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Autor(en) / Beteiligte
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.
Sprache
Englisch
Identifikatoren
ISSN: 0148-6071
eISSN: 1941-2444
DOI: 10.1002/jpen.1164
Titel-ID: cdi_proquest_miscellaneous_2032421324

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