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A nordic multicenter study on contemporary outcomes of pediatric short bowel syndrome in 208 patients

Mutanen, Annika ; Engstrand Lilja, Helene ; Wester, Tomas ; Norrby, Heimir ; Borg, Helena ; Persson, Sara ; Bjornland, Kristin ; Brun, Anne Charlotte ; Telborn, Lovisa LU and Stenström, Pernilla LU orcid , et al. (2023) In Clinical Nutrition 42(7). p.1095-1103
Abstract

Background & aims: Despite advances in the management of short bowel syndrome related intestinal failure (SBS-IF), large-scale contemporary pediatric studies are scarce. The aim of this multicenter study was to assess key outcomes and clinical prognostic factors in a recent Nordic pediatric SBS-IF population. Methods: Patients with SBS-IF treated during 2010–2019, whose parenteral support (PS) started at age <1 year and continued >60 consecutive days were included and retrospectively reviewed. All six participating centers followed multidisciplinary SBS-IF management. Risk factors for PS dependency, intestinal failure associated liver disease (IFALD) and mortality were assessed with Cox regression and Kaplan Meier analyses.... (More)

Background & aims: Despite advances in the management of short bowel syndrome related intestinal failure (SBS-IF), large-scale contemporary pediatric studies are scarce. The aim of this multicenter study was to assess key outcomes and clinical prognostic factors in a recent Nordic pediatric SBS-IF population. Methods: Patients with SBS-IF treated during 2010–2019, whose parenteral support (PS) started at age <1 year and continued >60 consecutive days were included and retrospectively reviewed. All six participating centers followed multidisciplinary SBS-IF management. Risk factors for PS dependency, intestinal failure associated liver disease (IFALD) and mortality were assessed with Cox regression and Kaplan Meier analyses. IFALD was defined with serum liver biochemistry levels. Results: Among 208 patients, SBS-IF resulted from NEC in 49%, gastroschisis w/wo atresia in 14%, small bowel atresia in 12%, volvulus in 11%, and other diagnoses in 14%. Median age-adjusted small bowel length was 43% (IQR 21–80%). After median follow up of 4.4 years (IQR 2.5–6.9), enteral autonomy was reached by 76%, none had undergone intestinal transplantation, and overall survival was 96%. Half of deaths (4/8) were caused by septic complications. Although biochemical cholestasis occurred only in 3% at latest follow-up and none of deaths were directly caused by IFALD, elevated liver biochemistry (HR 0.136; P = 0.017) and shorter remaining small bowel (HR 0.941; P = 0.040) predicted mortality. Shorter remaining small bowel and colon, and presence of end-ostomy were the main predictors of PS dependency, but not IFALD. Patients with NEC reached enteral autonomy more efficiently and had decreased incidence of IFALD compared to other etiologies. Conclusions: Although with current multidisciplinary management, prognosis of pediatric SBS is encouraging, septic complications and IFALD still associated with the remaining low mortality rate.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Epidemiology, Intestinal failure, Intestinal failure associated liver disease, Mortality, Multidisciplinary, Necrotizing enterocolitis
in
Clinical Nutrition
volume
42
issue
7
pages
9 pages
publisher
Elsevier
external identifiers
  • pmid:37270343
  • scopus:85160766750
ISSN
0261-5614
DOI
10.1016/j.clnu.2023.05.017
language
English
LU publication?
yes
id
c25df52e-2c46-422c-b1ad-608ff029aa80
date added to LUP
2023-08-28 13:25:39
date last changed
2024-04-20 02:04:36
@article{c25df52e-2c46-422c-b1ad-608ff029aa80,
  abstract     = {{<p>Background &amp; aims: Despite advances in the management of short bowel syndrome related intestinal failure (SBS-IF), large-scale contemporary pediatric studies are scarce. The aim of this multicenter study was to assess key outcomes and clinical prognostic factors in a recent Nordic pediatric SBS-IF population. Methods: Patients with SBS-IF treated during 2010–2019, whose parenteral support (PS) started at age &lt;1 year and continued &gt;60 consecutive days were included and retrospectively reviewed. All six participating centers followed multidisciplinary SBS-IF management. Risk factors for PS dependency, intestinal failure associated liver disease (IFALD) and mortality were assessed with Cox regression and Kaplan Meier analyses. IFALD was defined with serum liver biochemistry levels. Results: Among 208 patients, SBS-IF resulted from NEC in 49%, gastroschisis w/wo atresia in 14%, small bowel atresia in 12%, volvulus in 11%, and other diagnoses in 14%. Median age-adjusted small bowel length was 43% (IQR 21–80%). After median follow up of 4.4 years (IQR 2.5–6.9), enteral autonomy was reached by 76%, none had undergone intestinal transplantation, and overall survival was 96%. Half of deaths (4/8) were caused by septic complications. Although biochemical cholestasis occurred only in 3% at latest follow-up and none of deaths were directly caused by IFALD, elevated liver biochemistry (HR 0.136; P = 0.017) and shorter remaining small bowel (HR 0.941; P = 0.040) predicted mortality. Shorter remaining small bowel and colon, and presence of end-ostomy were the main predictors of PS dependency, but not IFALD. Patients with NEC reached enteral autonomy more efficiently and had decreased incidence of IFALD compared to other etiologies. Conclusions: Although with current multidisciplinary management, prognosis of pediatric SBS is encouraging, septic complications and IFALD still associated with the remaining low mortality rate.</p>}},
  author       = {{Mutanen, Annika and Engstrand Lilja, Helene and Wester, Tomas and Norrby, Heimir and Borg, Helena and Persson, Sara and Bjornland, Kristin and Brun, Anne Charlotte and Telborn, Lovisa and Stenström, Pernilla and Pakarinen, Mikko P.}},
  issn         = {{0261-5614}},
  keywords     = {{Epidemiology; Intestinal failure; Intestinal failure associated liver disease; Mortality; Multidisciplinary; Necrotizing enterocolitis}},
  language     = {{eng}},
  number       = {{7}},
  pages        = {{1095--1103}},
  publisher    = {{Elsevier}},
  series       = {{Clinical Nutrition}},
  title        = {{A nordic multicenter study on contemporary outcomes of pediatric short bowel syndrome in 208 patients}},
  url          = {{http://dx.doi.org/10.1016/j.clnu.2023.05.017}},
  doi          = {{10.1016/j.clnu.2023.05.017}},
  volume       = {{42}},
  year         = {{2023}},
}