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
. 2022 Jun;57(6):1513-1519.
doi: 10.1002/ppul.25882. Epub 2022 Mar 14.

Cystic fibrosis lung transplant recipients 10 years of age or younger: Predisposing factors for end-stage disease

Ernestina Melicoff  1 , Fadel E Ruiz  1 , Kathleen Hosek  2 , George B Mallory  1
Affiliations

Cystic fibrosis lung transplant recipients 10 years of age or younger: Predisposing factors for end-stage disease

Ernestina Melicoff et al. Pediatr Pulmonol. 2022 Jun.

Abstract

Background: The largest age group among children and adolescents referred for lung transplantation for cystic fibrosis (CF) have been those in the pubertal or postpubertal age range. However, over 100 younger patients with CF have undergone lung transplantation over the last three decades in the United States.

Methods: We performed a retrospective review of our experience with 18 children with CF who underwent lung transplantation in our center before the age of 11 years and compared them to our older CF lung transplant recipients and our larger CF Center population.

Results: The transplant population was demographically distinct from our CF center in terms of ethnicity, country of origin, and insurance status. Other notable findings were a high prevalence of methicillin-resistant Staphylococcus aureus, a high prevalence of CF-related diabetes mellitus, and a high prevalence of consolidated lobar or whole lung disease. Posttransplant outcomes were comparable to those older than 10 years of age in our center until 5 years after transplant after which the younger cohort showed a superior enduring survival.

Conclusions: In an era of increasingly effective medications modifying the natural history of CF, identification of risk factors for early severe lung disease in CF remains relevant to permit interventions to prevent or postpone the time of future lung transplantation.

Keywords: cystic fibrosis; diabetes mellitus; lobar consolidation; lung transplantation; respiratory failure.

PubMed Disclaimer

References

REFERENCES

    1. Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D. Up-to-date and projected estimate for people with cystic fibrosis using baseline characteristics: a longitudinal study using UK patient registry data. J Cyst Fibros. 2018;17:218-227.
    1. Stephenson AL, Tom M, Berthiaume Y, et al. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. Eur Resp J. 2015;45:670-679.
    1. MacKenzie T, Gifford AH, Sabadosa KA, et al. Longevity of patients with cystic fibrosis in 2000-2010 and beyond: survival analysis of the cystic fibrosis foundation patient registry. Ann Intern Med. 2014;161:233-241.
    1. Farrell PM, Lai HJ, Li Z, et al. Evidence on improved outcomes with early diagnosis of cystic fibrosis through neonatal screening: enough is enough! J Pediatr. 2005;147:S30-S36.
    1. Tridello G, Castellini C, Menegheilli I, Tamanini A, Assael BM. Early diagnosis from newborn screening maximizes survival in severe cystic fibrosis. ERJ Open Res. 2018;(2):00109-2017. doi:10.1183/23120541.00109-2017