title: Liver Failure From Ultra-Short Bowel Syndrome on the Intestinal Transplant Waiting List: A Retrospective Study.
abstract: BACKGROUND
  Patients with intestinal failure (IF) are candidates for intestinal transplantation (ITx). In Japan, these patients have few opportunities to undergo cadaveric ITx because of low rates of organ donation. The donor criteria and recipient priority for ITx are still unknown. We reviewed our cases of IF to investigate which patients should be prioritized for ITx.
  
  
  METHODS
  Patients with IF who were registered as candidates for cadaveric ITx between January 2010 and November 2015 in our institute were included in this retrospective study. Their data were gathered from their charts and analyzed.
  
  
  RESULTS
  Five patients were included. Their primary diseases included total colon aganglionosis (n = 1), chronic idiopathic intestinal pseudo-obstruction syndrome (n = 2), superior mesenteric vein embolization (n = 1), and graft loss after ITx (n = 1). Two patients died of liver failure (LF) during the waiting period. The remaining three are now alive and waiting for transplantation. The lengths of the remaining intestine were more than 20 cm in living cases but less than 20 cm in fatal cases. In the fatal cases, they had several episodes of catheter-related blood stream infection, which caused LF and acute renal failure.
  
  
  CONCLUSIONS
  We identified two patients with less than 20 cm residual small bowel who died after acute deterioration of liver function. Patients with ultra-short bowel could have a higher risk of LF. Therefore, they should be referred as soon as possible to a specialized hospital where ITx is a choice of treatment for IF.
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Noguchi
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan. Electronic address: ueno@pedsurg.med.osaka-u.ac.jp.
authors.forename: T
authors.initials: T
authors.lastname: Ueno
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Matsuura
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kodama
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Deguchi
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Umeda
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Yamamichi
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Nakahata
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Zenitani
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Takama
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Yamanaka
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Tazuke
authors.affiliation: Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Okuyama
mesh_terms: D000328:Adult; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007421:Intestine, Small / Q000637:transplantation*; D007564:Japan; D017093:Liver Failure / Q000453:epidemiology* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000150:complications* / Q000401:mortality; D016896:Treatment Outcome; D014850:Waiting Lists