Paediatric Intestinal Pseudo-Obstruction: Evidence and Consensus-Based Recommendations from an ESPGHAN-Led Expert Group

Objectives: Chronic intestinal pseudo-obstructive (CIPO) conditions are considered the most severe disorders of gut motility. They continue to present significant challenges in clinical care despite considerable recent progress in our understanding of pathophysiology, resulting in unacceptable levels of morbidity and mortality. Major contributors to the disappointing lack of progress in paediatric CIPO include a dearth of clarity and uniformity across all aspects of clinical care from definition and diagnosis to management. In order to assist medical care providers in identifying, evaluating and managing children with CIPO, experts in this condition within the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as well as selected external experts, were charged with the task of developing a uniform document of evidence- and consensus-based recommendations. Methods: Ten clinically relevant questions addressing terminology, diagnostic, therapeutic, and prognostic topics were formulated. A systematic literature search was performed from inception to June 2017 using a number of established electronic databases as well as repositories. The approach of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) was applied to evaluate outcome measures for the research questions. Levels of evidence and quality of evidence were assessed using the classification system of the Oxford Centre for Evidence-Based Medicine (diagnosis) and the GRADE system (treatment). Each of the recommendations were discussed, finalized and voted upon using the nominal voting technique to obtain consensus. Results: This evidence- and consensus- based position paper provides recommendations specifically for chronic intestinal pseudo-obstruction in infants and children. It proposes these be termed paediatric intestinal pseudo-obstructive (PIPO) disorders to distinguish them from adult onset CIPO. The manuscript provides guidance on the diagnosis, evaluation, and treatment of children with PIPO in an effort to standardise the quality of clinical care and improve short and long-term outcomes. Key recommendations include the development of specific diagnostic criteria for PIPO, red flags to alert clinicians to the diagnosis and guidance on the use of available investigative modalities. The group advocates early collaboration with expert centres where structured diagnosis and management is guided by a multi-disciplinary team, and include targeted nutritional, medical and surgical interventions as well as transition to adult services. Conclusions: This document is intended to be used in daily practice from the time of first presentation and definitive diagnosis PIPO through to the complex management and treatment interventions such as intestinal transplantation. Significant challenges remain to be addressed through collaborative clinical and research interactions. Address correspondence and reprint requests to Dr. Nikhil Thapar, PhD, BM, MRCPCH, MRCP, BSc, Senior Lecturer and Honorary Consultant in Paediatric Gastroenterology, Great Ormond Street Hospital NHS Foundation Trust, Great Ormond Street, London, WC1N 3JH, United Kingdom (e-mail: n.thapar@ucl.ac.uk). Received 16 August, 2017 Accepted 19 February, 2018 DISCLAIMER: "ESPGHAN is not responsible for the practices of physicians and provides guidelines and position papers as indicators of best practice only. Diagnosis and treatment is at the discretion of physicians". Funding: Guideline development was financially supported by ESPGHAN. No support was received from industry. Conflicts of interest: NT has participated as clinical investigator, and/or advisory board member, and/or consultant, and/or speaker for Danone, Nutricia ELN and Mead Johnson. ES– No conflict of interest. MB has participated as a consultant for Shire, Sucampo, AstraZeneca, Norgine, Coloplast, Danone, Frieslandcampina, Sensus, Novolac. OB – No conflict of interest. JC- No conflict of interest. RDG has participated as a consultant for Shire, Sucampo, Coloplast, and Takeda. CDL has participated as a consultant for Merck, Nestle, Sucampo, IMHealth, QOL. CF has participated as clinical investigator, and consultant for Sucampo. GG – No conflict of interest. CK has received research support and continues to act as a paid consultant and speaker for Medtronic Inc. AS has participated as a clinical investigator, and/or advisory board member, and/or consultant, and/or speaker for Aboca, Angelini, Danone, D.M.G. Italy, Menarini, Miltè, Nestlé, Sucampo, and Valeas. YV has participated as a clinical investigator, and/or advisory board member, and/or consultant, and/or speaker for Abbott Nutrition, Aspen, Biogaia, Biocodex, Danone, Hero, Kabrita, Nestle Nutrition Institute, Nutricia, Mead Johnson Nutrition, Merck, Olygose, Orafti, Phacobel, Rontis, Sari Husada, United Pharmaceuticals, Wyeth and Yakult. © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

from Gastroenterology via xlomafota13 on Inoreader http://ift.tt/2GeYZkV
via IFTTT