Atypical presentation of cystic fibrosis at 70 years of age

Cystic fibrosis (CF) is an autosomal recessive condition, mostly diagnosed in infancy. It is uncommon for adults to be diagnosed with CF, especially after the age of 65. Individuals, who are diagnosed later in life, usually have milder disease and single organ involvement which can be challenging fo...

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Bibliographic Details
Published in:BMJ case reports 2019-06, Vol.12 (6), p.e228821
Main Authors: Saleem, Muhammad Adnan, Phillips, Aled, Ganaie, Muhammad Badar
Format: Article
Language:English
Subjects:
Age
Aged
Bronchiectasis
Case reports
Chloride
Cystic fibrosis
Cystic Fibrosis - diagnosis
Cystic Fibrosis - diagnostic imaging
Cystic Fibrosis - genetics
Cysts
Diagnosis, Differential
Female
Humans
Medical screening
Mutation
Patients
Pneumonia
Pneumonia, Staphylococcal
Rare Disease
Staphylococcus aureus - isolation & purification
Staphylococcus infections
Thorax
Tomography, X-Ray Computed
Citations: Items that this one cites
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Description
Summary:Cystic fibrosis (CF) is an autosomal recessive condition, mostly diagnosed in infancy. It is uncommon for adults to be diagnosed with CF, especially after the age of 65. Individuals, who are diagnosed later in life, usually have milder disease and single organ involvement which can be challenging for clinicians to diagnose. Adult CF patients are more likely to be pancreatic sufficient. They have predominantly upper lobe bronchiectasis, lower incidence of Pseudomonas aeruginosa compared with Staphylococcus aureus and are more likely to have mutations other than ΔF508.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2018-228821