title: Successful rehabilitation in pediatric ultrashort small bowel syndrome.
abstract: OBJECTIVE
  To examine treatment outcomes in pediatric patients with ultrashort small bowel (USSB) syndrome in an intestinal rehabilitation program (IRP).
  
  
  METHODS
  We reviewed IRP records for 2001-2011 and identified 28 children with USSB (≤ 20 cm of small bowel). We performed univariate analysis using the Fisher exact test and Wilcoxon rank-sum test to compare characteristics of children who achieved parenteral nutrition (PN) independence with intact native bowel and those who did not. Growth, nutritional status, and hepatic laboratory test results were compared from the time of enrollment to the most recent values using the Wilcoxon signed-rank test.
  
  
  RESULTS
  Of the 28 patients identified, 27 (96%) survived. Almost one-half (48%) of these survivors achieved PN independence with their native bowel. The successfully rehabilitated patients were more likely to have an intact colon and ileocecal valve (P = .01). Significant improvements in PN kcal/kg, total bilirubin, and height and weight z-scores were seen in all patients, but serum hepatic transaminase levels did not improve in the nonrehabilitated patients.
  
  
  CONCLUSIONS
  Enrollment in an IRP provides an excellent probability of survival for children with USSB. The presence of an intact ileocecal valve and colon are positively associated with rehabilitation in this population, but are not requisite. Approximately one-half of patients with USSB can achieve rehabilitation, with a median time to PN independence of less than 2 years. The USSB population can attain reduced PN dependence, improvement of PN-associated liver disease, and enhanced growth with the aid of an IRP.
authors.affiliation: Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE. Electronic address: benjamin.infantino@unmc.edu.
authors.forename: Benjamin J
authors.initials: BJ
authors.lastname: Infantino
authors.forename: David F
authors.initials: DF
authors.lastname: Mercer
authors.forename: Brandy D
authors.initials: BD
authors.lastname: Hobson
authors.forename: Ryan T
authors.initials: RT
authors.lastname: Fischer
authors.forename: Brandi K
authors.initials: BK
authors.lastname: Gerhardt
authors.forename: Wendy J
authors.initials: WJ
authors.lastname: Grant
authors.forename: Alan N
authors.initials: AN
authors.lastname: Langnas
authors.forename: Rubén E
authors.initials: RE
authors.lastname: Quiros-Tejeira
mesh_terms: D001663:Bilirubin / Q000378:metabolism; D001827:Body Height; D002648:Child; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007410:Intestinal Diseases / Q000150:complications / Q000601:surgery; D007421:Intestine, Small / Q000503:physiopathology*; D008297:Male; D010289:Parenteral Nutrition, Total / Q000379:methods*; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000628:therapy*; D013997:Time Factors; D000637:Transaminases / Q000097:blood; D016896:Treatment Outcome
chemical_list: D000637:Transaminases; D001663:Bilirubin
keywords: ALT; AST; Alanine aminotransferase; Aspartate aminotransferase; IRP; Intestinal rehabilitation program; PN; PNALD; Parenteral nutrition; Parenteral nutrition–associated liver disease; STEP; Serial transverse enteroplasty procedure; USSB; Ultrashort small bowel