More long-term data on the outcome of intestinal failure (IF) are available (Squire RH et al. J Pediatr 2012; 161: 723-8).
A retrospective analysis of infants <12 months (n=272) who were receiving parenteral nutrition (PN) for more than 60 days was performed by the Pediatric Intestinal Failure Consortium (PIFCon). This study took place between January 2000-December 2007.
- Etiologies: necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), atresia (27, 10%), volvulus (24, 9%), aganglionosis (11, 4%), other/multiple causes (94, 35%)
- Residual small bowel length in 144 patients was 41 cm (25-65.5cm).
- Catheter-related blood stream infections: 8.9 per 1000 catheter days. (Newer techniques like antibiotic & ethanol locks were not commonly used during study period.)
- Enteral autonomy continued into 5th year after study entry & occurred in 47%. Of the 154 patients who were alive without transplant, 27 remained on PN at the completion of the study period. Of the patients who acheived enteral autonomy, this occurred in 31% of initial cohort at 12 months, 40% by 24 months, 44% by 36 months, and 47% at 60 months.
- At 72 months, a high incidence of death, 27%, was noted in this cohort. 58 died without transplantation & 10 deaths occurred in patients after transplant. Sepsis was 2nd leading cause of death (after multisystem organ failure).
- At 72 months, cumulative percentage of intestinal transplantation was 26%.
Previous related blog entries:
- Four advances for intestinal failure | gutsandgrowth
- Optimizing lipids to minimize cholestasis | gutsandgrowth
- cycling | gutsandgrowth
- PNALD | gutsandgrowth
- cholestasis | gutsandgrowth
- PNAC, PNALD, and IFAC | gutsandgrowth
- More on ethanol locks | gutsandgrowth
- oley | gutsandgrowth
- CLMP–why some children are born with a short gut | gutsandgrowth