Recent advances in the treatment of gastrointestinal motility disorders in children.

INTRODUCTION: Pediatric gastrointestinal motility disorders represent some of the most challenging clinical conditions with largely undefined pathogenetic pathways and therefore limited therapeutic options. Herein, we provide an overview of the recent advances in treatment options for these disorders and their clinical impact. AREAS COVERED: PubMed and Medline databases were searched for relevant articles related to the treatment of achalasia, esophageal atresia, gastroparesis, PIPO and constipation published between 2017 and 2022. In this article, we review and summarize recent advances in management of gastrointestinal motility disorders in children with a particular focus on emerging therapies as well as novel diagnostic modalities that help guide their application or develop new, more targeted treatments. EXPERT OPINION: Gastrointestinal motility disorders represent one of the most challenging conundrums in pediatric age and despite significant advances in investigative tools, the palette of treatment options remain limited. Overall, while pharmacological options have failed to bring a curative solution, recent advances in minimal invasive therapeutic and diagnostic techniques have emerged as potential keys to symptom and quality of life improvement, such as ENDOFLIP, POEM, cine-MRI, fecal microbiota transplantation.

Pancreatic family history does not predict disease progression but connotes alcohol consumption in adolescents and young adults with acute pancreatitis: Analysis of an international cohort of 2,335 patients.

Background: In pediatric acute pancreatitis (AP), a family history of pancreatic diseases is prognostic for earlier onset of recurrent AP (ARP) and chronic pancreatitis (CP). No evidence supports the same association in adult-onset pancreatitis. Age-specific reasons for familial aggregation are also unclear. We aimed to examine the prognostic role of pancreatic family history for ARP/CP and observe possible underlying mechanisms. Methods: We conducted a secondary analysis of the Hungarian Pancreatic Study Group's (HPSG) multicenter, international, prospective registry of patients with AP, both children and adults. We compared the positive family history and the negative family history of pancreatic diseases, in different age groups, and analyzed trends of accompanying factors. Chi-square and Fisher exact tests were used. Results: We found a higher rate of ARP/CP in the positive pancreatic family history group (33.7 vs. 25.9%, p = 0.018), peaking at 6-17 years. Idiopathic AP peaked in childhood in the positive family history group (75% 0-5 years) and was consistently 20-35% in the negative group. A higher rate of alcohol consumption/smoking was found in the positive groups at 12-17 years (62.5 vs. 15.8%, p = 0.013) and 18-29 years (90.9 vs. 58.1%, p = 0.049). The prevalence of diabetes and hyperlipidemia steadily rose with age in both groups. Conclusion: Positive family history most likely signifies genetic background in early childhood. During adolescence and early adulthood, alcohol consumption and smoking emerge-clinicians should be aware and turn to intervention in such cases. Contrary to current viewpoints, positive pancreatic family history is not a prognostic factor for ARP and CP in adults, so it should not be regarded that way.

Adequacy of scoring systems in diagnosing paediatric autoimmune hepatitis: retrospective study using a control group children with Hepatitis B infection.

AIM: To evaluate how correct is made the diagnosis of autoimmune hepatitis (AIH) in children, comparing the adults scoring systems - the 1999 revised scoring system by International Autoimmune Hepatitis Group and the 2007 Simplified Scoring System. The inaccuracies in diagnosis have been analysed. METHODS: The two scoring systems have been applied for 28 children with AIH pre-treatment and 28 with Hepatitis B infection - matched by sex and age, all diagnosed between 2010 and 2017 in an emergency children's hospital in Bucharest, Romania. They were retrospectively followed throughout the period assessed. RESULTS: Autoimmune hepatitis was present in 85.7% cases according to the 1999 revised score, respectively, 57.0% as per the 2007 simplified score. The 2007 simplified score led to false negative results when AIH was associated with another autoimmune disease (p = 0.009). Patients who did not undergo liver biopsy were associated with a downgraded diagnosis by the 2007 simplified score (p = 0.001). CONCLUSION: There is a need for a scoring system tailored to children's characteristics. Two compulsory components of the future score might be liver biopsy and associated autoimmune disorders. The existing scores could be used in children with consideration, preferably both at once and together with liver biopsy for increased accuracy.