From between 1996 to November 1997 13 bilateral lung transplantation i
n Cystic Fibrosis patients (4 m, 9 f) aged 19-38 years (mean 26 years)
are performed. Pre Tx evaluation included: Shwachman Score, Chrispin
Score, HR chest tomography, respiratory function tests, pulmonary perf
usion and ventilation scans, cardiac catheterism. The basterial, funga
l and viral status of each patient was also evaluated. Immunosoppressi
on was achieved with cyclosporine 2 mg/kg/day, azathioprine 2 mg/Kg/da
y; ALG (10 mg/Kg/day) for 5 days and metilprednisolone 125 mg x 3 day
for 3 days. 11 pts are alive after a follow up from 5-16 months, 2 pts
(1 m 1 f) died for sepsis and multiorgam failure, 2 pts needed Nd-Yag
Laser and 1 of them stenting of the bronchus for bronchial anastomoti
c stricture. Other complication were: diabetes (3), seizures (2), rena
l failure (2), meconium ileus equivalent (1), acute rejection (4), bac
terial pulmonary infection (10), CMV infection (10), varicella (1). Sh
wachman score modified (Shw score) and respiratory function (FEV1) imp
roved (Shwachman score mean: pre Tx 43/75 vs post Tx 70/75; FEV1 mean:
pre Tx 16% vs 77% at 6 months post Tx and 72% at 12 months post Tx).
This reports documents that, for CF pts end-stage lung disease, double
lung transplantation can offer an improved survival and quality of li
fe.