The aim of this study was to assess the prognostic significance of muc
oid and non-mucoid isolates of Pseudomonas aeruginosa (muPs and non-mu
Ps) from the sputa of patients with cystic fibrosis (CF). Eighty-one c
hildren with CF who coughed up sputum daily were recruited and followe
d over 12 months with frequent sputum cultures. At the end of this obs
ervation period they were classified to one of three age-matched group
s. In 50 mPs was isolated on one or more occasions; 19 grew non-muPs b
ut not muPs, and 12 grew no isolates of Ps aeruginosa. These 81 childr
en and adolescents were followed for a further 8 years or until they d
ied. Twenty-one (42%) of the muPs patients died compared with two (11%
) of the non-muPs and one (8%) of the no Ps patients (P < 0.01). Stepw
ise regression indicated that forced expiratory volume in 1 second (FE
V1) had the main predictive effect but that age, Shwachman score and m
uPs also had a predictive effect. Identification of mucoid forms of Ps
aeruginosa is an unfavorable prognostic factor but the isolation of n
on-mucoid strains does not appear to be any more important than the is
olation of other common respiratory pathogens.