The Association Between Calretinin and the Function of Ganglion Cells in Long Segment and Total Hirschsprung Disease
试验速览
- 阶段
- 不适用
- 发起方
- 入组人数
- 50
- 试验地点
- 1
- 主要终点
- Long-term outcomes (Quality of life questionnaire)
研究概览
简要总结
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Retrospective
入排标准
- 年龄范围
- 0 Years 至 18 Months（Child）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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
排除标准
- •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
结局指标
主要结局
Long-term outcomes (Quality of life questionnaire)
时间窗: 1 year
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.
次要结局
- Functional outcomes(1 year)
- Surgical complications(1 year)
研究者
Nelson Piche
Principal Investigator
St. Justine's Hospital