Comorbidity of inflammatory bowel disease with atypical hemolytic uremic syndrome in pediatric patients.

• Type of Material: Article
• Authors: Hyunjung Stella Shin, Emory University; Carla M. Nester, University of Iowa; Bradley P. Dixon, University of Colorado
• Language: English
• Date: 2019
• Publisher: Dustri-verlag Dr. Karl Feistle
• Publication Version: Final Published Version
• Copyright Statement: © Dustri-Verlag Dr. K. Feistle
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.5414/CNCS109511
• Title of Journal or Parent Work: Clinical Nephrology. Case Studies
• ISSN: 2196-5293
• Volume: 7
• Issue: 1
• Start Page: 35
• End Page: 40
• Grant/Funding Information: All authors declare that they receive no funding support for the work contained within this manuscript.
• Keywords: complement; atypical hemolytic uremic syndrome; inflammatory bowel disease
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a form of thrombotic microangiopathy mediated by dysregulation of the alternative complement pathway. Complement-amplifying conditions such as respiratory and gastrointestinal infections, pregnancy, malignancy, and systemic autoimmune diseases such as systemic lupus erythematosus have been associated with the clinical manifestation of aHUS. Inflammation of the gastrointestinal tract is a potent stimulus for complement activation, and we describe a series of three pediatric patients with aHUS and comorbidity of inflammatory bowel disease (IBD). In two of the three cases, the diagnosis of aHUS preceded the diagnosis of IBD, perhaps suggesting a mechanistic link between complement dysregulation and thrombotic microangiopathy in the gastrointestinal tract and the ensuing inflammatory changes of IBD.