Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Jan 19;13(2):585.
doi: 10.3390/jcm13020585.

Cystic Fibrosis in Adults: A Paradigm of Frailty Syndrome? An Observational Study

Paola Iacotucci  1 , Vincenzo Carnovale  2 , Lorenza Ferrillo  2 , Jolanda Somma  2 , Marialuisa Bocchino  1 , Marcella D'Ippolito  2 , Alessandro Sanduzzi Zamparelli  1 , Giuseppe Rengo  2 , Nicola Ferrara  2 , Valeria Conti  3 , Graziamaria Corbi  2
Affiliations

Cystic Fibrosis in Adults: A Paradigm of Frailty Syndrome? An Observational Study

Paola Iacotucci et al. J Clin Med. .

Abstract

This study aimed to assess the main clinical and anamnestic characteristics of adult Cystic Fibrosis (CF) patients and to evaluate the association of frailty with the CF genotyping classification. In an observational cross-sectional study, all ambulatory CF patients over 18 years old who received a diagnosis at the Regional Cystic Fibrosis Center for adults were enrolled and assessed by spirometry for respiratory function, by ADL and IADL for functional status, and by the Study of Osteoporotic Fractures (SOF) Index for frailty. The study population consisted of 139 CF patients (mean age 32.89 ± 10.94 years old, 46% women). Most of the subjects were robust (60.4%). The pre-frail/frail group was more frequently females (p = 0.020), had a lower BMI (p = 0.001), worse respiratory function, a higher number of pulmonary exacerbations/years, cycles of antibiotic therapy, and hospitalization (all p < 0.001) with respect to robust patients. The pre-frail/frail subjects used more drugs and were affected by more CF-related diseases (all p < 0.001). In relation to logistic regression, the best predictor of the pre-frail/frail status was a low FEV1 level. The CF patients show similarities to older pre-frail/frail subjects, suggesting that CF might be considered an early expression of this geriatric syndrome. This finding could help to better define the possible progression of CF, but overall, it could also suggest the usefulness employing of some tools used in the management and therapy of frailty subjects to identify the more severe CF subjects.

Keywords: Cystic Fibrosis; frailty; functional status; geriatric syndrome.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Prevalence of the main causes of infections in the study population stratified by frailty status.
Figure 1
Figure 1
Prevalence of the main causes of infections in the study population stratified by frailty status.
Figure 2
Figure 2
Differences in genotyping classification of the study population by frailty status.
See this image and copyright information in PMC

References

    1. Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., Zielenski J., Lok S., Plavsic N., Chou J.L. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science. 1989;245:1066–1073. doi: 10.1126/science.2475911. - DOI - PubMed
    1. Kerem B., Rommens J.M., Buchanan J.A., Markiewicz D., Cox T.K., Chakravarti A., Buchwald M., Tsui L.C. Identification of the cystic fibrosis gene: Genetic analysis. Science. 1989;245:1073–1080. doi: 10.1126/science.2570460. - DOI - PubMed
    1. Campagna G., Amato A., Majo F., Ferrari G., Quattrucci S., Padoan R., Floridia G., Salvatore D., Carnovale V., Puppo Fornaro G., et al. Rapporto 2019–2020 [Italian Cystic Fibrosis Registry (ICFR). Report 2019–2020] Epidemiol. Prev. 2022;46((Suppl. 2)):1–38. doi: 10.19191/EP22.4S2.060. - DOI - PubMed
    1. Rowe S.M., Miller S., Sorscher E.J. Cystic fibrosis. N. Engl. J. Med. 2005;352:1992–2001. doi: 10.1056/NEJMra043184. - DOI - PubMed
    1. Orenti A., Zolin A., Jung A., van Rens J., Adamoli A., Prasad V., Fox A., Krasnyk M., Mayor S.L., Naehrlich L., et al. ECFSPR Annual Report 2021. 2023. [(accessed on 20 July 2023)]. Available online: https://www.ecfs.eu/sites/default/files/Annual%20Report_2021_09Jun2023.pdf.

LinkOut - more resources