Chest
Clinical InvestigationsCYSTIC FIBROSISClinical Manifestations of Cystic Fibrosis Among Patients With Diagnosis in Adulthood
Section snippets
Patient Ascertainment
In Toronto, patients with CF attend the pediatric CF Program until age 16 to 18 years, when they are transferred to the adult CF Program. All patients who received a diagnosis at the Toronto Adult and Pediatric CF Clinics between January 1960 and June 2001, with focus on patients who received a diagnosis in adulthood since 1990 (after the institution of a separate Adult CF Program), were included in the study. Patients with CF diagnosed between 1944 and 1959 (92 children) were excluded due to
Patients
The number of patients (approximately 550 patients) attending the Toronto CF clinics has remained quite constant over the last 10 years. However, the proportion of adults has increased and now exceeds 50% of the population. Of 1,051 patients (53% male patients) with CF diagnosed in the Toronto CF clinics since 1960, 73 patients (7%) had CF diagnosed in adulthood. The number and proportion of patients with CF diagnosed in adulthood has increased dramatically over the past 3 decades (Table 1).
Clinical Findings at Diagnosis in all Adult Patients With CF Diagnosed Between 1960 and 2001
The
Discussion
This study demonstrates that the proportion and number of patients with CF diagnosed in adulthood has increased. A large number of these patients presented with subtle symptoms or single-organ disease. The majority had pulmonary disease and PS. In one third of these patients, the diagnosis of CF could not be confirmed by sweat testing, or by analysis of CF-causing mutations based on the current criteria.2 Nasal PD measurements proved to be an important diagnostic tool for confirmation of
ACKNOWLEDGMENT
The authors thank Sandi Peroff for help with the data collection.
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At the time of the study, Dr. Gilljam was a clinical fellow in the Adult CF Program, St Michael's Hospital Toronto, where the study was undertaken.
Dr. Gilljam was supported by the Swedish Medical Society, Gothenburg Medical Society, and by King Oscar II Jubilee Foundation.
Funding was provided by the Canadian Cystic Fibrosis Foundation and NIH-DK49096–09.