Reversal of intestinal failure-associated liver disease in infants and children on parenteral nutrition: experience with 93 patients at a referral center for intestinal rehabilitation.
OBJECTIVE
Intestinal failure (IF)-associated liver disease (IFALD) complicates the treatment of children with IF receiving parenteral nutrition (PN). We hypothesized that prevention or resolution of IFALD was possible in most children and that this would result in improved outcomes.


METHODS
We reviewed prospectively gathered data on all children referred to the intestinal rehabilitation and transplantation center at our institution. Total bilirubin level (TB) was used as the marker for IFALD. Patients were grouped based on TB at referral and at subsequent inpatient stays and outpatient visits. Standard treatment consisted of cycling of PN, limiting lipid infusion, enteral stimulation, use of ursodeoxycholic acid, and surgical intervention when necessary. Outcomes such as mortality, dependence on PN, and need for transplantation were assessed. Statistical analyses were performed using Fisher's exact, Mann-Whitney U, and Wilcoxon signed rank tests.


RESULTS
Ninety-three patients with intestinal failure and on PN were treated at our center from 2003 to 2009. Median age at referral was 5 months (0.5-264 months). Prematurity was a complicating factor in 63 patients and necrotizing enterocolitis was the most common diagnosis. Eighty-two children had short bowel syndrome, whereas the remaining 11 had extensive motility disorders. 97% of children required significant alteration of their PN administration. At referral, 76 of 93 children had TB 2.0 mg/dL or higher, and 17 had TB below 2.0 mg/dL. TB normalized in 57 of 76 children with elevated TB at referral, and TB remained elevated in 19. Normalization of TB was associated with a mortality of 5.2%, and transplantation was needed in 5.2%. Conversely, when TB remained elevated, mortality was 58% (P = .0002 vs TB normalized), and transplantation occurred in 58% owing to failure of surgical and medical rehabilitation.


CONCLUSIONS
Most children referred for treatment of IF have IFALD. A dedicated IF rehabilitation program can reverse IFALD in many children, and this is associated with improved outcome.
Cowles
Robert A
RA
Division of Pediatric Surgery, Morgan Stanley Children's Hospital of New York-Presbyterian and Columbia University Medical Center, New York, NY 10032, USA. rc2114@columbia.edu
Ventura
Kara A
KA
Martinez
Mercedes
M
Lobritto
Steven J
SJ
Harren
Patricia A
PA
Brodlie
Susan
S
Carroll
Joanne
J
Jan
Dominique M
DM
D001663:Bilirubin / Q000097:blood*; D015415:Biomarkers / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000209:etiology / Q000401:mortality / Q000628:therapy; D004750:Enteral Nutrition / Q000379:methods*; D020345:Enterocolitis, Necrotizing / Q000150:complications / Q000401:mortality / Q000628:therapy; D005217:Fat Emulsions, Intravenous / Q000627:therapeutic use; D005769:Gastrointestinal Motility; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature / Q000097:blood; D007410:Intestinal Diseases / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D007415:Intestinal Obstruction / Q000401:mortality / Q000601:surgery / Q000628:therapy; D007421:Intestine, Small / Q000637:transplantation; D007422:Intestines / Q000637:transplantation; D008107:Liver Diseases / Q000209:etiology / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D016377:Organ Transplantation; D010288:Parenteral Nutrition / Q000009:adverse effects* / Q000401:mortality; D010348:Patient Care Team; D011183:Postoperative Complications / Q000401:mortality; D012017:Referral and Consultation; D012047:Rehabilitation Centers; D012778:Short Bowel Syndrome / Q000401:mortality / Q000601:surgery / Q000628:therapy; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid
D015415:Biomarkers; D005217:Fat Emulsions, Intravenous; D014580:Ursodeoxycholic Acid; D001663:Bilirubin