320.3: Ultra-Short Bowel Syndrome (USBS) – Does the lengthening procedures improve the outcomes? Intestinal Rehabilitation Program (IRP) Children’s National Medical Center (CNMC) Washington DC - USA

Torres, Clarivet; Badalyan, Vahe; Mohan, Parvathi; Sandler, Anthony D.

doi:10.1097/01.tp.0000575496.96821.0a

Cited by 1 publication

(4 citation statements)

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“…24 Other centres have reported rates of EA from 0% to 44%, but there is significant variations in the definitions of USBS and surgical management making comparisons challenging. 12,21,23,25,26 In most studies, the strongest predictor of EA is the residual SB length. A 2017 cohort study evaluating the probabilities of achieving EA based on the residual length of SB and large bowel (LB) showed that unsurprisingly those with >50% SB had an 80-100% chance of weaning from PN despite the length of their LB.…”

Section: Enteral Autonomymentioning

confidence: 99%

“…It remains a significant complication in children with USBS due to the duration of PN dependence with rates between 63% and 88% in historical reports. 12,21,22,25,26 More recent publications, employing alternative lipid management strategies and improved multidisciplinary care have demonstrated reduced hepatic complications (22-25%). 36,37 In a case series of 5 patients with USBS by Sanchez et al, they reported significant elevation in direct bilirubin in 4/5 patients (range 12-14/ mg/dL) with 4 receiving Omegaven© and/or lipid restriction.…”

Section: Central Line-associated Bloodstream Infectionsmentioning

confidence: 99%

“…21 Torres et al also reported a significant proportion of patients with liver disease in 21/24 patients with 19 patients having a mean conjugated bilirubin (CB) of 7.5mg/dL with 18/19 patients having normalized liver function after treatment over an 11week period. 26 Interestingly, 13/21 had a liver biopsy with 8 patients showing grade 3-4 fibrosis. 26 Infantino et al also reported median total bilirubin values of 6.4 and 9.1mg/dL, respectively in their non-adapted and adapted cohort of patients at initial presentation with significant improvement over time with median total bilirubin at final follow-up of 0.3-0.5mg/dL.…”

Section: Central Line-associated Bloodstream Infectionsmentioning

confidence: 99%

“…26 Interestingly, 13/21 had a liver biopsy with 8 patients showing grade 3-4 fibrosis. 26 Infantino et al also reported median total bilirubin values of 6.4 and 9.1mg/dL, respectively in their non-adapted and adapted cohort of patients at initial presentation with significant improvement over time with median total bilirubin at final follow-up of 0.3-0.5mg/dL. They did however, report persistent elevation of transaminases in the nonadapted group that may represent continued use of PN.…”

Section: Central Line-associated Bloodstream Infectionsmentioning

confidence: 99%

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<p>Multidisciplinary Management in Pediatric Ultrashort Bowel Syndrome</p>

Belza¹,

Wales²

2020

JMDH

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Pediatric intestinal failure (IF) remains a complex and devastating condition resulting in the inability of the gastrointestinal tract to absorb adequate fluids and nutrients to sustain life. The goal in the management of IF is to achieve enteral autonomy and when not possible to avoid and minimize the development of long-term complications. Survival rates for children with IF have continued to improve resulting in an increased population of children with more altered anatomy. While IF remains a rare disease, children with IF secondary to ultrashort bowel syndrome comprise an even smaller patient population. The goal of this article is to review the recent literature related to the impact of multidisciplinary intestinal rehabilitation programs (IRPs) on the management and outcomes of intestinal failure in children with ultrashort bowel syndrome and potential avenues to further improve the long-term outcomes of this patient population.

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