A New Scoring System Improves Diagnostic Accuracy of Intestinal Dysganglionosis --a Prospective Study

Efficacy and Accuracy of a New Diagnostic Scoring System to Differentiate Hirschsprung Disease From Hirschsprung Disease Allied Disorders in the Patients With Suspected Intestinal Dysganglinosis: a Prospective Study

1. The investigators previously reported a simple diagnostic scoring system to differentiate Hirschsprung disease (HD) from Hirschsprung disease allied disorders (HAD) in the patients with suspected intestinal dysganglionosis. In the retrospective study, the investigators concluded that the patients with a predicting score of more than 5 are more likely to be diagnosed with HD, whereas a score less than 5 is mostly indicative of HAD.
2. Since it is essential to confirm the accuracy and efficacy of the scoring system in a prospective manner before it is used as a standard procedure, this prospective study is designed and performed.

Study Overview

• Status: Unknown
• Conditions: Hirschsprung Disease
• Intervention / Treatment: Drug: high dose lactulose; Behavioral: conservative treatment; Drug: paraffin oil

Detailed Description

1. Patients have hard or firm stools for 2 or less per week, and with ages from newborn to 3 years old are recruited in this study.
2. The recruited patients is undergoing preoperative work-up including barium enema (BE), anorectal manometry (ARM) and histochemical acetylcholinesterase (AChE) staining of rectal mucosa. Known risk factors for intestinal dysganglionosis (IDs) are recorded. The predicting score is calculated by summing the scores of the risk factors and 3 preoperative tests.
3. The patients with a predicting score of more than 5 are diagnosed with HD, and are performed surgery to remove the aganglionic bowel.
4. The patients with a score less than 5 are mostly indicative of HAD, and receive conservative therapies that included colonic irrigation, enema, high dose lactulose, and oral paraffin oil for at least 6 months. When there is no clinical improvement, patients are consented for surgical procedures to remove the dysganglionic bowel segments.

• Study Type: Interventional
• Enrollment (Anticipated): 80
• Phase: Not Applicable

Contacts and Locations

Study Locations

• China
  • Hubei
    • Wuhan, Hubei, China, 430030
      • Recruiting
      • Tongji Hospital
      • Contact: Hongyi Zhang, MD; Phone Number: 01186 27-83665209; Email: zhanghongyidoc@gmail.com
      • Principal Investigator: Jiexiong Feng, MD,PhD
      • Sub-Investigator: Jinshi Huang, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 3 years to 5 months (Child)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Hard or firm stools for 2 or less per week
• Age are from newborn to 3 years old

Exclusion Criteria:

• Children>3 years of age
• Patients presented severe inflammation or malnutrition, unconsciousness or perforation of intestine

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Diagnostic
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm

Intervention / Treatment

Other: surgery treatment; The patients with a predicting score of more than 5 are diagnosed with HD, and are performed surgery to remove the aganglionic bowel. The patients with a score less than 5 are mostly indicative of HAD, and receive conservative treatments that included colonic irrigation, enema, high dose lactulose, and oral paraffin oil for at least 6 months. When there is no clinical improvement, patients are consented for surgical procedures to remove the dysganglionic bowel segments. one stage pull through procedure to remove the dysganglionic bowel segments.

Drug: high dose lactulose; The patients with a score less than 5 are mostly indicative of HAD, and receive conservative therapies that included colonic irrigation, enema, high dose lactulose, and oral paraffin oil for at least 6 months. When there is no clinical improvement, patients are consented for surgical procedures to remove the dysganglionic bowel segments.; Other Names: Duphalac®; Behavioral: conservative treatment; The patients with a score less than 5 are mostly indicative of HAD, and receive conservative therapies that included colonic irrigation, enema, high dose lactulose, and oral paraffin oil for at least 6 months. When there is no clinical improvement, patients are consented for surgical procedures to remove the dysganglionic bowel segments.; Other Names: colonic irrigation; Drug: paraffin oil; The patients with a score less than 5 are mostly indicative of HAD, and receive conservative therapies that included colonic irrigation, enema, high dose lactulose, and oral paraffin oil for at least 6 months. When there is no clinical improvement, patients are consented for surgical procedures to remove the dysganglionic bowel segments.; Other Names: Shilayou®

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
the predicting score calculation	The recruited patients is undergoing preoperative work-up including barium enema (BE), anorectal manometry (ARM) and histochemical acetylcholinesterase (AChE) staining of rectal mucosa. Known risk factors for IDs are recorded. The predicting score is calculated by summing the scores of the risk factors and 3 preoperative tests. The patients with a predicting score of more than 5 are diagnosed with HD, and are performed surgery to remove the aganglionic bowel. The patients with a score less than 5 are mostly indicative of HAD, and receive conservative therapies that included colonic irrigation, enema, high dose lactulose, and oral paraffin oil for at least 6 months. When there is no clinical improvement, patients are consented for surgical procedures to remove the dysganglionic bowel segments.	6-12 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
pathological diagnosis	The intestinal specimens were reviewed by 3 experienced pathologists all of whom had participated in a consensus meeting on diagnostic criteria of IDs.	12-18 months

Collaborators and Investigators

• Sponsor: Tongji Hospital
• Collaborators: Jiangxi Province Children's Hospital
• Investigators: Principal Investigator: Jiexiong Feng, MD, PhD, Tongji Medical College,Huazhong University of Science and Technology

Study record dates

Study Major Dates

• Study Start: January 1, 2013
• Primary Completion (Anticipated): December 1, 2014
• Study Completion (Anticipated): June 1, 2015

Study Registration Dates

• First Submitted: August 6, 2014
• First Submitted That Met QC Criteria: August 14, 2014
• First Posted (Estimate): August 15, 2014

Study Record Updates

• Last Update Posted (Estimate): October 28, 2014
• Last Update Submitted That Met QC Criteria: October 27, 2014
• Last Verified: October 1, 2014

More Information

Terms related to this study

• Keywords: Prospective study; Hirschsprung disease; Intestinal dysganglinosis; Hirschsprung disease allied disorders; diagnostic scoring system
• Additional Relevant MeSH Terms: Digestive System Diseases; Congenital Abnormalities; Gastrointestinal Diseases; Colonic Diseases; Intestinal Diseases; Digestive System Abnormalities; Megacolon; Hirschsprung Disease; Gastrointestinal Agents; Dermatologic Agents; Emollients; Lactulose; Mineral Oil
• Other Study ID Numbers: TJCD-D-13-00074; tj2014701 (Other Identifier: Tongji Hospital)