It takes a long time, even when there is no longer biochemical evidence of parenteral nutrition-associated liver disease (PNALD).  A recent study provides long-term data from a population-based, cross-sectional study on liver histology from pediatric intestinal failure (IF) patients (Hepatology 2013; 58: 729-38).  Patients were followed from 1984-2010.  IF was defined as having either >50% small bowel resection or need for PN >30 days.

The 38 IF participants had a median age of 7.2 years.  16 remained on PN after 74 months (range  2.5-204), 22 had weaned off PN 8.8 years (range 0.3-27) earlier after an average of 35 months of PN exposure.

Key findings:

• Abnormal liver histology was present in 94% of patients on PN and 77% off PN.
• Nearly 60% of patients on long-term PN had significant or severe fibrosis (Metavir stage ≥2).
• Significant liver fibrosis and steatosis persisted after weaning off PN. That is, “liver histology remains abnormal up to 9 years after weaning off PN in the majority of IF patients.”
• One patient off PN developed esophageal varices.
• Risk factors for increased fibrosis: extensive small intestinal resection, (P=.002) loss of ileocecal valve (P=.048), and recurrent sepsis (P=.002).

Bottomline: While there have been important clinical advances in the management of IF, the data from this study indicate that many patients who have normal liver biochemistries continue to have abnormal liver histology.  Whether this will have an important clinical impact is not known.

Previous related blog entries:

• IFfy outcome | gutsandgrowth
• Fish Oil, IFALD, and Liver Fibrosis | gutsandgrowth
• Four advances for intestinal failure | gutsandgrowth
• Optimizing lipids to minimize cholestasis | gutsandgrowth
• PNAC, PNALD, and IFAC | gutsandgrowth