Long-Term Outcomes and Disease Burden of Neonatal Onset Short Bowel Syndrome
Copyright © 2019. Published by Elsevier Inc..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:55 |
|---|---|
| Enthalten in: |
Journal of pediatric surgery - 55(2020), 1 vom: 17. Jan., Seite 164-168 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Han, Sam M [VerfasserIn] |
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| Links: |
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| Themen: |
Adolescent |
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| Anmerkungen: |
Date Completed 02.09.2020 Date Revised 02.09.2020 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.jpedsurg.2019.09.071 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM302841318 |
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| 500 | |a Date Completed 02.09.2020 | ||
| 500 | |a Date Revised 02.09.2020 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2019. Published by Elsevier Inc. | ||
| 520 | |a PURPOSE: The study aims to describe long-term outcomes and disease burden of neonatal onset short bowel syndrome (SBS) | ||
| 520 | |a 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 | ||
| 520 | |a 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) | ||
| 520 | |a 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 | ||
| 520 | |a TYPE OF STUDY: Prognosis study | ||
| 520 | |a LEVEL OF EVIDENCE: Level II | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Adolescent | |
| 650 | 4 | |a Intestinal failure | |
| 650 | 4 | |a Intestinal rehabilitation | |
| 650 | 4 | |a Parenteral nutrition | |
| 650 | 4 | |a Short bowel syndrome | |
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| 700 | 1 | |a Hong, Charles R |e verfasserin |4 aut | |
| 700 | 1 | |a Han, Grace Y |e verfasserin |4 aut | |
| 700 | 1 | |a Staffa, Steven J |e verfasserin |4 aut | |
| 700 | 1 | |a Modi, Biren P |e verfasserin |4 aut | |
| 700 | 1 | |a Jaksic, Tom |e verfasserin |4 aut | |
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