Identifying risk factors for enterocolitis in children with Hirschsprung disease

Identification of Genetic, Immunologic and Microbial Markers of Hirschsprung Associated Enterocolitis in Children With Hirschsprung Disease

Observational Cedars-Sinai Medical Center · NCT02193685

This study is trying to find out what genetic and other factors might lead to enterocolitis in children with Hirschsprung disease to help improve their care.

Quick facts

Study typeObservational
Enrollment400 (estimated)
Ages1 Day to 17 Years
SexAll
SponsorCedars-Sinai Medical Center Academic / other
Locations1 site (Los Angeles, California)
Trial IDNCT02193685 on ClinicalTrials.gov

What this trial studies

This study aims to identify genetic, immunologic, and microbial markers that influence the development of Hirschsprung Associated Enterocolitis (HAEC) in children diagnosed with Hirschsprung Disease (HD). It will recruit 200 patients with HD who do not have HAEC and 200 patients who do, collecting clinical and demographic data along with biological samples for genetic analysis. The research will involve collaboration among multiple centers to ensure a comprehensive understanding of the factors contributing to HAEC. The goal is to enhance the understanding of this condition and improve patient outcomes through better risk assessment.

Who should consider this trial

Good fit: Ideal candidates for this study are children aged 0 to 17 with a confirmed diagnosis of Hirschsprung disease.

Not a fit: Patients with intestinal neuronal dysplasia or pseudo-obstruction may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to improved identification and management of children at risk for developing HAEC, potentially reducing severe complications.

How similar studies have performed: While there is emerging data on HAEC, this study represents a novel approach to understanding its genetic and microbial underpinnings.

Eligibility criteria

Show full inclusion / exclusion criteria
Inclusion Criteria:

* 1. Males and females of all ages with a confirmed diagnosis of HD based on standardized histological criteria. Only Males and females ages 0 to 17 with a confirmed diagnosis of HD based on standardized histological criteria will be enrolled at CSMC.
* 2. Able to provide written informed assent if between the ages of 7 and 17. If age 6 and under, able to participate with parental permission.
* 3. Have consented to have specimens tested for genetics, immune responses, stool microflora.

Case Ascertainment:

All patients with a confirmed diagnosis of HD are eligible for enrollment. A diagnosis of HD for this study will require:

* 1)Documented histopathology showing absence of ganglion cells and is consistent with the diagnosis of HD.

Exclusion Criteria:

* 1. Intestinal neuronal dysplasia
* 2. Pseudo-obstruction

Where this trial is running

Los Angeles, California

Study contacts

How to participate

  1. Review the eligibility criteria above with your treating physician.
  2. Visit the official trial page on ClinicalTrials.gov for the most current contact information and recruitment status.
  3. Contact the listed study coordinator or principal investigator to request pre-screening. Pre-screening is free and never obligates you to enroll.
Conditions Hirschsprung DiseaseEnterocolitisHirschsprung Associated EntrocolistisInflammatory Bowel DiseaseChildren
Last reviewed 2026-06-13 by the Find a Trial editorial team. Information on this page is for educational purposes and is not medical advice. Always consult qualified healthcare professionals about clinical trial participation.