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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.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Calretinin staining | Diagnostic Test | 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. |
|
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Surgical complications |
| 1 year |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with Hirschsprung disease and a transition zone more proximal to the distal sigmoid
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU Sainte-Justine | Recruiting | Montreal | Quebec | H3T 1C5 | Canada |
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| ID | Term |
|---|---|
| D006627 | Hirschsprung Disease |
| D045888 | Ganglion Cysts |
| ID | Term |
|---|---|
| D004065 | Digestive System Abnormalities |
| D004066 | Digestive System Diseases |
| D008531 | Megacolon |
| D003108 | Colonic Diseases |
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| ID | Term |
|---|---|
| D007150 | Immunohistochemistry |
| ID | Term |
|---|---|
| D006651 | Histocytochemistry |
| D003584 | Cytological Techniques |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
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| Functional outcomes |
|
| 1 year |
| D007410 |
| Intestinal Diseases |
| D005767 | Gastrointestinal Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D003560 | Cysts |
| D009369 | Neoplasms |
| D017520 | Mucinoses |
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D003933 | Diagnosis |
| D006652 | Histological Techniques |
| D008919 | Investigative Techniques |
| D007158 | Immunologic Techniques |