Defining an exacerbation of pulmonary disease in cystic fibrosis

Despite the importance of pulmonary exacerbations in CF in both clinical an d research settings, both published evidence and consensus are lacking conc erning the criteria used to define an exacerbation. The use of hospitalizat ion as a surrogate measure presupposes uniformity among clinicians in diagn osis and treatment of exacerbations. Our aims were to evaluate consensus am ong clinicians about the variables considered helpful in diagnosing an exac erbation requiring treatment. A comprehensive list of symptoms, signs, and investigations used to define exacerbations was compiled from published tri als. A written self-administered questionnaire included the list in age-app ropriate groups to survey opinion about the helpfulness of each item, and t he estimated proportion of patients admitted within a month of diagnosis of an exacerbation. This was sent to all clinicians managing CF patients in A ustralia. There were replies from 59/91 clinicians (65%), 41/60 (68%) from those mana ging children and 18/31 (58%) from those managing adults. Responses of thos e managing children and adults differed for 7/32 variables (Mann-Whitney te st, P < 0.05). Clinic grouping did not show greater consensus among respons es of pediatricians (Kruskal-Wallis test, P = 0.362). Consensus, > 74% or < 26% of respondents rating a variable helpful/very helpful, was found in on ly 50% of variables listed. Estimated admission rate within a month of diag nosis was 61% (30-100%) for those managing adults and 48% (5-100%) for pedi atricians. A lack of consensus was found among clinicians managing CF about the variab les considered in diagnosing an exacerbation. The estimated proportion admi tted within a month of diagnosis was very variable. This demonstrated inhom ogeneity in approach to diagnosis and management of an exacerbation suggest s a significant heterogeneity of clinical care. (C) 2001 Wiiey-Liss, Inc.