Hirschsprung Disease
Conditions
Keywords
Intestinal dysganglinosis, Hirschsprung disease, Hirschsprung disease allied disorders, diagnostic scoring system, Prospective study
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.
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.
Interventions
DRUGhigh 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.
BEHAVIORALconservative 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.
DRUGparaffin 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.
Sponsors
Jiangxi Province Children's Hospital
Tongji Hospital
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
1 Days to 3 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| the predicting score calculation | 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
| Measure | Time frame | Description |
|---|---|---|
| pathological diagnosis | 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. |
Countries
China
Contacts
Primary ContactHongyi Zhang, MD
Outcome results
None listed