Details

Autor(en) / Beteiligte

• Merras‐Salmio, Laura
• Mutanen, Annika
• Ylinen, Elisa
• Rintala, Risto
• Koivusalo, Antti
• Pakarinen, Mikko P.

Titel

Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984–2017

Ist Teil von

• JPEN. Journal of parenteral and enteral nutrition, 2018-11, Vol.42 (8), p.1304-1313

Ort / Verlag

United States

Erscheinungsjahr

2018

Quelle

Wiley Online Library

Beschreibungen/Notizen

• Background: Pediatric‐onset intestinal failure (IF) remains a severe illness with life‐threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades. Methods: All children with IF who required parenteral nutrition (PN) >2 months or small‐intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses. Results: In total, 100 patients with median PN duration of 1.2 (interquartile range, 0.4–3.5) years were identified. Causes of IF were short bowel syndrome (SBS; n = 78), primary intestinal motility disorders (n = 14), and congenital intestinopathies (n = 8). Patients with SBS had median 40 (25–60) cm of small bowel remaining. Overall, Kaplan‐Meier 5‐ and 10‐year weaning‐off estimates were 67% (95% CI, 57–77) and 73% (95% CI, 63–84), respectively. Weaning off PN was predicted by remaining bowel anatomy, multidisciplinary treatment era, and absence of immune deficiency. Catheter‐related bloodstream infections decreased from 1.4 to 0.6/1000 PN days (P = .0003) with systematic use of taurolidine locks. None had progressive liver disease. Thirty‐one percent of patients with SBS underwent autologous intestinal reconstructive surgery. Five patients received and 2 were listed for isolated intestinal transplantation. Eight patients died, and overall 15‐year survival rate estimate was 91% (95% CI, 85–98). Conclusions: Despite reassuring rates of survival and weaning off PN, long‐term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging.