title: Enteral autonomy, cirrhosis, and long term transplant-free survival in pediatric intestinal failure patients.
abstract: OBJECTIVE
  Patient selection for transplant evaluation in pediatric intestinal failure is predicated on the ability to assess long-term transplant-free survival. In light of trends toward improved survival of intestinal failure patients in recent decades, we sought to determine if the presence of biopsy-proven hepatic cirrhosis or the eventual achievement of enteral autonomy were associated with transplant-free survival.
  
  
  METHODS
  After IRB approval, records of all pediatric intestinal failure patients (parenteral nutrition (PN) >90 days) treated at a single intestinal failure center from February 2002 to September 2014 were reviewed. Chi-squared, Mann-Whitney, and log-rank testing were performed as appropriate.
  
  
  RESULTS
  Of 313 patients, 174 eventually weaned off PN. Liver biopsies were available in 126 patients (most common indication was intestinal failure associated liver disease, IFALD), and 23 met histologic criteria for cirrhosis. Transplant-free survival for the whole cohort of 313 patients was 94.7% at 1 year and 89.2% at 5 years. Among patients with liver biopsies, transplant-free survival in cirrhotics vs. noncirrhotics was 95.5% vs. 94.1% at one year and 95.5% vs. 86.7% at 5 years (P=0.29). Transplant-free survival in patients who achieved enteral autonomy compared with patients who remained PN dependent was 98.2% vs. 90.3% at one year and 98.2% vs. 76.9% at 5 years (P<0.001). There was no association between cirrhosis and eventual enteral autonomy (P=0.88).
  
  
  CONCLUSIONS
  Achieving enteral autonomy was associated with improved transplant-free survival in pediatric intestinal failure patients. There was no association between histopathological diagnosis of cirrhosis and transplant-free survival in the cohort. These data suggest that automatic transplant referral may not be required for histopathological diagnosis of cirrhosis alone, and that ongoing efforts aimed at achievement of enteral autonomy remain paramount in pediatric intestinal failure.
authors.affiliation: Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA; Department of Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Brenna S
authors.initials: BS
authors.lastname: Fullerton
authors.affiliation: Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA; Department of Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Eric A
authors.initials: EA
authors.lastname: Sparks
authors.affiliation: Department of Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Amber M
authors.initials: AM
authors.lastname: Hall
authors.affiliation: Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA; Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA.
authors.forename: Christopher
authors.initials: C
authors.lastname: Duggan
authors.affiliation: Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA; Department of Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Tom
authors.initials: T
authors.lastname: Jaksic
authors.affiliation: Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA; Department of Surgery, Boston Children's Hospital, Boston, MA. Electronic address: biren.modi@childrens.harvard.edu.
authors.forename: Biren P
authors.initials: BP
authors.lastname: Modi
mesh_terms: D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D004750:Enteral Nutrition; D005260:Female; D006801:Humans; D007223:Infant; D007410:Intestinal Diseases / Q000150:complications / Q000401:mortality / Q000628:therapy*; D007422:Intestines / Q000637:transplantation*; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications* / Q000473:pathology; D008297:Male; D010288:Parenteral Nutrition; D011379:Prognosis
keywords: Cirrhosis; IFALD; Intestinal failure; Intestinal transplant; Multivisceral transplant; Parenteral nutrition