Sam M Han1, Jamie Knell1, Owen Henry2, Charles R Hong1, Grace Y Han1, Steven J Staffa2, Biren P Modi1, Tom Jaksic3. 1. Center for Advanced Intestinal Rehabilitation and Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA. 2. Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA. 3. Center for Advanced Intestinal Rehabilitation and Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA. Electronic address: tom.jaksic@childrens.harvard.edu.
Abstract
PURPOSE: The study aims to describe long-term outcomes and disease burden of neonatal onset short bowel syndrome (SBS). METHODS: Utilizing the WHO criteria for adolescence, patients 10-19 years of age with neonatal onset SBS requiring parenteral nutrition (PN) for >90 days and followed by our multidisciplinary intestinal rehabilitation center between 2009 and 2018 were included for analysis. RESULTS: Seventy adolescents with SBS were studied. Median (IQR) age at last follow up in our center was 15 (11, 17) years. There was 0% mortality in the cohort, and 94% remained transplant free. Fifty-three patients (76%) achieved enteral autonomy. Three patients were weaned from PN without transplantation after six years of follow-up and another four after ten years of care at our multidisciplinary center. Disease burden remained higher in adolescents receiving PN, including inpatient hospitalizations (p < 0.01), procedures (p = 0.01), clinic visits (p < 0.01), and number of prescribed medications (p < 0.01). CONCLUSION: Survival for adolescents with neonatal onset SBS is excellent. Of the cohort studied, there was no mortality, and more than 75% achieved enteral autonomy. Disease burden remains high for adolescents who remain dependent on PN. However, achievement of enteral autonomy is feasible with long-term multidisciplinary rehabilitation. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.
PURPOSE: The study aims to describe long-term outcomes and disease burden of neonatal onset short bowel syndrome (SBS). METHODS: Utilizing the WHO criteria for adolescence, patients 10-19 years of age with neonatal onset SBS requiring parenteral nutrition (PN) for >90 days and followed by our multidisciplinary intestinal rehabilitation center between 2009 and 2018 were included for analysis. RESULTS: Seventy adolescents with SBS were studied. Median (IQR) age at last follow up in our center was 15 (11, 17) years. There was 0% mortality in the cohort, and 94% remained transplant free. Fifty-three patients (76%) achieved enteral autonomy. Three patients were weaned from PN without transplantation after six years of follow-up and another four after ten years of care at our multidisciplinary center. Disease burden remained higher in adolescents receiving PN, including inpatient hospitalizations (p < 0.01), procedures (p = 0.01), clinic visits (p < 0.01), and number of prescribed medications (p < 0.01). CONCLUSION: Survival for adolescents with neonatal onset SBS is excellent. Of the cohort studied, there was no mortality, and more than 75% achieved enteral autonomy. Disease burden remains high for adolescents who remain dependent on PN. However, achievement of enteral autonomy is feasible with long-term multidisciplinary rehabilitation. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.
Authors: Helena A S Goldani; Marilia R Ceza; Liege L Godoy; Juliana M Giesta; Simone Beier; Juliana G Oliveira; Daltro L Nunes; Leticia Feldens; Iara R S Lucena; Adriano N R Taniguchi; Silvia C Hallberg; Daiane Durant; Simone Boettcher; Marcia A Schneider; Patricia P Mello; Mariana G L Riberg; Alana V Signorini; Cristina Miller; Berenice L Santos; Claudete O Silveira; Maira C M Morais; Terezinha V Laggazio; Carla C Costa; Carlos O Kieling Journal: J Pediatr Gastroenterol Nutr Date: 2022-05-17 Impact factor: 3.288