期刊
ALIMENTARY PHARMACOLOGY & THERAPEUTICS
卷 55, 期 11, 页码 1414-1422出版社
WILEY
DOI: 10.1111/apt.16837
关键词
infantile-onset inflammatory bowel disease; interleukin 10; interleukin 10 receptor; haematopoietic stem cell transplantation
资金
- Haiju International Joint Lab Fund
This study retrospectively evaluated disease progression and clinical outcomes in 109 patients with interleukin-10 signalling deficiency. The results showed that there was no significant difference in survival probability between patients who received hematopoietic stem cell transplantation and those who did not. In non-transplanted patients, survival probability was significantly associated with perforation, ileus, and absence of thalidomide treatment.
Background Infantile-onset inflammatory bowel disease can be caused by defects in interleukin-10 signalling. The natural history and clinical outcomes of allogeneic haematopoietic stem cell transplantation, medical treatment and surgery have not been thoroughly described. Aims This study evaluates disease progression and clinical outcome in patients with interleukin-10 signalling deficiency. Methods One hundred and nine patients with interleukin-10 signalling deficiency were retrospectively reviewed from a single tertiary centre. The Kaplan-Meier method was applied to calculate probabilities of survival and interval between transplant and stoma closure. Results One hundred and nine patients were reviewed, and 102 patients were included in the survival analysis. One hundred and eight patients were identified with IL10RA mutations, and one patient harboured IL10RB mutation. Seventy-three patients received haematopoietic stem cell transplantation. The overall survival after transplantation was 64.2% (95% confidence interval, 52.8 to 75.6), and without transplantation, it was 47.5% (95% confidence interval, 14.8 to 80.2, P = 0.47). The median timeframe between transplant and stoma closure was 19.6 months. The probability of survival was significantly lower in patients with perforation (P < 0.001), ileus (P = 0.038) and without thalidomide treatment (P < 0.001) among patients who did not receive haematopoietic stem cell transplantation. The survival probability was not associated with timeframe between transplant and onset, graft source and genotypes. Conclusions The survival probability was not significantly different between patients with transplantation and the non-transplanted patients.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据