Published ahead of print on December 10, 2004, doi:10.1164/rccm.200403-404OC Am. J. Respir. Crit. Care Med., Volume 171, Number 6, March 2005, 621-626 A more recent version of this article appeared on March 15, 2005
Submitted on March 30, 2004 Late Diagnosis Defines a Unique Population of Long-term Survivors of Cystic FibrosisDavid M Rodman1*,1 Cystic Fibrosis Center, The University of Colorado Health Sciences Center, Denver, CO, USA, 2 National Jewish Medical and Research Center, Denver, CO, USA, 3 The Children's Hospital, Denver, CO, USA, 4 The Children's Hospital, Denver, CO, USA; Cystic Fibrosis Center, The University of Colorado Health Sciences Center, Denver, CO, USA, 5 National Jewish Medical and Research Center, Denver, CO, USA; Cystic Fibrosis Center, The University of Colorado Health Sciences Center, Denver, CO, USA * To whom correspondence should be addressed. E-mail: David.Rodman{at}uchsc.edu.
While the median survival for CF patients is 32.9 years, a small group of patients live much longer. We analyzed the genotype and phenotype of CF patients age 40 and older seen between 1992-2004 at National Jewish Medical and Research Center (n=55). These patients were divided into two groups based on age of diagnosis: an early diagnosis (ED) group, median age of diagnosis 2.0 (range 0.1-15, n=28) and a late diagnosis (LD) group, median age of diagnosis 48.8 (range 24-72.8, n=27) years. Consistent with the hypothesis that the CFTR genotype affects the age of diagnosis, CFTR Key words: Cystic Fibrosis, nontuberculous mycobacteria, Pseudomonas aeruginosa, genotype
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F508 homozygous individuals were more common in the ED group. While patients in the ED group were predominantly male, the majority of LD patients were female. CF patients diagnosed late had a significantly lower prevalence of pancreatic insufficiency, CF-related diabetes, and better lung function. Fewer patients in the LD groups were infected with P. aeruginosa, while a greater percentage had cultures positive for nontuberculous mycobacteria. This is the largest cohort of older CF patients described to date, and our findings indicate that patients diagnosed as adults differ distinctly from long-term CF survivors diagnosed as children.
