Long-term outcomes of pediatric intestinal failure

Fullerton, Brenna S.; Hong, Charles R.; Jaksic, Tom

doi:10.1053/j.sempedsurg.2017.09.006

Cited by 59 publications

(74 citation statements)

References 110 publications

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“…( 64 ) It has been reported that the incidence of CLABSIs was highest in children with SBS, PN dependence, and age <1 year, precisely the group most at risk for IFALD. ( 65 ) Thus, strategies for prevention of CLABSIs are critical for prevention of IFALD. In addition to strict education of caregivers regarding sterile central line management, use of ethanol locks of central venous catheters reduced the rate of CLABSIs by 81% in pediatric patients with IF.…”

Section: Therapeutic and Preventative Approachesmentioning

confidence: 99%

New Insights Into Intestinal Failure–Associated Liver Disease in Children

2020

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Development of intestinal failure–associated liver disease (IFALD) is a common complication of long‐term parenteral nutrition (PN) in children and adults. The molecular and cellular mechanisms and the phases of IFALD are now being delineated. Components of PN lipid emulsions, including plant sterols, interact with hepatic innate immune activation promoted by products of gut bacterial overgrowth/dysbiosis and altered intestinal barrier function (gut‐liver axis) and by episodes of sepsis to cause cholestasis and IFALD. New therapeutic strategies, including modifications of intravenous lipid emulsions to reduce pro‐inflammatory fatty acids and plant sterol content, can lower the risk of IFALD, reverse cholestasis, and reduce complications, although the significance of persisting hepatic fibrosis is unknown. This review will provide an update on advances in the pathogenesis of IFALD, newer therapeutic and preventative strategies, and challenges that confront managing patients with IFALD.

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Section: Therapeutic and Preventative Approachesmentioning

confidence: 99%

New Insights Into Intestinal Failure–Associated Liver Disease in Children

2020

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“…It is thought that parenteral vitamin D requirements might be lower than enteral requirements as no enteral intake of minerals needs to be facilitated . However, suboptimal 25‐OH vitamin D levels have been reported in children on long‐term PN in 40% despite receiving appropriate fat‐soluble vitamin solutions . In our experience, suboptimal levels of vitamin D (<50 nmol/L) are encountered in our cohort despite administration of 200–400 IU/day.…”

Section: Long‐term Laboratory Monitoringmentioning

confidence: 67%

“…The genetic target height based on mid‐parental height should be factored into expectations for growth . Growth failure, particularly short stature, has been reported in long‐term PN . Stunting may be due to chronic intestinal inflammation, poor nutritional intake at critical periods, the underlying disease itself, and side effects of medications .…”

Section: Growth and Body Composition Monitoringmentioning

confidence: 99%

“…Intestinal failure‐associated liver disease (IFALD) is the most frequent complication in children on long‐term PN . Although some hepatic complications can be reversible, severe outcomes with end‐stage liver disease may occur .…”

Section: Monitoring Of Long‐term Complicationsmentioning

confidence: 99%

“…Pathogenesis of IFALD is multifactorial . The nutrition‐related factors include absence of enteral feeding, overfeeding, excess of lipid or glucose intake, imbalanced amino acids composition, continuous PN (non‐cyclical), lipid emulsions containing high w‐6/w‐3 ratio and high phytosterols with low α‐tocopherol levels, and micronutrient toxicity (copper, manganese, iron, aluminum).…”

Section: Monitoring Of Long‐term Complicationsmentioning

confidence: 99%

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Monitoring of long‐term parenteral nutrition in children with intestinal failure

Zemrani

Bines

2019

JGH Open

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Pediatric intestinal failure (IF) is a rare and complex condition associated with significant morbidity and mortality. It is defined as the reduction of gut mass or function below the minimal needed for absorption of nutrients and fluid to sustain life and growth. Since the advent of specialized multidisciplinary intestinal rehabilitation centers, IF management has considerably evolved in the last years, but serious complications of long‐term parenteral nutrition (PN) can occur. Main complications include intestinal failure‐associated liver disease, growth failure, body composition imbalance, central venous access complications, micronutrient deficiencies and toxicities, metabolic bone disease, small intestinal bacterial overgrowth, and renal disease. With improvement in survival rates of patients over the last 20 years, emphasis should be on limiting IF‐related comorbidities and improving quality of life. Close monitoring is pivotal to ensuring quality of care of these patients. The care of children with chronic IF should involve a comprehensive monitoring plan with flexibility for individualization according to specific patient needs. Monitoring of children on long‐term PN varies significantly across units and is mainly based on experience, although few guidelines exist. This narrative review summarizes the current knowledge and practices related to monitoring of children with IF. The authors also share their 20‐year experience at the Royal Children's Hospital in Melbourne Australia on this topic.

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Key Determinants for Achieving Enteral Autonomy and Reduced Parenteral Nutrition Exposure in Pediatric Intestinal Failure

Enman

Wilkinson

Meloni

et al. 2019

J Parenter Enteral Nutr

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Background We sought to evaluate the relationship between determinants of intestinal failure (IF) and achieving enteral autonomy from parenteral nutrition (PN) in a large single‐center cohort of children. Methods This is a retrospective chart review of pediatric subjects enrolled in a database for the Center for Advanced Intestinal Rehabilitation at Children's of Alabama from 1989 to 2016. IF was defined as dependence on PN for >60 days. Subjects were included if they were followed since birth or infancy for a minimum of 3 months and sufficient documentation of study variables were available. Gestational age, race, diagnosis, anatomy (percent small and large bowel remaining, presence of ileocecal valve [ICV]), county of residence (rural/urban), and days of PN use were recorded. Kaplan‐Meier curves and parametric survival regression models were used to investigate the relationship between the demographic and clinical variables with the length of PN use. Results Initially, 290 subjects were available to review. After inclusion/exclusion were applied, 158 subjects remained. Gestational age, diagnosis (necrotizing enterocolitis), small‐bowel length (>50%), and presence of an ICV were all positive predictors for reaching enteral autonomy. Residual colon length was associated with shorter duration of PN in days. Conclusion Enteral autonomy is a key outcome among children with IF. In our cohort, we found that gestational age, diagnosis, remaining small bowel, and presence of ICV are positive predictors for reaching this important milestone. Colon length is also an important factor with respect to duration of PN in days.

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Long-term outcomes of pediatric intestinal failure

Cited by 59 publications

References 110 publications

New Insights Into Intestinal Failure–Associated Liver Disease in Children

New Insights Into Intestinal Failure–Associated Liver Disease in Children

Monitoring of long‐term parenteral nutrition in children with intestinal failure

Key Determinants for Achieving Enteral Autonomy and Reduced Parenteral Nutrition Exposure in Pediatric Intestinal Failure

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