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

• ClinicalTrials.gov Identifier: NCT02216994
• Recruitment Status: Unknown
• First Posted: August 15, 2014
• Last Update Posted: October 28, 2014
• Sponsor: Tongji Hospital
• Collaborator: Jiangxi Province Children's Hospital
• Information provided by (Responsible Party): Jiexiong Feng, Tongji Hospital

Study Description

Brief Summary:

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.

Condition or disease	Intervention/treatment	Phase
Hirschsprung Disease	Drug: high dose lactulose; Behavioral: conservative treatment; Drug: paraffin oil	Not Applicable

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 Design

• Study Type: Interventional (Clinical Trial)
• Estimated Enrollment: 80 participants
• Allocation: N/A
• Intervention Model: Single Group Assignment
• Masking: None (Open Label)
• Primary Purpose: Diagnostic
• Official Title: 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
• Study Start Date: January 2013
• Estimated Primary Completion Date: December 2014
• Estimated Study Completion Date: June 2015

Arms and Interventions

Arm

Intervention/treatment

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 Name: 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 Name: 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 Name: Shilayou®

Outcome Measures

Primary Outcome Measures :

1. the predicting score calculation [ Time Frame: 6-12 months ]

   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.

Secondary Outcome Measures :

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

Eligibility Criteria

• Ages Eligible for Study: 1 Day to 3 Years (Child)
• Sexes Eligible for Study: All
• Accepts Healthy Volunteers: No

Criteria

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

Contacts and Locations

Contacts

Contact: Hongyi Zhang, MD; 27-83665209 ext 01186; zhanghongyidoc@foxmail.com

Locations

China, Hubei

Tongji Hospital; Recruiting

Wuhan, Hubei, China, 430030

Contact: Hongyi Zhang, MD 27-83665209 ext 01186 zhanghongyidoc@gmail.com

Principal Investigator: Jiexiong Feng, MD,PhD

Sub-Investigator: Jinshi Huang, MD

Sponsors and Collaborators

Tongji Hospital

Jiangxi Province Children's Hospital

Investigators

• Principal Investigator: Jiexiong Feng, MD, PhD; Tongji Medical College,Huazhong University of Science and Technology

More Information

• Responsible Party: Jiexiong Feng, Director of pediatric surgery department, Tongji Hospital
• ClinicalTrials.gov Identifier: NCT02216994
• Other Study ID Numbers: TJCD-D-13-00074; tj2014701 ( Other Identifier: Tongji Hospital )
• First Posted: August 15, 2014
• Last Update Posted: October 28, 2014
• Last Verified: October 2014

Keywords provided by Jiexiong Feng, Tongji Hospital:

Intestinal dysganglinosis; Hirschsprung disease; Hirschsprung disease allied disorders; diagnostic scoring system; Prospective study

Additional relevant MeSH terms:

Hirschsprung Disease; Digestive System Abnormalities; Digestive System Diseases; Megacolon; Colonic Diseases; Intestinal Diseases; Gastrointestinal Diseases; Congenital Abnormalities; Mineral Oil; Lactulose; Gastrointestinal Agents; Emollients; Dermatologic Agents