Abstract

Twelve year outcomes of intestinal failure associated liver disease in children with short bowel syndrome: 97% transplant-free survival and 81% enteral autonomy

JPEN J Parenter Enteral Nutr. 2021 Apr 1. doi: 10.1002/jpen.2112. Online ahead of print.

Clarivet Torres 1 2, Vahe Badalyan 2 3, Parvathi Mohan 2 4

 
     

Author information

  • 1Pediatrics, Director, Intestinal Rehabilitation Program, Division of Gastroenterology, Hepatology, and Nutrition, Children's National Hospital, Washington, DC, USA.
  • 2George Washington University School of Medicine Washington, DC, USA.
  • 3Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Children's National Hospital, Washington, DC, USA.
  • 4Pediatrics, Director of Hepatology, Division of Gastroenterology, Hepatology, and Nutrition, Children's National Hospital, Washington, DC, USA.

Abstract

Objective: To analyze the outcomes in children with short bowel syndrome (SBS), parenteral nutrition dependence (PND) and intestinal failure associated liver disease (IFALD), treated in our Intestinal Rehabilitation Program (IRP) during 2007-2018.

Methods: We retrospectively reviewed charts of 135 patients with SBS-PND at the time of enrollment in IRP; of these 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2mg/dL at enrollment and/or abnormal liver biopsy showing stage 2-4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, AST to Platelet Ratio Index), growth trends, transplant rates, and mortality.

Results: Of the 89 SBS patients with IFALD, 74 had elevated CB at enrollment; the other 15 had normalized CB but had fibrosis on liver biopsy. Thirty-eight patients had liver biopsies: 36 (95%) had fibrosis, including 21/36 with bridging fibrosis/cirrhosis. Median proportion of residual small bowel was 23% (IQR 13 - 38%) of the expected length for age and median daily caloric requirement by PN was 100%. Two were transplanted, three died (one post-transplant), remaining 85 survived; 69 (81%) achieved enteral autonomy. Seventy-three (99%) of the 74 patients with hyperbilirubinemia normalized their CB with medical treatment. In a subset of 8/89 patients with initial platelet count of <100×103 /mcl (median 50.5) and median CB of 21 mg/dL, 7 achieved CB normalization and had improved platelet count. Overall survival was 97% (censored 96.3%).

Conclusion: We demonstrate high transplant-free survival and enteral autonomy rates among children with SBS-IFALD relying on low-dose soybean lipid emulsion. 

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