A recent review (CP Duggan, T Jaksic. NEJM 2017; 377: 666-75) concisely reviews recent advances in pediatric intestinal failure.  Most of the review has been covered elsewhere in this blog.  A couple of key points:

Outcomes of intestinal failure:

• The authors note that a 2012 study identified a 25% mortality rate of infants enrolled between 2000-2004.  “More recent advances have resulted in substantially improved survival rates (>90%).”

Epidemiology of intestinal failure:

• Using a definition of needing parenteral nutrition for more than 42 days after bowel resection or a residual small-bowel length of less than 25% of normal (for gestational age), intestinal failure was identified in 24.5 cases per 100,000 live births
• Among infants with birth weight <1500 g, the incidence is 7 per 1000 live births.
• Frequent causes: necrotizing enterocolitis, gastroschisis

Adaptation of Intestine:

• Improved chances of attaining enteral autonomy if longer residual small bowel, younger age at time of intestinal resection, preservation of ileocecal valve, absence of severe liver disease, diagnosis of necrotizing enterocolitis, and normal motility.

Parenteral nutrition:

• Lower rates of liver disease noted with routine restriction of soy-based fat emulsions to 1 g per kilogram
• Fish oil preparations (with n-3 fatty acids): switching to fish oil preparation “reduces biochemical measures of cholestasis.”
• Newer preparations of fat emulsions: Smoflipid, Clinolipid are FDA-approved for adults.  Smoflipid, in small studies, is associated with lower conjugated bilirubin compared with soy-based lipids.

Enteral nutrition:

• “Prompt initiation of enteral feeding after bowel resection has been reported to improve the rate of enteral autonomy….little justification for prolonged ‘gut rest'”
• The authors note that human milk is often chosen for enteral nutrition and when unavailable, amino acid based formulas are typically chosen due to “more favorable outcomes than protein hydrolysates.”
• Chronic diarrhea is improved with drip feedings, though bolus feeds may have trophic effects.  “In our experience, a combined approach (e.g. continuous feeding at night and bolus feeding during the day) is feasible.”
• Oral motor stimulation is important.  Thus, try to give oral human milk feeds when feasible.

Medical Therapies:

• Acid blockers: used for hyperacidity after massive resection
• Loperamide
• Bile acid sequestrants (eg. cholestyramine)
• NOT evaluated in intestinal failure: octreotide, racecadotril, crofelemer
• Motility agents
• Antibiotics for bacterial overgrowth.  “Cyclical use (1 week per month) of broad-spectrum antibiotics…is the mainstay of therapy…at many centers.”
• Probiotics: “No evidence of benefit in small studies; risk of sepsis”
• Pancreatic enzymes: rarely used. Indicated if pancreatic atrophy or exocrine insufficiency
• Growth factors: Teduglutide -licensed for adults, studies in children are ongoing

Surgical Therapies:

• Central lines
• Gastrostomy Tubes
• STEP procedure or possibly lengthening procedure (Bianchi).  STEP procedure is less technically difficult.

Previous related blog entries:

• How long does it take the liver to recover from PNALD?
• Green beans for short gut syndrome | gutsandgrowth
• Nutrition Week (Day 4) Trophic Hormone for Pediatric Short Bowel Syndrome
• Nutrition Week (Day 2) SMOFlipid
• Outcomes with STEP procedure | gutsandgrowth
• Enteral Autonomy in Pediatric Intestinal Failure | gutsandgrowth
• Predicting Short Bowel Syndrome Enteral Autonomy: Small Bowel Diameter | gutsandgrowth
• Severe Hypothyroidism due to Iodine Deficiency Associated with Parenteral Nutrition | gutsandgrowth
• Nutrition Support for Intestinal Failure | gutsandgrowth
• IFfy outcome | gutsandgrowth
• Four advances for intestinal failure | gutsandgrowth
• Optimizing lipids to minimize cholestasis | gutsandgrowth
• More on ethanol locks | gutsandgrowth