TY - JOUR
T1 - Pediatric Intestinal Pseudo-Obstruction
T2 - An International Survey on Diagnostic and Management Strategies in the European Reference Network for Rare Inherited and Congenital Anomalies Intestinal Failure Teams
AU - Mutanen, Annika
AU - Demirok, Aysenur
AU - Wessel, Lucas
AU - Tabbers, Merit
N1 - Publisher Copyright: © 2023 Lippincott Williams and Wilkins. All rights reserved.
PY - 2023/7/1
Y1 - 2023/7/1
N2 - Objectives: Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical, and surgical care while available evidence is scarce. The aim of this study was to outline the current diagnostic and management strategies in intestinal failure (IF) teams of the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) and to compare these practices to the latest PIPO international guidelines. Methods: An online survey on institutional diagnostic and management strategies of PIPO was conducted among the ERNICA IF teams. Results: In total, 11 of 21 ERNICA IF centers from 8 countries participated. On average, 64% of teams had ≥6 and 36% had 1-5 PIPO patients under active follow-up. In total, 80 of 102 PIPO patients were parenteral nutrition (PN) dependent while each IF team had median 4 (range 0-19) PN dependent PIPO patients under follow-up. On average, each center received 1-2 new PIPO patients per year. Diagnostics mostly followed current guidelines while medical and surgical management strategies were diverse. Conclusions: Numbers of PIPO patients are low and management strategies are diverse among ERNICA IF teams. To improve PIPO patient care, regional reference centers with specialized multidisciplinary IF teams and continuous collaboration across centers are needed.
AB - Objectives: Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical, and surgical care while available evidence is scarce. The aim of this study was to outline the current diagnostic and management strategies in intestinal failure (IF) teams of the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) and to compare these practices to the latest PIPO international guidelines. Methods: An online survey on institutional diagnostic and management strategies of PIPO was conducted among the ERNICA IF teams. Results: In total, 11 of 21 ERNICA IF centers from 8 countries participated. On average, 64% of teams had ≥6 and 36% had 1-5 PIPO patients under active follow-up. In total, 80 of 102 PIPO patients were parenteral nutrition (PN) dependent while each IF team had median 4 (range 0-19) PN dependent PIPO patients under follow-up. On average, each center received 1-2 new PIPO patients per year. Diagnostics mostly followed current guidelines while medical and surgical management strategies were diverse. Conclusions: Numbers of PIPO patients are low and management strategies are diverse among ERNICA IF teams. To improve PIPO patient care, regional reference centers with specialized multidisciplinary IF teams and continuous collaboration across centers are needed.
KW - ERNICA
KW - children
KW - home parenteral nutrition
KW - multidisciplinary team
KW - parenteral nutrition
KW - pediatric pseudo-obstruction syndrome
UR - http://www.scopus.com/inward/record.url?scp=85163427316&partnerID=8YFLogxK
U2 - https://doi.org/10.1097/MPG.0000000000003788
DO - https://doi.org/10.1097/MPG.0000000000003788
M3 - Article
C2 - 37027146
SN - 0277-2116
VL - 77
SP - 24
EP - 30
JO - Journal of pediatric gastroenterology and nutrition
JF - Journal of pediatric gastroenterology and nutrition
IS - 1
ER -