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.
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.
Authors: Michael B Krawinkel; Dietmar Scholz; Andreas Busch; Martina Kohl; Lukas M Wessel; Klaus-Peter Zimmer Journal: Dtsch Arztebl Int Date: 2012-06-04 Impact factor: 5.594
Authors: Ana M G A Sant'Anna; Eyad Altamimi; Rose-Frances Clause; Joanne Saab; Heather Mileski; Brian Cameron; Peter Fitzgerald; Guilherme M Sant'Anna Journal: Can J Gastroenterol Date: 2012-05 Impact factor: 3.522
Authors: Govardhana Rao Yannam; Debra L Sudan; Wendy Grant; Jean Botha; Alan Langnas; Jon S Thompson Journal: J Gastrointest Surg Date: 2010-08-24 Impact factor: 3.452
Authors: Matthew W Ralls; Farokh R Demehri; Yongjia Feng; Kathleen M Woods Ignatoski; Daniel H Teitelbaum Journal: Surgery Date: 2015-02-20 Impact factor: 3.982
Authors: Faraz A Khan; Robert H Squires; Heather J Litman; Jane Balint; Beth A Carter; Jeremy G Fisher; Simon P Horslen; Tom Jaksic; Samuel Kocoshis; J Andres Martinez; David Mercer; Susan Rhee; Jeffrey A Rudolph; Jason Soden; Debra Sudan; Riccardo A Superina; Daniel H Teitelbaum; Robert Venick; Paul W Wales; Christopher Duggan Journal: J Pediatr Date: 2015-04-25 Impact factor: 4.406
Authors: Matthew W Ralls; Ryo Sueyoshi; Richard S Herman; Brent Utter; Isabel Czarnocki; Nancy Si; Jonathan Luntz; Diann Brei; Daniel H Teitelbaum Journal: Pediatr Surg Int Date: 2013-01 Impact factor: 1.827