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Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984–2017
Author(s) -
MerrasSalmio Laura,
Mutanen Annika,
Ylinen Elisa,
Rintala Risto,
Koivusalo Antti,
Pakarinen Mikko P.
Publication year - 2018
Publication title -
journal of parenteral and enteral nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.935
H-Index - 98
eISSN - 1941-2444
pISSN - 0148-6071
DOI - 10.1002/jpen.1164
Subject(s) - medicine , short bowel syndrome , parenteral nutrition , interquartile range , weaning , enteral administration , intestinal failure , retrospective cohort study , single center , pediatrics , gastroenterology , surgery
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.

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