Liver Transplant Stabilizes Lung Function in Cystic Fibrosis

For selected patients with cystic fibrosis liver disease, transplant preserves nutritional status

WEDNESDAY, Jan. 18 (HealthDay News) -- For a select group of patients with cirrhosis due to cystic fibrosis liver disease (CFLD), liver transplantation can stabilize long-term lung function and nutritional status and reduce the need for intravenous antibiotics, according to a study published online Jan. 6 in the American Journal of Transplantation.

Joanna K. Dowman, M.D., of the University of Birmingham in the United Kingdom, and colleagues conducted a retrospective analysis of 21 adult and 19 pediatric patients with CFLD who had undergone a single liver transplantation; the patients were followed for an average of 47.8 months. Lung function was assessed using forced expiratory volume in one second. Body mass index (BMI) was used to assess nutritional status.

The investigators found that, at one year, survival rates were 85 and 90 percent for adults and pediatric patients, respectively. The corresponding survival rates at five years were 64 and 85 percent. Lung function was stable up to four years, with a mean annual decline in lung function of 0.74 percent per year (48.4 percent pre-transplant versus 45.9 percent at four years). Lung function declined by five years (42.4 percent). Intravenous antibiotic use was reduced from a pre-transplant rate of 3.9 courses per year to 1.1 courses per year for the first five years post-transplant. BMI remained stable: 18.0 kg/m² pre-transplant and 19.6 kg/m² at five years post-transplant.

"We suggest that liver transplantation is an effective treatment for patients with decompensated cirrhosis due to CFLD, even in the presence of moderately impaired lung function," the authors write.

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