ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease

Show full item record



Orphanet Journal of Rare Diseases. 2020 Jun 25;15(1):164

Title: ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease
Author: Kyrklund, Kristiina; Sloots, Cornelius E J; de Blaauw, Ivo; Bjørnland, Kristin; Rolle, Udo; Cavalieri, Duccio; Francalanci, Paola; Fusaro, Fabio; Lemli, Annette; Schwarzer, Nicole; Fascetti-Leon, Francesco; Thapar, Nikhil; Johansen, Lars S; Berrebi, Dominique; Hugot, Jean-Pierre; Crétolle, Célia; Brooks, Alice S; Hofstra, Robert M; Wester, Tomas; Pakarinen, Mikko P.
Publisher: BioMed Central
Date: 2020-06-25
Language: en
Abstract: Abstract Background Hirschsprung’s disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to assist clinical decision-making regarding diagnostics and management. Aims This guideline aims to cover the diagnostics and management of rectosigmoid HSCR up to adulthood. It aims to describe the preferred approach of ERNICA, the European Reference Network for rare inherited and congenital digestive disorders. Methods Recommendations within key topics covering the care pathway for rectosigmoid HSCR were developed by an international workgroup of experts from 8 European countries within ERNICA European Reference Network from the disciplines of surgery, medicine, histopathology, microbiology, genetics, and patient organization representatives. Recommendation statements were based on a comprehensive review of the available literature and expert consensus. AGREE II and GRADE approaches were used during development. Evidence levels and levels of agreement are noted. Results Thirty-three statements within 9 key areas were generated. Most recommendations were based on expert opinion. Conclusion In rare or low-prevalence diseases such as HSCR, there remains limited availability of high-quality clinical evidence. Consensus-based guidelines for care are presented.
Subject: Rectosigmoid Hirschsprung’s disease

Files in this item

Total number of downloads: Loading...

Files Size Format View
13023_2020_Article_1362.pdf 658.4Kb PDF View/Open

This item appears in the following Collection(s)

Show full item record