Overview of pediatric short bowel syndrome.
ABSTRACT Short bowel syndrome (SBS) is a malabsorptive state occuring as a result of surgical resection or congenital disease of a significant portion of the small intestine . The amount of resection or remaining bowel generally dictates the degree of malabsorption and consequentely the need for specialized enteral nutrition or parenteral nutrition (PN). Intestinal failure in the context of SBS is defined as a dependence on PN to maintain minimal energy and fluid requirement for growth in children. Common causes of SBS in infants and children include necrotizing enterocolitis, midgut volvulus, intestinal atresia, and gastroschisis. Early identification of patients at risk for long-term PN dependency is the first step toward avoiding severe complications. Close monitoring of nutritional status, steady and early introduction of enteral nutrition, and aggressive prevention, diagnosis, and treatment of infections such as central venous catheter sepsis and bacterial overgrowth can significantly improve the prognosis. Intestinal transplantation is an emerging treatment that may be considered when intestinal failure is irreversible and children are experiencing serious complications related to TPN administration.
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ABSTRACT: Purpose: This study examined predictors of achieving enteral autonomy among pediatric short bowel syndrome (SBS) patients remaining on parenteral nutrition (PN) beyond one year. Methods: A retrospective single-institution study of 171 pediatric SBS patients (defined as >= 50% small bowel (SB) loss or >= 60 days of PN with onset before 6 weeks of age) was performed. Multivariate Cox proportional hazards analysis was conducted, with subgroup analysis of patients on PN for >= 1 year (n = 59). Primary outcome was successful wean from PN. Results: Over a follow-up of 4.1 +/- 4.8 years, 64.3% of children weaned from PN. Mortality was 15.2%. Presence of >= 10% expected SB length (hazard ratio [HR] 6.48, p=0.002) or an ileocecal valve (ICV; HR, 2.86, p < 0.001) predicted PN weaning. Of those on PN >= 1 year, the wean rate was 50.8%, and ICV no longer predicted weaning (p= 0.153). Predictors among those on PN >= 1 year were: >= 10% expected SB length (HR, 8.27, p= 0.010), intestinal atresia (HR, 4.26, p = 0.011), and necrotizing enterocolitis (NEC, HR, 2.84, p = 0.025). Conclusions: SBS children on PN >= 1 year continue to wean from PN, and those with >= 10% of predicted SB length, NEC, or atresia are more likely to do so. These findings may help direct management and advice for these challenging patients.Journal of Pediatric Surgery 10/2014; 50(1). DOI:10.1016/j.jpedsurg.2014.10.011 · 1.31 Impact Factor
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ABSTRACT: Although improvements in managing pediatric gastrointestinal diseases have occurred over time, the use of technical adjuncts to aid in management has greatly improved the survival and quality of life for these patients. This has increased the scope of presenting disorders, unique and challenging adverse effects, complications, and technical difficulties for which emergency care providers must be familiar. This article will include a review of gastrointestinal tubes and their complications and presurgical and postsurgical considerations for gastroschisis, omphalocele, and Hirschsprung disease. In addition, with increasing pediatric survival from organ transplantation, pretransplantation and posttransplantation issues will be discussed, specifically for liver and small bowel transplantation. Pediatric patients with short bowel syndrome are being encountered more frequently and use multiple high technical adjuncts to aid in their survival. These adjuncts, as well as a review of short bowel syndrome, will be discussed.Clinical Pediatric Emergency Medicine 06/2012; 13(2):106-113. DOI:10.1016/j.cpem.2012.04.001
2014 American Academy of Pediatrics National Conference and Exhibition; 10/2014