Outcomes of Children With Chronic Intestinal Failure: Experience Over 2 Decades at a Tertiary Paediatric Hospital

Background and Aims: The aim of the study was to aid decisions on prognosis and transplantation; this study describes the outcome of children with intestinal failure managed by the multidisciplinary intestinal rehabilitation program at the Royal Children's Hospital, Melbourne. Methods: Retrospective review of children requiring parenteral nutrition (PN) for>3 months who were assessed for home PN between 1991 and 2011. Results: A total of 51 children were included. Forty-two (82%) had short bowel syndrome (SBS), 5 (10%) had chronic intestinal pseudo-obstruction syndrome, and 4 (8%) had congenital enteropathies. Median small bowel length for patients with SBS was 45 cm (interquartile range 30–80) or 23.9% of the expected length for age (interquartile range 17.0%–40.6%). Overall survival rate was 84% (43/51). Mortality in children (n = 7) occurred after a median of 13.2 months (range 6.2–29.2) with intestinal failure–associated liver disease (IFALD) being the only predictor (P = 0.001). Out of 50 children 21 (42%) had IFALD. Children who were premature (P = 0.013), had SBS (P = 0.038), and/or frequent sepsis (P = 0.014) were more likely to develop IFALD. PN weaning occurred in 27 of 35 (77%) SBS survivors, after a median of 10.8 months (up to 8.2 years), with longer residual small bowel (P = 0.025), preservation of the ileocecal valve (P = 0.013) and colon (P = 0.011) being predictors. None of 5 (0%) patients w...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Nutrition Source Type: research

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Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Purpose of review The current review provides a summary of available lipid products and discusses current literature and the limitations to the use of various lipid products for treatment and prevention of intestinal failure-associated liver disease (IFALD) in pediatric patients dependent on parenteral nutrition. Recent findings Improvements in markers of cholestasis and liver function have been seen with minimizing soybean lipid, fish oil lipid, and mixed fish oil-containing lipid emulsions. Soybean-based lipid products are thought to be the biggest contributor to development of IFALD. Mixed fish oil-containing lipid...
Source: Current Opinion in Organ Transplantation - Category: Surgery Tags: SMALL BOWEL TRANSPLANTATION: Edited by Douglas G. Farmer Source Type: research
Abstract PURPOSE OF REVIEW: The current review provides a summary of available lipid products and discusses current literature and the limitations to the use of various lipid products for treatment and prevention of intestinal failure-associated liver disease (IFALD) in pediatric patients dependent on parenteral nutrition. RECENT FINDINGS: Improvements in markers of cholestasis and liver function have been seen with minimizing soybean lipid, fish oil lipid, and mixed fish oil-containing lipid emulsions. Soybean-based lipid products are thought to be the biggest contributor to development of IFALD. Mixed fish ...
Source: Current Opinion in Organ Transplantation - Category: Transplant Surgery Authors: Tags: Curr Opin Organ Transplant Source Type: research
Purpose of review This review discusses the monitoring aimed at achieving good long-term outcomes in people dependent on home parenteral nutrition (HPN). Recent findings There have been recent studies highlighting the importance of treating low bone mineral density in HPN-dependent patients, methods of screening for liver disease, assessment of quality of life (QoL) and the need for early assessment for intestinal transplantation and growth factor therapy. Summary High-quality HPN services require expert multidisciplinary teams with a focus on strict aseptic catheter care protocols alongside regular monitoring and ...
Source: Current Opinion in Gastroenterology - Category: Gastroenterology Tags: NUTRITION: Edited by Eamonn M.M. Quigley Source Type: research
This study's objective was to investigate our institution's outcomes for children with IFALD treated with 6 months of FO and who then restarted SO. METHODS: Inclusion criteria for FO included children with IFALD. Parenteral nutrition (PN)-dependent children resumed SO after FO and were prospectively followed for 4.5 years or until death, transplant, or PN discontinuation. The primary outcome was the cumulative incidence rate (CIR) for cholestasis after FO. RESULTS: Forty-eight subjects received FO, and conjugated bilirubin decreased over time (-0.22 mg/dL/week; 95% confidence interval [CI]: -0.25, -0.19; P 
Source: JPEN Journal Of Parenteral And Enteral Nutrition - Category: Nutrition Authors: Tags: JPEN J Parenter Enteral Nutr Source Type: research
AbstractHepatobiliary complications of hypoalimentation and parenteral nutrition (PN) are widely recognised. Hypoalimentation includes conditions such as anorexia nervosa (AN), obesity malnutrition and liver disease following bariatric surgery. Treatment of the underlying condition causing hypoalimentation can result in an improvement in liver dysfunction. Liver function test abnormalities are also commonly found in patients on PN, with the three main complications being steatosis, cholestasis and biliary system sludge/stones. Patients with intestinal failure receiving PN often have multiple possible aetiologies for liver ...
Source: Irish Journal of Medical Science - Category: General Medicine Source Type: research
CONCLUSIONS: Despite reassuring rates of survival and weaning off PN, long-term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging. PMID: 29701871 [PubMed - as supplied by publisher]
Source: JPEN Journal Of Parenteral And Enteral Nutrition - Category: Nutrition Authors: Tags: JPEN J Parenter Enteral Nutr Source Type: research
Pediatric intestinal transplantation has moved from the theoretic to an actual therapy for children with irreversible intestinal failure who are suffering from complications of total parenteral nutrition. Owing to significant advancement in the management of intestinal failure and prevention of parenteral nutrition-related complications that have led to reduction in incidence of parenteral nutrition-associated liver disease and have improved intestinal adaptation, the indications for intestinal transplantation are evolving. Long-term outcomes have improved, but challenges in long-term graft function owing to chronic reject...
Source: Gastroenterology Clinics of North America - Category: Gastroenterology Authors: Source Type: research
CONCLUSION: Close to one-quarter of the ITx recipients had advanced liver fibrosis. In the current era of improved PN management, our data suggests that previously reported risk factors for IFALD, such as extreme short gut syndrome and PN duration, may have a lesser impact on development of liver fibrosis. A prolonged duration of bilirubin elevation may be associated with advanced liver fibrosis in patients with IFALD, but this requires validation in a larger cohort. PMID: 29534292 [PubMed - as supplied by publisher]
Source: JPEN Journal Of Parenteral And Enteral Nutrition - Category: Nutrition Authors: Tags: JPEN J Parenter Enteral Nutr Source Type: research
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Source: American Journal of Transplantation - Category: Transplant Surgery Authors: Tags: Minireview Source Type: research
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