Some people are born with or develop irreversible intestinal failure. Intestinal failure occurs when the intestines cannot digest food and absorb the fluids, electrolytes and nutrients that are essential to life and normal development. Total parenteral nutrition (TPN) is then indicated, which provides liquid nutrition through a catheter or needle inserted into a vein in the arm, groin, neck or chest.
The most common cause of intestinal failure is short-bowel syndrome where at least half or more of the small intestine has been removed. Short-bowel syndrome is typically a postsurgical condition for treatment of conditions such as trauma, necrotizing enterocolitis or midgut volvulus. Other causes are congenital malformations such as small-bowel atresia, gastroschisis, and aganglionosis. Intestinal failure may also be caused by functional disorders such as crohn's disease, chronic idiopathic intestinal pseudo-obstruction syndrome or absorptive impairment (e.g. intestinal pseudo-obstruction, microvillus inclusion disease). The conditions leading to intestinal failure are age-dependent.
ERNICA covers the following diseases within the intestinal failure working group:
- Congenital enteropathies
- Intractable diarrhea of infancy
- Chronic intestinal pseudo-obstruction
- Congenital chronic diarrhea with protein-losing enteropathy
- Epithelial dysplasia
- Microvillus inclusion disease
- Intestinal failure
- Short bowel syndrome
- Intestinal disease due to fat malabsorption