Isabelle Scheers

Pain is often the presenting symptom of acute pancreatitis (AP) and has a prognostic significance, with poorly treated pain affecting patient outcomes. Despite this, there are currently no international recommendations for pain management in patients admitted with AP. This current guideline was developed following the United European Gastroenterology framework for the development of high‐quality clinical guidelines. Nine working groups were formed to develop statements on pain management in AP from admission to discharge. After systematic literature reviews, the evidence was evaluated according to the Grading of Recommendations Assessment, Development, and Evaluation methodology, as appropriate. Statements and comments were developed by the working groups and voted using three rounds of Delphi method. The guidelines concluded that acute postoperative pain management guidelines are applicable for treating acute pain due to AP. The numerical rating scale is preferred for pain assessment because of better compliance. Opioids currently form the cornerstone of severe pain management. Potent opioids such as buprenorphine and pentazocine provide superior pain relief over non‐opioid medications and decrease the need for rescue analgesia. Current evidence does not consistently demonstrate harm from opioids in AP. In patients with severe pain, safety concerns should not delay the timely use of opioids to ensure adequate pain management. Specific recommendations concerning the use of epidural analgesia, acupuncture and management of pain in the paediatric population and during pregnancy are provided based on evidence and expert opinions. Finally, the unmet needs for future research were discussed and proposed.

Background: While pediatric liver transplantation has markedly improved long‐term survival, adult outcomes remain insufficiently explored beyond graft function, particularly regarding mental health and disease understanding−key dimensions of meaningful survival. To this aim, we evaluated psychosocial, behavioral, and lifestyle outcomes of adults who received a liver transplant during childhood, using validated tools. Methods: Fifty adult patients transplanted before 18 years of age at Cliniques Universitaires Saint‐Luc completed an anonymous online questionnaire including measures of anxiety (STAI‐Trait), depression (BDI‐SF), and alcohol use (AUDIT), as well as questions on lifestyle, treatment adherence, and disease knowledge. Results: The results showed that clinically relevant anxiety and depressive symptoms were reported in 49% and 33% of respondents, respectively. Problematic alcohol use was found in 8% of participants, and 24% reported never receiving medical counseling about alcohol risks. Only 31% could correctly explain the pathophysiology of their liver disease, and 36% were unaware of its transmissible genetic nature. Despite these challenges, 76% were professionally or academically active. Conclusion: These findings highlight the persistence of high psychological distress and limited disease understanding into adulthood, supporting the integration of mental health screening and patient education into long‐term post‐transplant care.

Pancreatic trauma in children is rare but may result in severe complications, particularly in injuries involving duct transection. Optimal management—surgical versus conservative—remains debated. We aimed to compare long-term complications between both management approaches. This multicenter retrospective and prospective study analyzed children (< 18 years) with grade III–V pancreatic trauma treated between 2009 and 2019 at Belgian Level 1 trauma centers. Patient characteristics, management, and outcomes were assessed with long-term follow-up. Among 31 patients, 35% underwent surgery and 65% were managed conservatively. Pseudocyst formation was significantly higher in the non-operative group (85%) vs. surgical (18%, p = 0.0013). Long-term complications included pancreatic atrophy (35%), recurrent pancreatitis (19%), and exocrine dysfunction; no endocrine insufficiency was reported. Conclusion: Surgical and conservative management of grade III and IV trauma are safe for pediatric pancreatic trauma, though pseudocysts are common in the conservative approach and often require endoscopic drainage. Ongoing follow-up is essential to monitor for long-term complications such as pancreatitis and pancreatic atrophy. What is Known: • Non-operative management of pancreatic trauma with complete duct transection is associated with a higher risk of pseudocyst formation compared with operative management. • Long-term comparative studies on outcomes of patients with pancreatic trauma and complete pancreatic duct transection are scarce, limited in time, and often retrospective. What is New: • Regardless of the initial management, children with grade III, IV, and V pancreas trauma are at risk to develop very long-term complications such as pancreatitis, pancreas atrophy, and exocrine insufficiency; clinical follow-up is therefore warranted.

Background: While pancreatic cysts have been described in syndromic ciliopathies, the pancreas is not commonly recognized as a target organ. However, several ciliary gene knockout mouse models develop a pancreatic phenotype combining acinar atrophy and adipocyte accumulation, hereby called adipopancreatosis, suggesting a link between ciliary dysfunction and pancreatic disease. Objective: We investigated whether mutations in ciliopathy-associated genes are linked to pancreatic dysfunction in humans. Design: We analyzed a cohort of 341 patients with pediatric-onset pancreatic anomalies and characterized the pancreatic phenotype of new mouse models with conditional Nphp3 inactivation or bearing Nphp3 mutations recapitulating human mutations. In patients, pancreatic fat content was quantified using Dixon-MRI. Results: Mutations in the cilium-related HNF1B and NPHP3 were identified in patients presenting with both renal and pancreatic dysfunction. Nphp3 mutant mice developed acinar atrophy, adipopancreatosis, and moderate inflammation. Adipocytes in the pancreas exhibited a white adipocyte-like profile and likely originated from mesothelial-derived fibroblasts. Reduced numbers and altered length of ductal cilia were monitored. Interestingly, secretory canaliculi, typically unnoticed structures found within and between acinar cells and connected to the acinar lumen, exhibited a microcystic morphology. Consistent with the mouse phenotype, Dixon-MRI revealed significantly increased pancreatic fat content in patients with HNF1B and NPHP3 mutations. Conclusion: We describe a previously unrecognized pancreatic manifestation of ciliopathies, which we name ciliogenic pancreatopathy. Patients with known ciliopathy-causing mutations should be evaluated for this pancreatic condition, particularly those with kidney disease, as concomitant exocrine pancreatic insufficiency may further compromise renal function or the outcome of kidney graft. What is already known on this topic: Ciliopathies, resulting from defects in primary cilia, are genetic disorders primarily affecting the kidney and liver. Pancreatic cysts have been sporadically reported in syndromic ciliopathies. The pancreas is not currently recognized as a major target organ of ciliary dysfunction. A clear link between ciliary gene mutations and pancreatic anomalies is still unknown. Animal studies have suggested a possible association between ciliary dysfunction and pancreatic anomalies. What this study adds: Identifies HNF1B and NPHP3 mutations as genetic causes of a pancreatic phenotype characterized by acinar atrophy and adipose replacement (adipopancreatosis). Demonstrates the presence of defective ductal cilia and moderate inflammation in the pancreas of Nphp3 mutant mice. Reveals that secretory canaliculi in the exocrine pancreas of Nphp3 mutant mice acquire a microcystic morphology. Shows that patients with HNF1B or NPHP3 mutations have significantly increased pancreatic fat content by Dixon-MRI. Defines a new disease entity, ciliogenic pancreatopathy, as a pancreatic manifestation of ciliopathies. How this study might affect research, practice or policy: Establishes the pancreas as a novel and clinically relevant target of ciliopathies. Expands the phenotypic spectrum of HNF1B- and NPHP3-related diseases to include exocrine pancreatic dysfunction. Suggests that patients with ciliopathy-causing mutations should be evaluated for exocrine pancreatic insufficiency. Highlights the need to consider pancreatic function monitoring in kidney disease and transplant settings. Opens new research avenues into the role of primary cilia in pancreatic homeostasis and disease.

Background/Objectives: Liver retransplantation (reLT) is the only option for pediatric patients experiencing graft loss. Despite recent advancements in surgical techniques and perioperative management, it remains a high-risk procedure. Our aim is to describe our experience in pediatric reLT, focusing on the technical aspects and surgical challenges. Methods: We systematically analyzed surgical reports from pediatric reLT performed at our center between 2006 and 2023 to identify recurrent intraoperative findings and specific surgical techniques. We focused on challenges encountered during different phases of reLT, including hepatectomy, vascular, and biliary reconstruction. Additionally, we compared patient and graft survival rates among different groups. Results: During the study period, 23 children underwent 25 reLT procedures at our center. Major surgical challenges included complex hepatectomy and vascular reconstructions, necessitating tailored approaches. Our analysis shows that patient and graft survival were significantly lower for reLT compared to primary transplantation (p = 0.002). Early reLT had a significantly lower graft survival compared to late reLT (p = 0.002), although patient survival was comparable (p = 0.278). Patient and graft survival rates were comparable between the first and second reLT (p = 0.300, p = 0.597). Patient survival tended to be higher after living-donor liver transplantation (LDLT) compared to deceased-donor liver transplantation (DDLT), although the difference was not statistically significant (p = 0.511). Conclusions: Pediatric reLT involves significant technical challenges and lower survival rates. Advances in perioperative management are crucial for improving outcomes. Further research is needed to optimize surgical strategies and evaluate the long-term benefits of LDLT in pediatric reLT.

Duodenal obstruction (DO) is an uncommon complication of pancreatitis. It has been described in groove and severe acute and chronic pancreatitis in adults but, to the best of our knowledge, it has not yet been reported in pediatric acute pancreatitis. Current guidelines comment on management of several early and late‐onset complications, but DO is not mentioned. We describe two patients with acute necrotizing pancreatitis who presented with several complications including walled‐off necrosis and DO. In adults, DO is generally managed with adapted nutrition but may require surgical bypass, such as gastroenterostomy. Our patients were managed conservatively and fully recovered 2 months after DO diagnosis. DO may require lengthy hospitalizations and markedly restrict patients' quality of life; however, prolonged conservative treatment was effective in our patients and should be considered even in severe pediatric cases.

Congenital exocrine pancreatic insufficiency is a rare condition. In a vast majority of patients, exocrine dysfunction occurs as part of a multisystemic disease, the most prevalent being cystic fibrosis and Shwachman-Bodian-Diamond syndrome. Recent fundamental studies have increased our understanding of the pathophysiology of these diseases. Exocrine pancreatic dysfunction should be considered in children with failure to thrive and fatty stools. Treatment is mainly supportive and consists of pancreatic enzyme replacement and liposoluble vitamins supplementation.