Autoimmunity in people with cystic fibrosis

Chadwick, Christina; Lehman, Heather K.; Luebbert, Shelby; Abdul-Aziz, Rabheh; Borowitz, Drucy

doi:10.1016/j.jcf.2023.03.007

Journal of Cystic Fibrosis

2023

DOI: 10.1016/j.jcf.2023.03.007

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Autoimmunity in people with cystic fibrosis

Christina Chadwick¹,

Heather K. Lehman²,

Shelby Luebbert³

et al.

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2024

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Cited by 2 publications

References 109 publications

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Improving growth in infants with CF

Gabel,

Gaudio,

Shaikhkhalil

2024

Pediatric Pulmonology

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Cystic fibrosis (CF) results in chronic pulmonary infections, inflammation, pancreatic insufficiency, and multiple gastrointestinal manifestations. Malnutrition and poor growth are hallmarks of CF, and strongly associated with poor outcomes. Through newborn screening, many infants can be diagnosed within a few days of life, which allows for early initiation of nutritional counseling and close clinical follow‐up. Obstacles to growth for infants with CF start in utero, as newborns with CF can have a lower birth weight than the general population. Improving infant growth has been linked to improved clinical outcomes and survival. It remains a top priority and challenge for caregivers and healthcare teams. An interdisciplinary approach, including registered dietitian and social work support, is essential to optimize health for infants with CF. Remaining barriers to normalcy include deficits in linear growth, lack of accurate nutrition biomarkers, persistence of inequities related to social determinant of health, particularly in the global CF community.

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Improving growth in infants with CF

Gabel,

Gaudio,

Shaikhkhalil

2024

Pediatric Pulmonology

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The ageing of people living with cystic fibrosis: what to expect now?

Felipe Montiel,

Fernández,

Amigo

et al. 2024

Eur Respir Rev

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The prognosis of people with cystic fibrosis (pwCF) has improved dramatically with the introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators (CFTRm). The ageing of the cystic fibrosis (CF) population is changing the disease landscape with the emergence of different needs and increasing comorbidities related to both age and long-term exposure to multiple treatments including CFTRm. Although the number of pwCF eligible for this treatment is expected to increase, major disparities in care and outcomes still exist in this population. Moreover, the long-term impact of the use of CFTRm is still partly unknown due to the current short follow-up and experience with their use, thus generating some uncertainties. The future spread and initiation of these drugs at an earlier stage of the disease is expected to reduce the systemic burden of systemic inflammation and its consequences on health. However, the prolonged life expectancy is accompanied by an increasing burden of age-related comorbidities, especially in the context of chronic disease. The clinical manifestations of the comorbidities directly or indirectly associated with CFTR dysfunction are changing, along with the disease dynamics and outcomes. Current protocols used to monitor slow disease progression will need continuous updates, including the composition of the multidisciplinary team for CF care, with a greater focus on the needs of the adult population.

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Autoimmunity in people with cystic fibrosis

Cited by 2 publications

References 109 publications

Improving growth in infants with CF

Improving growth in infants with CF

The ageing of people living with cystic fibrosis: what to expect now?

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