Abstract: Purpose: We elucidated the life-threatening risk factors for intestinal failure (IF) and characterized the role of intestinal transplantation (ITx) in affected patients. Methods: We conducted a retrospective review of 38 patients with short bowel (SB) and 19 with motility disorders (MD). The SB patients were divided into three categories according to the length of their residual small bowel and the presence of the ileocecal valve. The four disease subcategories were grouped into two categories: low-risk category (mild and moderated SB) and high-risk category (extensive SB and MD). The age at the introduction of parenteral nutrition (PN) was <1 year in 50 patients (infant group, IG) and 1-15 years in 7 patients (pediatric group, PG). Results: Enteral autonomy was rarely achieved in the high-risk category (p < 0.0001). IG was associated with a higher incidence of developing intestinal failure-associated liver disease (IFALD) (p = 0.004). Eight patients died, due to IFALD in four, sepsis in three and acute heart failure in one. Twenty-eight patients (49 %) are currently alive without PN, including four after ITx. Conclusion: The treatment of high-risk IF is still challenging. Inclusion of ITx in appropriate timing, along with aggressive medical, nutritional and surgical management, may reduce advanced morbidity and mortality of high-risk IF.
- Intestinal failure
- Intestinal failure-associated liver disease
- Intestinal rehabilitation
- Intestinal transplantation
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health