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Chronic intestinal failure — Clinical Guidelines

Overview

Chronic intestinal failure (CIF) involves the inability of the intestines to maintain adequate fluid, electrolyte, and nutrient absorption, often necessitating parenteral support. It encompasses a range of conditions requiring long-term management including home parenteral nutrition (HPN) and intestinal rehabilitation. 1 4

Diagnosis

Clinical Presentation: Symptoms include malnutrition, dehydration, electrolyte imbalances, and weight loss. 1

Laboratory Tests: Regular monitoring of micronutrients (vitamin C, vitamin D, vitamins A, E, B12, folate, zinc, selenium, copper), electrolytes, and liver function tests. 2 3 5

Imaging: MRI for assessing liver steatosis in patients on HPN. 5

Consensus Definitions: Updated definitions reviewed and validated by ESPEN for consistent diagnosis and management. 1

Management

Home Parenteral Nutrition (HPN): Primary treatment for CIF, tailored to individual patient needs with close monitoring of micronutrient deficiencies. 1 3 4

Micronutrient Supplementation: Regular assessment and supplementation for deficiencies, particularly vitamin C, vitamin D, vitamins A and E, zinc, and selenium. 2 3 5

Intestinal Rehabilitation: Consideration for patients suitable for enteral feeding optimization and potential weaning from HPN. 1 4

Liver Health Monitoring: Regular evaluation for IFALD, especially in long-term HPN users, using biomarkers like vitamin D levels and MRI for liver steatosis. 5

Special Populations

Pregnancy: Specific guidelines for managing CIF during pregnancy, emphasizing multidisciplinary care and monitoring nutritional status closely. 1

Transition to Adult Care: Structured transition protocols focusing on patient autonomy, self-management skills, and continuity of care from pediatric to adult centers. 6

Elderly: Considerations for age-related changes in micronutrient absorption and metabolism, necessitating individualized HPN formulations. 3

Key Recommendations

Establish and maintain care in specialized CIF centers for comprehensive management and multidisciplinary support. (Evidence: Strong 1)

Regularly monitor and supplement micronutrients, particularly vitamin C, vitamin D, and others as indicated by laboratory findings, to prevent deficiencies. (Evidence: Moderate 2 3 5)

Implement structured transition programs for pediatric patients moving to adult care, ensuring continuity and patient empowerment. (Evidence: Expert opinion 6)

Screen for and manage liver health, including monitoring for IFALD, especially in patients on long-term HPN. (Evidence: Moderate 5)

Tailor HPN formulations to individual patient needs, considering age-specific factors and potential comorbidities. (Evidence: Expert opinion 3)

References

1 Pironi L, Cuerda C, Jeppesen PB, Joly F, Jonkers C, Krznarić Ž et al.. ESPEN guideline on chronic intestinal failure in adults - Update 2023. Clinical nutrition (Edinburgh, Scotland) 2023. link 2 Stevens P. 25 year experience of patterns of plasma vitamin C levels in patients requiring home parenteral support. Clinical nutrition ESPEN 2023. link 3 Culkin A, Brundrett D, Pearson M, Gabe S. A one size vial does not fit all: An evaluation of the micronutrient status of adult patients receiving home parenteral nutrition (HPN). Clinical nutrition ESPEN 2023. link 4 Cuerda C, Pironi L, Arends J, Bozzetti F, Gillanders L, Jeppesen PB et al.. ESPEN practical guideline: Clinical nutrition in chronic intestinal failure. Clinical nutrition (Edinburgh, Scotland) 2021. link 5 Rondaij T, Kozjek NR, Popovič P, Jordan T. Vitamin D deficiency in patients with chronic intestinal failure on home parenteral nutrition. Clinical nutrition ESPEN 2021. link 6 Diamanti A, Capriati T, Lezo A, Spagnuolo MI, Gandullia P, Norsa L et al.. Moving on: How to switch young people with chronic intestinal failure from pediatric to adult care. a position statement by italian society of gastroenterology and hepatology and nutrition (SIGENP) and italian society of artificial nutrition and metabolism (SINPE). Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver 2020. link 7 Pironi L, Malucelli E, Guidetti M, Lanzoni E, Farruggia G, Pinna AD et al.. The complex relationship between magnesium and serum parathyroid hormone: a study in patients with chronic intestinal failure. Magnesium research 2009. link

Chronic intestinal failure