How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old

Abstract

Background and aimsTo specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics.Patients and methodsObservational study of CF patients which were diagnosed at age ≥60 year-old. The analyzed variables were: age, sex, nationality, lung function parameters, conditions present at diagnosis, microbiological characteristics and genetic findings.Resultseight patients were included. 7 patients were female (87.5%) with a mean age of 70.6 years (median 71.5 years, range 60–78 years). The most important findings were: sweat test >60 mEq/l; heterozygotes F508del; bronchiectasis in CT; methicillin-sensitive Staphylococcus aureus (50%) in sputum. The most patients presented a normal or mild obstructive lung function.ConclusionsCF must also be considered a disease diagnosed in adulthood, incorporating the sweat test within the usual techniques of differential diagnosis in patients with different diseases associated with CF, because genetic counselling is esencial.

Atypical presentation of cystic fibrosis at 70 years of age

“…The incidence of CF is approximately one in 2500 live births in the UK. Majority of the patients are diagnosed by 3 years of age and it is extremely rare for CF to be diagnosed after 65 years of age 1 2…”

“…CF was long considered to be a disease of childhood but it is being diagnosed in adults with increasing frequency. It is still a rare entity in patients over the age of 65 1. Diagnosis can be made with genetic testing and sweat chloride when patient presents with multiple organ involvement but it can be quite challenging if there is atypical presentation with single organ involvement and borderline or normal sweat chloride.…”