| Autor: |
Molmenti, Ernesto P., Squires, Robert H., Nagata, David, Roden, Jay S., Molmenti, Hebe, Fasola, Carlos G., Prestidge, Claude, D'Amico, Lisa, Casey, Donna, Sanchez, Edmund Q., Goldstein, Robert M., Levy, Marlon F., Benser, Margareta, McPhail, Wanda, Andrews, Walter, Andersen, John A., Klintmalm, Goran B. |
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| Zdroj: |
Pediatric Transplantation; Apr2003, Vol. 7 Issue 2, p93-97, 5p |
| Abstrakt: |
Abstract: 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 of their liver disease and without liver transplantation, variceal hemorrhage, malnutrition, or end-stage liver disease can lead to death. Prospective data were collected and reviewed on 311 liver transplants performed in 283 patients at the Children's Medical Center of Dallas between October 1984 and November 2000. Ten children received an orthotopic liver transplant (OTLX) for end-stage liver disease associated with cystic fibrosis. Pulmonary function tests were obtained preoperatively in all cases. There were nine boys and one girl. Six are currently alive, and four are dead. Both patient and graft survival was 5.75 yr. Among those currently alive, mean patient and graft survival is 7.71 yr (range 0.10–12.62 yr). Mean patient and graft survival of those who died was 2.35 yr (range 0.78–5.33 yr). No survivor required re-transplantation and currently, all have normal serum aminotransferase values. Chronic sinusitis was not a significant pre- or post-transplant morbidity, although systematic radiographic evaluation of the sinuses did not occur. Pulmonary deaths occurred in three patients from pulmonary hemorrhage, pulmonary infection with Aspergillus and Candida glabrata , and acute bronchopneumonia associated with polymicrobial sepsis because of Pseudomonas , Klebsiella , and Candida albicans 1.44, 0.78, and 1.83 yr, respectively, after transplantation. The fourth death was associated with chronic rejection, and occurred 5.33 yr after transplantation. All non-survivors were below the 5th percentile for height and weight at the time of liver transplantation. Mean age at transplantation was 9.72 yr (range 1.23–19.09, median... [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
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