INTRODUCTION: Parenteral nutrition-associated cholestasis (PNAC) is a complication of long-term parenteral nutrition (PN). Removal of lipids may reverse PNAC but compromises the energy to ensure infant growth. The purpose of this study was to test whether a low-fat, high-carbohydrate PN regimen, which prevents and reverses PNAC in adults, could do the same in infants. This regimen could potentially avoid the problem of diminished energy input after removing nutritional lipids. METHODS: Infants developing PNAC over a 2-year period were started on a low-fat PN regimen with calories primarily from carbohydrates. The fat-free PN, containing 314 kJ/ml, was provided 5-6 times a week and fat, including essential fatty acids and fat-soluble vitamins, 1-2 times a week. Enteral feeding was continued according to individual tolerance. RESULTS: The study included 10 infants with short bowel syndrome (six with intestinal failure due to necrotizing enterocolitis, one with gastroschisis, one with complications due to unrecognized anal atresia and two with midgut volvulus). Median duration of PN with fat before initiating the low-fat regime was 69 days (25-75 % percentile: 41-75 days), and mean s-bilirubin was 139 µmol/l (range 87-323 µmol/l). Median duration with low-fat regimen was 69 days (25-75 % percentile: 18-123 days). Bilirubin reversed to normal (<50 µmol/l) in all infants. Seven children showed catch-up growth. No essential fatty acid deficiency, steatosis or deaths were observed. CONCLUSIONS: A low-fat, high-carbohydrate PN regimen together with enteral feeding is well tolerated and may be used in reversing liver disease in PN-dependent infants without compromising growth.
Digestive Diseases and Sciences, 2015, Vol 60, Issue 1, p. 252-9