Role of an intestinal rehabilitation program in the treatment of advanced intestinal failure

Clarivet Torres, Debra Sudan, Jon Vanderhoof, Wendy Grant, Jean Botha, Stephen C Raynor, Alan Norman Langnas

Research output: Contribution to journalArticle

103 Citations (Scopus)

Abstract

OBJECTIVE: To analyze outcomes in children with intestinal failure treated by our Intestinal Rehabilitation Program (IRP) in a 4-year period. PATIENTS AND METHODS: A total of 51 parenteral nutrition (PN)-dependent patients (20 male) were enrolled in the IRP. Median age was 1.7 years, with the primary diagnoses being gastroschisis, necrotizing enterocolitis, volvulus, and congenital atresia. Median small bowel intestinal length was 35 cm, with the majority of patients having only jejunum as remaining bowel anatomy. Thirty-six of the 51 patients had liver disease characterized by cirrhosis, advance bridging fibrosis, and portal and periportal fibrosis. Height, weight z score, platelet count, albumin, and bilirubin levels were measured at the beginning and end of the study. RESULTS: Of the 51 patients, 29 had 46 different surgical intestinal repairs. Twenty-nine of the 36 patients with hyperbilirubinemia had normalized serum bilirubin with treatment. Ten patients required transplantation. Five patients died of sepsis, influenza, or complications after intestinal transplantation. Of the remaining 37 patients in the IRP, 31 were weaned from parenteral nutrition (5 with cirrhosis); 6 patients are in the process of weaning. Survival rate of the patients in the IRP was 90%. Growth has continued along the same curve, and some patients have exhibited significant catch-up. CONCLUSIONS: With an aggressive medical/surgical approach, even patients with intestinal failure and advanced liver disease can avoid transplantation. Patients in the IRP showed improved liver function and nutritional parameters with the ability to discontinue PN while maintaining growth. Early referral of these patients to specialized centers before the development of advanced liver disease is recommended.

Original languageEnglish (US)
Pages (from-to)204-212
Number of pages9
JournalJournal of pediatric gastroenterology and nutrition
Volume45
Issue number2
DOIs
StatePublished - Aug 1 2007

Fingerprint

Rehabilitation
Therapeutics
liver diseases
parenteral feeding
Fibrosis
Liver Diseases
Transplantation
Parenteral Nutrition
bilirubin
fibrosis
Bilirubin
Gastroschisis
hyperbilirubinemia
volvulus
enterocolitis
Intestinal Volvulus
Necrotizing Enterocolitis
sepsis (infection)
Hyperbilirubinemia
blood platelet count

Keywords

  • Intestinal failure
  • Intestinal rehabilitation
  • Short bowel syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Gastroenterology

Cite this

Role of an intestinal rehabilitation program in the treatment of advanced intestinal failure. / Torres, Clarivet; Sudan, Debra; Vanderhoof, Jon; Grant, Wendy; Botha, Jean; Raynor, Stephen C; Langnas, Alan Norman.

In: Journal of pediatric gastroenterology and nutrition, Vol. 45, No. 2, 01.08.2007, p. 204-212.

Research output: Contribution to journalArticle

Torres, Clarivet ; Sudan, Debra ; Vanderhoof, Jon ; Grant, Wendy ; Botha, Jean ; Raynor, Stephen C ; Langnas, Alan Norman. / Role of an intestinal rehabilitation program in the treatment of advanced intestinal failure. In: Journal of pediatric gastroenterology and nutrition. 2007 ; Vol. 45, No. 2. pp. 204-212.
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N2 - OBJECTIVE: To analyze outcomes in children with intestinal failure treated by our Intestinal Rehabilitation Program (IRP) in a 4-year period. PATIENTS AND METHODS: A total of 51 parenteral nutrition (PN)-dependent patients (20 male) were enrolled in the IRP. Median age was 1.7 years, with the primary diagnoses being gastroschisis, necrotizing enterocolitis, volvulus, and congenital atresia. Median small bowel intestinal length was 35 cm, with the majority of patients having only jejunum as remaining bowel anatomy. Thirty-six of the 51 patients had liver disease characterized by cirrhosis, advance bridging fibrosis, and portal and periportal fibrosis. Height, weight z score, platelet count, albumin, and bilirubin levels were measured at the beginning and end of the study. RESULTS: Of the 51 patients, 29 had 46 different surgical intestinal repairs. Twenty-nine of the 36 patients with hyperbilirubinemia had normalized serum bilirubin with treatment. Ten patients required transplantation. Five patients died of sepsis, influenza, or complications after intestinal transplantation. Of the remaining 37 patients in the IRP, 31 were weaned from parenteral nutrition (5 with cirrhosis); 6 patients are in the process of weaning. Survival rate of the patients in the IRP was 90%. Growth has continued along the same curve, and some patients have exhibited significant catch-up. CONCLUSIONS: With an aggressive medical/surgical approach, even patients with intestinal failure and advanced liver disease can avoid transplantation. Patients in the IRP showed improved liver function and nutritional parameters with the ability to discontinue PN while maintaining growth. Early referral of these patients to specialized centers before the development of advanced liver disease is recommended.

AB - OBJECTIVE: To analyze outcomes in children with intestinal failure treated by our Intestinal Rehabilitation Program (IRP) in a 4-year period. PATIENTS AND METHODS: A total of 51 parenteral nutrition (PN)-dependent patients (20 male) were enrolled in the IRP. Median age was 1.7 years, with the primary diagnoses being gastroschisis, necrotizing enterocolitis, volvulus, and congenital atresia. Median small bowel intestinal length was 35 cm, with the majority of patients having only jejunum as remaining bowel anatomy. Thirty-six of the 51 patients had liver disease characterized by cirrhosis, advance bridging fibrosis, and portal and periportal fibrosis. Height, weight z score, platelet count, albumin, and bilirubin levels were measured at the beginning and end of the study. RESULTS: Of the 51 patients, 29 had 46 different surgical intestinal repairs. Twenty-nine of the 36 patients with hyperbilirubinemia had normalized serum bilirubin with treatment. Ten patients required transplantation. Five patients died of sepsis, influenza, or complications after intestinal transplantation. Of the remaining 37 patients in the IRP, 31 were weaned from parenteral nutrition (5 with cirrhosis); 6 patients are in the process of weaning. Survival rate of the patients in the IRP was 90%. Growth has continued along the same curve, and some patients have exhibited significant catch-up. CONCLUSIONS: With an aggressive medical/surgical approach, even patients with intestinal failure and advanced liver disease can avoid transplantation. Patients in the IRP showed improved liver function and nutritional parameters with the ability to discontinue PN while maintaining growth. Early referral of these patients to specialized centers before the development of advanced liver disease is recommended.

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