Correlation of chest radiograph pattern with genotype, age, and gender in adult cystic fibrosis: A single-center study

Vaidehi Kaza, Marcia F. Katz, Suzanne Cumming, Adaani E. Frost, Zeenat Safdar

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Introduction: Cystic fibrosis (CF) is a common lethal genetic disorder. The aim of this study was to determine the common chest radiograph (CXR) patterns in adult CF, and correlate disease distribution on CXRs with genotype, age, and gender. Methods: One hundred nine CF patients treated at Baylor Adult Cystic Fibrosis Center were identified. The intake CXR was reviewed and characterized as diffuse bilateral (DB), unilateral, upper lobe (UL), and lower lobe (LL) disease, or relatively normal. Lack of intake CXR, and/or genotype excluded 41 patients from analysis. Results: Of 68 patients, 38 were homozygous for ΔF508 and 30 were heterozygous. Mean age of the population was 30 ± 8 years (± SD) [range, 18 to 48 years]. The most common CXR pattern was DB; 62% had DB, 28% had UL, and 7% had LL predominance. This is in contrast to the UL-predominant CXR pattern commonly described in the pediatric population. In 18 DB patients, archived pediatric films were available, and the average patient age was 15.7 years. DB pattern was present in 16 of 18 CXRs that antedated adult intake CXRs by an average of 12.7 years. Homozygous ΔF508 genotype was identified in 56% of patients and did not distinguish radiologic phenotypes. There was no association between radiograph pattern and identified infecting/colonizing organisms and percentage of predicted FEV1. Conclusions: CF has commonly been reported as an UL disease. However, in this study of adult patients, the common pattern observed was DB. A small subgroup analysis suggests that DB disease was not a pattern of disease evolution but may be present from disease onset.

Original languageEnglish (US)
Pages (from-to)569-574
Number of pages6
JournalChest
Volume132
Issue number2
DOIs
StatePublished - Aug 2007

Fingerprint

Cystic Fibrosis
Thorax
Genotype
Pediatrics
Inborn Genetic Diseases
Population
Phenotype

Keywords

  • Airway
  • Chest radiograph
  • Clinical research
  • Lung function
  • Respiratory care
  • Thoracic radiology

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Correlation of chest radiograph pattern with genotype, age, and gender in adult cystic fibrosis : A single-center study. / Kaza, Vaidehi; Katz, Marcia F.; Cumming, Suzanne; Frost, Adaani E.; Safdar, Zeenat.

In: Chest, Vol. 132, No. 2, 08.2007, p. 569-574.

Research output: Contribution to journalArticle

Kaza, Vaidehi ; Katz, Marcia F. ; Cumming, Suzanne ; Frost, Adaani E. ; Safdar, Zeenat. / Correlation of chest radiograph pattern with genotype, age, and gender in adult cystic fibrosis : A single-center study. In: Chest. 2007 ; Vol. 132, No. 2. pp. 569-574.
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abstract = "Introduction: Cystic fibrosis (CF) is a common lethal genetic disorder. The aim of this study was to determine the common chest radiograph (CXR) patterns in adult CF, and correlate disease distribution on CXRs with genotype, age, and gender. Methods: One hundred nine CF patients treated at Baylor Adult Cystic Fibrosis Center were identified. The intake CXR was reviewed and characterized as diffuse bilateral (DB), unilateral, upper lobe (UL), and lower lobe (LL) disease, or relatively normal. Lack of intake CXR, and/or genotype excluded 41 patients from analysis. Results: Of 68 patients, 38 were homozygous for ΔF508 and 30 were heterozygous. Mean age of the population was 30 ± 8 years (± SD) [range, 18 to 48 years]. The most common CXR pattern was DB; 62{\%} had DB, 28{\%} had UL, and 7{\%} had LL predominance. This is in contrast to the UL-predominant CXR pattern commonly described in the pediatric population. In 18 DB patients, archived pediatric films were available, and the average patient age was 15.7 years. DB pattern was present in 16 of 18 CXRs that antedated adult intake CXRs by an average of 12.7 years. Homozygous ΔF508 genotype was identified in 56{\%} of patients and did not distinguish radiologic phenotypes. There was no association between radiograph pattern and identified infecting/colonizing organisms and percentage of predicted FEV1. Conclusions: CF has commonly been reported as an UL disease. However, in this study of adult patients, the common pattern observed was DB. A small subgroup analysis suggests that DB disease was not a pattern of disease evolution but may be present from disease onset.",
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N2 - Introduction: Cystic fibrosis (CF) is a common lethal genetic disorder. The aim of this study was to determine the common chest radiograph (CXR) patterns in adult CF, and correlate disease distribution on CXRs with genotype, age, and gender. Methods: One hundred nine CF patients treated at Baylor Adult Cystic Fibrosis Center were identified. The intake CXR was reviewed and characterized as diffuse bilateral (DB), unilateral, upper lobe (UL), and lower lobe (LL) disease, or relatively normal. Lack of intake CXR, and/or genotype excluded 41 patients from analysis. Results: Of 68 patients, 38 were homozygous for ΔF508 and 30 were heterozygous. Mean age of the population was 30 ± 8 years (± SD) [range, 18 to 48 years]. The most common CXR pattern was DB; 62% had DB, 28% had UL, and 7% had LL predominance. This is in contrast to the UL-predominant CXR pattern commonly described in the pediatric population. In 18 DB patients, archived pediatric films were available, and the average patient age was 15.7 years. DB pattern was present in 16 of 18 CXRs that antedated adult intake CXRs by an average of 12.7 years. Homozygous ΔF508 genotype was identified in 56% of patients and did not distinguish radiologic phenotypes. There was no association between radiograph pattern and identified infecting/colonizing organisms and percentage of predicted FEV1. Conclusions: CF has commonly been reported as an UL disease. However, in this study of adult patients, the common pattern observed was DB. A small subgroup analysis suggests that DB disease was not a pattern of disease evolution but may be present from disease onset.

AB - Introduction: Cystic fibrosis (CF) is a common lethal genetic disorder. The aim of this study was to determine the common chest radiograph (CXR) patterns in adult CF, and correlate disease distribution on CXRs with genotype, age, and gender. Methods: One hundred nine CF patients treated at Baylor Adult Cystic Fibrosis Center were identified. The intake CXR was reviewed and characterized as diffuse bilateral (DB), unilateral, upper lobe (UL), and lower lobe (LL) disease, or relatively normal. Lack of intake CXR, and/or genotype excluded 41 patients from analysis. Results: Of 68 patients, 38 were homozygous for ΔF508 and 30 were heterozygous. Mean age of the population was 30 ± 8 years (± SD) [range, 18 to 48 years]. The most common CXR pattern was DB; 62% had DB, 28% had UL, and 7% had LL predominance. This is in contrast to the UL-predominant CXR pattern commonly described in the pediatric population. In 18 DB patients, archived pediatric films were available, and the average patient age was 15.7 years. DB pattern was present in 16 of 18 CXRs that antedated adult intake CXRs by an average of 12.7 years. Homozygous ΔF508 genotype was identified in 56% of patients and did not distinguish radiologic phenotypes. There was no association between radiograph pattern and identified infecting/colonizing organisms and percentage of predicted FEV1. Conclusions: CF has commonly been reported as an UL disease. However, in this study of adult patients, the common pattern observed was DB. A small subgroup analysis suggests that DB disease was not a pattern of disease evolution but may be present from disease onset.

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