ESPGHAN favourite Songs can be found on Spotify https://open.spotify.com/playlist/0YIHKjxITLEm9XNyHyypTo

Dr Alex Knisely today speaks with Prof Yvan Vandenplas of Brussels, where he was chief of paediatrics for many years. He’s a hollow-viscus gastroenterologist rather than a “liver man”, and he has made many contributions in his chosen field, particularly in feeding disorders of infancy and in cows’-milk allergy, a topic on which he has selected three articles for us, all published in 2023 : From JPGN, “An ESPGHAN position paper on the diagnosis, management and prevention of cow's milk allergy”, with him as lead author, and (both by Meyer R et al.) from World Allergy Organization Journal, “World Allergy Organization (WAO) Diagnosis and Rationale for Action against Cow’s Milk Allergy (DRACMA) Guideline update – VII – Milk elimination and reintroduction in the diagnostic process of cow’s milk allergy” and from Pediatric Allergy and Immunology, “The role of online symptom questionnaires to support the diagnosis of cow's milk allergy in children for healthcare professionals – A Delphi consensus study”. What is known about cows’-milk allergy, how to decide if cows’-milk allergy is a strong consideration in a particular patient, how to approach diagnosis and treatment in such a patient, and what may come next – all taken up in this podcast, which we hope you will enjoy.

Dr. Vandenplas favourite song: Last Ons Ein Bloom https://open.spotify.com/track/2zcjUcLW0JaMCLEzkSWxke?si=d8340e40db4f4a9b

ESPGHAN favourite Songs can be found on Spotify https://open.spotify.com/playlist/0YIHKjxITLEm9XNyHyypTo

Here again is JPGN Journal Club. It’s Spring, people!  Asparagus! Strawberries! White wine! All of these can be enjoyed as an ESPGHAN podcast listener, so let’s get at it :  Raise your sauce béarnaise-laden forks, your Sancerre glasses, and your play-volume settings.
Dr Jake Mann has chosen for today from Hepatology, by Stonebraker et al., Genetic variation in severe cystic fibrosis liver disease is associated with novel mechanisms for disease pathogenesis.  Genomes of substantial numbers of CFTR  disease patients, both with and without substantial liver disease (the former collected principally by centres in North Carolina and a centre In Ontario), were sieved for associations with the relatively uncommon but clinically burdensome features of biliary-tract injury and of malperfusional injury.  Indeed some were found, but what do they tell us about “novel mechanisms” ?  Hand-waving ensues.  A proof-of-concept study, then.  
Jake also has chosen, from J Pediatr Gastroenterol Nutr, by Di Lorenzo et al., Clinical trial :  Randomized controlled trial of linaclotide in children aged 6−17 years with functional constipation.  “News you can use”, perhaps:  Stooling improved to a respectable extent in those given higher doses of the agent, with the dosage ceiling still high above those employed so far. Response as a new discriminator, a new classifier, to permit dissection and separation of forms of functional constipation?  
Well, Jake as advocate will tell us what strengths and flaws he perceives in the two studies. Listen up, and don’t omit to pass the strawberries!

Dr. Alex Knisely today is talking to Dr. Georg-Friedrich Vogel – call him “Georg” – of the Medizinische Universität Innsbruck, in Austria, where he serves on the paediatric-hepatology wards and conducts research in the department of cell biology. In Vienna this May, at the ESPGHAN annual meeting, he presented observations on the utility of an ileal bile-acid transport inhibitor, odevixibat (those last four letters, i – b – a – t, are acronymic), in a collective of children suffering from cholestasis associated with ATP8B1 mutation (progressive familial intrahepatic cholestasis [PFIC], type 1) and from diarrhoea, metabolic acidosis, and allograft steatosis after liver transplantation. Although, as is to be expected in a mixed bag of patients, not all the data for which one might wish are available, Georg’s team have encountered for the most part favourable results. As you proceed in managing your PFIC patients with ATP8B1 disease, and as you consider IBAT- inhibitor therapy, consultations with Georg may be of considerable value – always good to walk behind someone who has broken trail, who can let you know where others have stumbled and where the footing is secure. Not only that, he can help you contribute to filling in those gaps in the collective data, bringing us all forward in our search to help these children and families. A very good example of what ESPGHAN, in facilitating multinational collaboration, is all about!

Dr. Vogel´s favourite song: Sgt. Peppers Lonely Heart Club https://open.spotify.com/track/4fUKE8EULjQdHF4zb0M8FO?si=df38f8c6709742df

ESPGHAN favourite Songs can be found on Spotify https://open.spotify.com/playlist/0YIHKjxITLEm9XNyHyypTo

JPGN Journal Club is again here for you! No, no point in all that applause, although we’re grateful: Remember, we can’t hear it.
As always, we’re glad to be back and we hope that you’re glad to have us back. Dr Jake Mann has chosen for today from Aliment Pharmacol Ther, by Ricciuto et al., Oral vancomycin is associated with improved inflammatory bowel disease clinical outcomes in primary sclerosing cholangitis-associated inflammatory bowel disease (PSC-IBD) :  A matched analysis from the Paediatric PSC Consortium.  And the consortium? Centres, 54 ; PSC patients, 1,362 ; PSC-IBD patients, 1061 ; PSC patients studied, 113. In matched cohorts, vancomycin recipients’ endoscopic and clinical-biochemistry indices of IBD improved substantially over control; no mention of how “liver numbers” responded. Fewer bacteria, less inflammation: Seems reasonable enough.  
Closer to ESPGHAN home is Jake’s next selection – from J Pediatr Gastroenterol Nutr – Lacaille et al., Awareness, referral and age at Kasai surgery for biliary atresia in Europe :  A survey of the Quality‐of‐Care Task Force of ESPGHAN.  Between 2015 and 2019, what referral patterns did 26 European hepatology centres see for 785 children with extrahepatic biliary atresia (EHBA)? Detection of jaundice by age 2wk to 3wk, with referral, is the goal – the reality, 55d (8wk ! ), hepatic portoenterostomy at age 61d, just past the 60d end of the period during which one can reasonably hope for good results.  Education campaigns thus far seem ineffective.  How to improve them? Also surveyed, 392 French paediatricians, a 20% response rate: Has introduction of stool-colour cards changed your handling of infantile cholestasis? Do you use the cards, do you feel that you understand cholestasis in infants? The faintly bilious answers, perhaps tinged with Gallic scepticism: Possibly ; Yes ; and . . . Frankly, no.  Too early to say if referral in France, and portoenterostomy results, will improve – card distribution, était-il battre l’eau avec un baton?