ESPGHAN Podcast ESPGHAN

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!

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?

Dr Alex Knisely today is speaking with Prof Isabelle Scheers of Louvain, Belgium, on pancreatitis in children. She has proposed three articles for discussion – from a coalition that she led, drawing on collaborators in Canada, the United States, and almost the full bank of Eurovision Song Contest participant nations, a summary and review, Autoimmune Pancreatitis in Children : Characteristic Features, Diagnosis, and Management ; a personal “position paper”, Inherited Pancreatic Exocrine Insufficiency and Pancreatitis : When Children Transition to Adult Care ; and a rara avis case report with others from her home institution, Cinacalcet Sustainedly Prevents Pancreatitis in a Child with a Compound Heterozygous SPINK1 / AP2S1 Mutation. One woman, but a pancreatic-disease panopticon, and if you don’t know that last word, hello Google ! She begins with the satisfaction through successful diagnosis and treatment that came her way when, as a junior doctor, she sorted out disease in the subject of her case report ; she takes us through how her need to educate herself to deal with the patients referred to her with pancreatic problems, an ever-growing stream, led her abroad for specialty training as she established a network of not only referrers but also advisers ; and she sketches for us how she became a pancreatologist who, through collaboration with other paediatricians and with adult pancreatologists, has helped us all by describing and defining what is to be expected in various types of pancreatitis in childhood, and, of course, how children in this as indeed in so many other things differ from adults. Follow along, and remember, after the podcast is over : Shared experience, shared through ESPGHAN, brings us all much further than any of us can go alone, and not just in pancreatitis !

JPGN Journal Club is in your ears again! 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 Gut, by Guo et al., early-life diet and risk of inflammatory bowel disease: A pooled study in two Scandinavian birth cohorts. This is the sort of thing that – thanks to the record-keeping in which the Northlands specialise – can’t be duplicated elsewhere but that indicates for us all how we can effectively address an aspect of disease. Fish, veggie and no sweet, sweet fizzy drinks for those babies if you want to reduce risk! It’s open-access so check it out -- you’ll be glad that you did.   
Closer to ESPGHAN home is Jake’s next selection – from J Pediatr Gastroenterol Nutr – Raghu et al., Impact of early immunosuppression on pediatric liver transplant outcomes within 1 year. This collaborative effort among a double handful of North American centres permits a rather sad compare-and-contrast exercise with states of care in Scandinavia and in the USA (yes, one Canadian participant).  Medical provision on a scattershot, pick-and-mix basis is almost by whim in North America, it seems, whilst the Nordic lands offer co-ordinated, well-reasoned, and well-assessed interventions that enable supranational fine-tuning. One wonders what a similar look-back in Europe would discover, nation by nation and centre by centre, and what the discoveries would permit setting right.  Not that our bedside tables aren’t already stacked with enough to read, mind you, but at the top of that teetering pile should be the most recent number of JPGN.  Please grab it and read the Raghu et al. article. It’s a call to action.  May European hepatologists soon answer that call!
 

Dr. Alex Knisely is talking today to Dr. Rut Anne Thomassen, of Oslo, who is a senior dietitian – one of only a few in the councils of ESPGHAN – and whose recent remit from ESPGHAN was to pull together a position paper that sets out for us all what is known in paediatric patients about a form of elimination-and- reïntroduction diet called FODMAP, an acronym that lists the classes of foodstuffs to be withdrawn. Some say that it works in some patients with irritable bowel syndrome . . . but consensus among caregivers as set out in Dr. Thomassen’s work is first, that one has to identify those patients carefully, and second, that how to do so is still unclear. Are you under pressure from families who want something, anything done, including the FODMAP diet, to help their children with abdominal pain – or with primary-care referrers who want something, anything done, including the FODMAP diet, to make those children your patients? Seek consultation from a dietitian, Dr. Thomassen advises, speaking for ESPGHAN. He or she can help you with screening those patients for FODMAP suitability, with educating families in what might be reasonably expected, and with guarding against inappropriate deployment of FODMAP – which carries its own not insubstantial risks.