AKA: intestinal epithelial dysplasia (IED)
Tufting enteropathy is a congenital disease that causes the villi in the gut to atrophy and deteriorate causing chronic diarrhea within the first few days of birth.
Signs and symptoms:
-Persistent diarrhea, despite any bowel rest – bowel actions usually range from 8-10 times per day – can be confused for looking like urine -Electrolyte imbalances and dehydration
-Failure to thrive
-An endoscopy/biopsy can reveal villi atrophy, which is where the villi in the digestive system erodes away -Some infants may also have an undeveloped esophagus, called esophageal atresia
-Tufting enteropathy is thought to be related to abnormal development of the intestinal epithelial cells in the small intestine and the colon. -Very little research and understanding into the complexities of how tufting enteropathy is caused -a link with the patients ethnicity has been found – children of Arabic background (middle eastern, Turkish and north African)
Long term management and outcomes:
-Tufting enteropathy will most lead to death if left untreated - Patients will require long-term total parenteral nutrition (TPN) to stay alive, which is given for 6 hours daily -Some patients that have milder symptoms can survive well on partial TPN, where TPN is given 3-4 times a week -TPN includes sugars, carbohydrates, lipids, electrolytes, vitamins and proteins - Because TPN solutions are highly concentrated and thick, the solutions must be given through catheters that are placed in large central veins in the neck, chest, or groin. -TPN works well for a long term treatment, however comes with complications such as;
-liver damage/liver failure
-Another issue with TPN is that over time it corrodes and damages the central veins it runs through. This can be an issue as TPN can only work using central veins. To try and fix...
Please join StudyMode to read the full document