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Pediatric Chronic Intestinal Failure: Something Moving?
Aysenur Demirok1, Sjoerd C J Nagelkerke1, Marc A Benninga1
1Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam University Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Insights
Pediatric chronic intestinal failure (PIF) requires home parenteral nutrition (HPN) due to nutrient malabsorption. Advances improve outcomes, but research is needed for better nutritional, pharmacological, and surgical care.
Area of Science:
- Pediatric gastroenterology and nutrition.
Background:
- Pediatric chronic intestinal failure (PIF) is a rare condition where the intestine cannot absorb sufficient nutrients and fluids.
- PIF necessitates home parenteral nutrition (HPN) for growth and homeostasis.
- Underlying causes include short bowel syndrome, motility disorders, and congenital enteropathies.
Purpose of the Study:
- To review the current understanding and management of pediatric chronic intestinal failure.
- To highlight common complications associated with HPN and recent therapeutic advancements.
Main Methods:
- A MEDLINE search was conducted in May 2024.
- Keywords used were "intestinal failure", "parenteral nutrition", and "pediatric".
Main Results:
- PIF encompasses various conditions requiring HPN, with common complications including infections, thrombosis, liver disease, bacterial overgrowth, bone disease, and renal impairment.
- Treatment has improved, reducing morbidity and mortality.
- Glucagon-like peptide 2 shows promise for short bowel syndrome.
Conclusions:
- Centralization of care and international collaboration are crucial for improving outcomes in PIF.
- Further research is essential for advancing nutritional, pharmacological, surgical, and preventative strategies in HPN.
- Despite progress, optimizing care for pediatric patients with intestinal failure remains a priority.
Abstract:
Pediatric chronic intestinal failure (PIF) is a rare and heterogeneous condition characterized by the inability of the patient's intestine to adequately absorb the required fluids and/or nutrients for growth and homeostasis. As a result, patients will become dependent on home parenteral nutrition (HPN). A MEDLINE search was performed in May 2024 with keywords "intestinal failure", "parenteral nutrition" and "pediatric". Different underlying conditions which may result in PIF include short bowel syndrome, intestinal neuromuscular motility disorders and congenital enteropathies. Most common complications associated with HPN are catheter-related bloodstream infections, catheter-related thrombosis, intestinal failure-associated liver disease, small intestinal bacterial overgrowth, metabolic bone disease and renal impairment. Treatment for children with PIF has markedly improved with a great reduction in morbidity and mortality. Centralization of care in specialist centers and international collaboration between centers is paramount to further improve care for this vulnerable patient group. A recently promising medical therapy has become available for children with short bowel syndrome which includes glucagon-like peptide 2, a naturally occurring hormone which is known to delay gastric emptying and induce epithelial proliferation. Despite advances in curative and supportive treatment, further research is necessary to improve nutritional, pharmacological and surgical care and prevention of complications associated with parenteral nutrition use.
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