2011 - ISBTS 2011 Symposium

Plenary Session I: Disappearing PNLD? + Oral Communications 1

3.101 - Ninety-seven percent survivability, ninety-seven percent resolution of cholestasis at the Intestinal Rehabilitation Program (IRP) - Children’s National Medical Center (CNMC) Washington DC

Presenter: Clarivet, Torres, Washington, United States
Authors: Clarivet Torres1, Anthony Sandler1, Sona Sehgal1, Artis Krystal1, Pennigton Roshnee1, Carola Cerezo1, Stephanie Cahill1, Parvathi Mohan1

Ninety-seven percent survivability, ninety-seven percent resolution of cholestasis at the Intestinal Rehabilitation Program (IRP) - Children’s National Medical Center (CNMC) Washington DC

Clarivet Torres, Anthony Sandler, Sona Sehgal, Artis Krystal, Pennigton Roshnee, Carola Cerezo, Stephanie Cahill, Parvathi Mohan

Intestinal Rehabilitation Program, Children's National Medical Center, Washington, DC, United States

The treatment of intestinal failure is long-term parenteral nutrition (PN), intestinal rehabilitation or transplantation.  PN complications can lead to mortality in up to 50% of pediatric patients. Recent studies suggest that soybean-intralipids (SBIL) contribute to the development of hepatotoxicity, while the Omega 3 fatty acids (Omegaven) may reverse liver disease.  

We analyzed the outcomes of IF-PN-dependent patients treated over four years in the Intestinal Rehabilitation Program at Children’s National Medical Center, Washington-DC.

Seventy-three IF-PN dependent patients, who were on SBIL,were enrolled. Sixty-five had short bowel syndrome. The median age was 6mo. and forty were male. Median intestinal length was 33.5 cm. Median caloric requirements by PN at the time of enrollment were 100%. Thirty-seven had hyperbilirrubinemia, (Mean direct bilirubin of 10.5 mg/dl). Seventeen had liver biopsy (9 had portal fibrosis, 3 bridging, 3 cirrhosis). Height, weight Z score, platelet, albumin, bilirubin were obtained at the beginning and end of the study..

Thirty-six out of 37 patients (97%) with hyperbilirubinemia normalized their bilirubin. Mean time to normalize bilirubin was 10 weeks. Thirty reversed cholestasis while receiving PN (81%). Among the seventy-three patients, twenty had 24 lengthening procedures (9-Bianchi). Six were listed for intestinal transplant. Two are inactive and had resolved their cholestasis. Three were transplanted. Two died (one on the waiting list, one post-transplant). Of the sixty-eight remaining patients, 50 (73.5%) weaned off PN.  All laboratory parameters showed improvement (p< 0.0001). Overall Survival was 97%.

Patients with intestinal-failure treated in our institution reverse their cholestasis with the use of traditional SBIL in a shorter time and effectively, in contrast to the outcome in recent reports with the use of Omegaven. With meticulous medical/surgical management, patients with advanced liver disease can show improvement in liver functions and nutritional parameters with the ability to discontinue PN and avoid need for transplantation.





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