Wd. Bennett et al., EFFECT OF URIDINE 5'-TRIPHOSPHATE PLUS AMILORIDE ON MUCOCILIARY CLEARANCE IN ADULT CYSTIC-FIBROSIS, American journal of respiratory and critical care medicine, 153(6), 1996, pp. 1796-1801
Citations number
26
Categorie Soggetti
Emergency Medicine & Critical Care","Respiratory System
Cystic fibrosis (CF) is characterized by abnormal airway epithelial el
ectrolyte transport leading to viscous airway secretions that are diff
icult to clear. By enhancing Cl- secretion onto and blocking Na+ absor
ption from the airway surface, treatment with aerosolized uridine 5'-t
riphophate (UTP) plus amiloride may improve the rheology of airway sec
retions and enhance mucociliary clearance in patients with CF. After p
erforming safety studies of aerosolized UTP/amiloride in adult patient
s with CF, we investigated the effects of inhaled vehicle and UTP/amil
oride on mucociliary clearance of [Tc-99m] iron oxide particles from t
he airways of adult patients with CF (n = 14). We found no clinically
significant adverse effects from inhalation of therapeutic doses of UT
P/amiloride. Mean baseline peripheral clearance rates during the first
40 min of clearance measurements were significantly less in patients
with CF than in normal subjects (mean +/- SE: 0.30 +/- 0.05 versus 0.5
4 +/- 0.07%/min, respectively; p = 0.01). Aerosolized UTP and amilorid
e in combination improved mucociliary clearance from the peripheral ai
rways of the CF lungs to near normal values (0.51 +/- 0.09%/min; p = 0
.04) during this period. These data support the concept for the use of
UTP in combination with amiloride as therapy to improve clearance of
secretions from the lungs of patients with CF.