Originally posted by Joanne Schum, cf lung transplant suvivor

[Guest guest]

Pediatric Transplantation

Volume 7 Issue 3 Page 240 - June 2003

Liver and intestinal transplantation in a child with cystic fibrosis: A

case report

A. Fridell3, V. Mazariegos1, Orenstein2, Rakesh

Sindhi1 and Reyes1

Abstract: Cystic fibrosis (CF) is an inherited disorder that presents as

a multisystem disease with meconium ileus being the presenting symptom in

20% of patients. Approximately half of these patients present with

complicated meconium ileus mandating early surgical intervention,

potentially resulting in short gut syndrome. Although liver

transplantation in children with CF has been described, this is the first

report of a combined liver and small bowel transplant in a recipient with

CF. A 7-month-old boy with CF presented with short bowel syndrome

following extensive small bowel resection for meconium ileus and

progressive cholestatic liver failure from intravenous hyperalimentation.

He underwent combined liver and small intestinal transplant. He was

discharged home three weeks post-transplant on enteral feeds with

supplemental intravenous fluid. He has had routine protocol small bowel

allograft biopsies with no documented rejection episodes. He has been

treated for minor respiratory infections without major sequelae.

Improvements in pulmonary therapy have impacted on the survival in the CF

population to the point where the need for multiorgan transplantation

will be increased in the future. Extrapolating from the excellent

experience of liver transplantation in children with CF, early liver and

small intestinal multivisceral transplantation, if indicated, can be

performed safely in children with CF.