Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population

• DOI: 10.1034/j.1399-3046.2003.00021.x
• Publication Date: 2004-12-28T17:10:27Z
• Authors: Ernesto P. Molmenti; Robert H. Squires; David Nagata; Jay S. Roden; Hebe Molmenti; Carlos G. Fasola; Claude Prestidge; Lisa D'Amico; Donna Casey; Edmund Q. Sanchez; Robert M. Goldstein; Marlon F. Levy; Margareta Benser; Wanda McPhail; Walter Andrews; John A. Andersen; Goran B. Klintmalm
• Keywords: Liver disease

The most common hepatic complications of cystic fibrosis (CF) are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis. Cholestatic liver disease is a slow progressive disorder, but will stabilize for many patients. CF patients may suffer from the consequences their without transplantation, variceal hemorrhage, malnutrition, or end-stage can lead to death. Prospective data were collected reviewed on 311 transplants performed in 283 at Children's Medical Center Dallas between October 1984 November 2000. Ten children received an orthotopic transplant (OTLX) associated with fibrosis. Pulmonary function tests obtained preoperatively all cases. There nine boys one girl. Six currently alive, four dead. Both patient graft survival was 5.75 yr. Among those mean 7.71 yr (range 0.10-12.62 yr). Mean who died 2.35 0.78-5.33 No survivor required re-transplantation currently, have normal serum aminotransferase values. Chronic sinusitis not significant pre- post-transplant morbidity, although systematic radiographic evaluation sinuses did occur. deaths occurred three pulmonary infection Aspergillus Candida glabrata, acute bronchopneumonia polymicrobial sepsis because Pseudomonas, Klebsiella, albicans 1.44, 0.78, 1.83 yr, respectively, after transplantation. fourth death chronic rejection, 5.33 All non-survivors below 5th percentile height weight time age transplantation 9.72 1.23-19.09, median 9.61). Survivors transplanted younger than (mean 9.21 vs. 10.66 yr), had shorter waiting times diagnosis (6.87 months 13.83 months). Eighty percentage (n = 8) pretransplant bleeds (83% survivors, 75% non-survivors). While history meconium ileus preoperative need pancreatic enzymes, only 67% alive experienced these complications. Preoperative forced vital capacity FVC 103% survivors 95% non-survivors. corresponding numbers expiratory flow (FEF) 25-75 74-84% respectively. identified 30% 3). Two alive. Cystic constitutes indication 3.5% pediatric transplants. Evaluation should be undertaken early age. Most pulmonary/septic events, less 2 OLTX. Those survive poor growth nutrition, prolonged prior older age, higher incidence insufficiency ileus. presence sputum does constitute contraindication