Disparities in Mortality of Hispanic Patients with Cystic Fibrosis in the United States. A National and Regional Cohort Study

Rho, Jason; Ahn, Chul; Gao, Ang; Sawicki, Gregory S.; Keller, Ashley; Jain, Raksha

doi:10.1164/rccm.201711-2357oc

Cited by 55 publications

(50 citation statements)

References 23 publications

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“…Respiratory infections, which negatively affect pulmonary function, are known to vary by ethnicity and by geographic region outside of CF. We found that Hispanic subjects were more likely to have P. aeruginosa than non‐Hispanic white subjects in CF.…”

Section: Discussionmentioning

confidence: 99%

“…Despite great advancements in care and outcomes, Hispanic patients with cystic fibrosis (CF) have worse outcomes compared to non‐Hispanic white patients. Hispanic patients have a higher rate of mortality than do non‐Hispanic white patients and Hispanic youth with CF have a worse pulmonary function . Our group has shown that Hispanic youth with CF have 5.8% lower percent‐predicted forced expiratory volume in 1 second (FEV 1 ) compared with non‐Hispanic white youth .…”

Section: Introductionmentioning

confidence: 90%

“…Our group has shown that Hispanic youth with CF have 5.8% lower percent‐predicted forced expiratory volume in 1 second (FEV 1 ) compared with non‐Hispanic white youth . The ethnic disparity in CF mortality varies significantly between United States Census regions . It is not known whether the ethnic disparity in pulmonary function also varies across geographic regions in the United States.…”

Section: Introductionmentioning

confidence: 99%

“…Given that Hispanic patients have higher mortality rates than non‐Hispanic white patients which varied by geographic region and that factors influencing pulmonary function vary by both ethnicity and geographic region, we sought to examine if pulmonary function disparities between ethnicities also vary between US geographic regions. We used the largest database of Hispanic and non‐Hispanic white subjects with CF, the United States CF Foundation Patient Registry (CFFPR), to compare whether pulmonary function between ethnicities varies by geographic region of the United States.…”

Section: Introductionmentioning

confidence: 99%

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Regional variations in longitudinal pulmonary function: A comparison of Hispanic and non‐Hispanic subjects with cystic fibrosis in the United States

McGarry

Neuhaus

Nielson

et al. 2019

Pediatric Pulmonology

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Background Hispanic subjects with cystic fibrosis (CF) have increased morbidity and mortality than non‐Hispanic white subjects. The ethnic disparity in mortality varies by region. Factors influencing pulmonary function vary by both ethnicity and region. Objective To determine if the ethnic difference in pulmonary function varies by region. Methods This retrospective cohort study compared differences in longitudinal pulmonary function (percent‐predicted forced vital capacity [FVC], forced expiratory volume in 1 second [FEV1], forced expiratory flow at 25% to 75% [FEF25‐75], FEV1/FVC, and FEV1 decline) between Hispanic and non‐Hispanic white subjects with CF by Census region of the United States (West, South, Midwest, and Northeast). Subjects were of ages 6 to 25 years and in the CF Foundation Patient Registry from 2008 to 2013. We used linear mixed effects models with subject‐specific slopes and intercepts, adjusting for 14 demographic and clinical variables. Results Of 14 932 subjects, 1433 (9.6%) were Hispanic and 13 499 (90.4%) were non‐Hispanic white. Hispanic subjects’ FEV1 was 9.0% (8.3%‐9.8%) lower than non‐Hispanic white subjects in the West, while Hispanic subjects’ FEV1 was only 4.0% (3.0%‐5.0%) lower in the Midwest, 4.4% (3.1%‐5.7%) lower in the Northeast, and 4.4% (3.2%‐5.5%) lower in the South. Similarly, FVC and FEF25‐75 were lower among Hispanic subjects compared to non‐Hispanic white subjects in all US regions, with the biggest differences in the West. Only in the West was FEV1/FVC significantly lower in Hispanic subjects (−0.019; −0.022 to −0.015). FEV1 decline was not significantly different between ethnicities in any region. Conclusions In CF, Hispanic subjects have lower pulmonary function than non‐Hispanic white subjects in all geographic regions with the largest difference in occurring in the West.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 90%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Regional variations in longitudinal pulmonary function: A comparison of Hispanic and non‐Hispanic subjects with cystic fibrosis in the United States

McGarry

Neuhaus

Nielson

et al. 2019

Pediatric Pulmonology

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“…In the US, an evaluation of treatment and outcome variation in Hispanic patients with CF, demonstrated reduced survival by 1.4 years and 1.27 higher risk of death compared with non‐Hispanic patients, even when adjusted for additional covariants. In addition, geographic variation was seen, with the worst survival for Hispanic patients in the Midwest . While these survival estimates included patients in specific subgroups, a study from the UK CF Registry showed a more accurate reflection of survival, based on age at diagnosis, current age, sex, or mutation class, were developed …”

Section: Infection Detection and Epidemiologymentioning

confidence: 99%

Cystic fibrosis year in review 2018, part 1

Savant

McColley

2019

Pediatric Pulmonology

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The CFTR variant profile of Hispanic patients with cystic fibrosis: Impact on access to effective screening, diagnosis, and personalized medicine

Januska

Marx

Walker

et al. 2020

Journal of Genetic Counseling

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Hispanic patients comprise an appreciable and increasing proportion of patients with cystic fibrosis (CF) in the United States (US). Hispanic patients with CF are known to have increased morbidity and mortality compared to non‐Hispanic white patients with CF, and ongoing investigations are underway to identify contributing factors amenable to intervention in order to address the disparate health outcomes. One contributing factor is the different CF transmembrane conductance regulator (CFTR) variant profile observed in Hispanic patients with CF. The most common CFTR variant, p.Phe508del (legacy name F508del), is proportionally underrepresented in Hispanic patients with CF. This difference has implications for prenatal screening, newborn screening (NBS), and CFTR variant‐specific therapeutic options. In particular, the recent approval of a highly effective CFTR modulator for patients carrying at least one copy of F508del, elexacaftor/tezacaftor/ivacaftor triple combination therapy, underscores the potential for unequal access to personalized treatment for Hispanic patients with CF. We report the CFTR variant profiles of Hispanic patients with CF and non‐CF Hispanic infants with a false‐positive New York State CF NBS at a single center in New York City over a 5‐year study period, as an opportunity to address the racial and ethnic disparities that currently exist in CF screening, diagnosis, and treatment. In addition to the previously documented disparate prevalence of the CFTR variant F508del in Hispanic patients, we observed two CFTR variants, p.His609Arg (legacy name H609R) and p.Thr1036Asn (legacy name T1036N), frequently identified in our Hispanic patients of Ecuadorian and Mexican ancestry, respectively, that are not well‐described in the US population. The presence of population‐specific and individually rare CFTR variants in Hispanic patients with CF further accentuates the disparity in health outcomes, as these CFTR variants are often absent from prenatal and NBS CFTR variant panels, potentially delaying diagnosis, and without an approved CFTR variant‐specific therapy.

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Disparities in Mortality of Hispanic Patients with Cystic Fibrosis in the United States. A National and Regional Cohort Study

Abstract: Patients with CF of Hispanic origin have a higher mortality rate than non-Hispanic patients with CF. This pattern was seen in the Midwest, Northeast, and West but not in the South.

Cited by 55 publications

References 23 publications

Regional variations in longitudinal pulmonary function: A comparison of Hispanic and non‐Hispanic subjects with cystic fibrosis in the United States

Regional variations in longitudinal pulmonary function: A comparison of Hispanic and non‐Hispanic subjects with cystic fibrosis in the United States

Cystic fibrosis year in review 2018, part 1

The CFTR variant profile of Hispanic patients with cystic fibrosis: Impact on access to effective screening, diagnosis, and personalized medicine

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