The Association Between Calretinin and the Function of Ganglion Cells in Hirschsprung Disease

Hirschsprung Disease Clinical Trial

Official title:

The Association Between Calretinin and the Function of Ganglion Cells in Long Segment and Total Hirschsprung Disease

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04149093
• Other study ID #: 2020-2597
• Status: Recruiting
• Start date: May 1, 2020
• Est. completion date: December 1, 2021

Study information

• Verified date: March 2021
• Source: St. Justine's Hospital
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

This study aims to compare the outcomes of patients with long segment Hirschsprung disease or total colonic aganglionosis who had negative calretinin staining and positive ganglion cells on the proximal resection margins to those who had both positive findings.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 50
• Est. completion date: December 1, 2021
• Est. primary completion date: September 1, 2021
• Accepts healthy volunteers: No
• Gender: All
• Age group: N/A to 18 Months

Eligibility

Inclusion Criteria:

• Confirmed histopathological diagnosis of Hirschsprung disease from January 1, 1990 to August 31, 2019
• Long-segment Hirschsprung disease (type A and B)
• Total colonic aganglionosis (TCA)
• Surgery at CHUSJ
• Tissue blocks of proximal resection margin available for pathological analysis
• Minimum 1 month of postoperative follow-up

Exclusion Criteria:

• Short-segment Hirschsprung disease (conventional form, rectosigmoid junction)
• Total colonic with small bowel aganglionosis (TCSA)
• Surgery done at another institution
• Early deaths
• Lost to follow-up
• No documented post-operative outcome

Related Conditions & MeSH terms

• Ganglion
• Ganglion Cysts
• Hirschsprung Disease
• Hirschsprung Disease, Long-Segment
• Hypoganglionosis

Intervention

Diagnostic Test:
• Calretinin staining
A paraffin-embedded hematoxylinphloxin-safran sections will be performed on tissue specimens of proximal resection margins to evaluate the presence or absence of ganglion cells. Immunohistochemistry (IHC) will then be done on paraffin-embedded sections for calretinin staining.

Locations

Country	Name	City	State
Canada	CHU Sainte-Justine	Montréal	Quebec

Sponsors (1)

Lead Sponsor	Collaborator
St. Justine's Hospital

Country where clinical trial is conducted

Canada, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Long-term outcomes (Quality of life questionnaire)	To determine if calretinin has a role in the function of ganglion cells in long segment Hirschsprung disease and total colonic aganglionosis.; HAQL (Hirschsprung's disease and Anorectal malformations Quality of Life) questionnaire. For each item the response is scored from 0 to 3 and then, linearly transformed to a 0 (minimum value) to 100 (maximum value) scale. Higher score suggests a better quality of life.	1 year
Secondary	Surgical complications	Number of reoperations; Number of rebiopsies; Number of readmissions; Number of bowel obstructions; Number of bowel perforation; Number of anastomotic leaks; Number of anastomotic strictures; Number of fistula; Number of anal stenosis; Number of Hirschsprung-associated enterocolitis	1 year
Secondary	Functional outcomes	Presence of constipation (yes/no); Presence of diarrhea (yes/no); Presence of incontinence and soiling (yes/no); Presence of abdominal distension (yes/no); Presence of dependance on enemas/medications (yes/no)	1 year