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Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants.

Publication ,  Journal Article
Daniels, MLA; Birchard, KR; Lowe, JR; Patrone, MV; Noone, PG; Knowles, MR
Published in: Ann Am Thorac Soc
October 2016

RATIONALE: Patients with idiopathic bronchiectasis are predominantly female and have an asthenic body morphotype and frequent nontuberculous mycobacterial respiratory infections. They also demonstrate phenotypic features (scoliosis, pectus deformity, mitral valve prolapse) that are commonly seen in individuals with heritable connective tissue disorders. OBJECTIVES: To determine whether lumbar dural sac size is increased in patients with idiopathic bronchiectasis as compared with control subjects, and to assess whether dural sac size is correlated with phenotypic characteristics seen in individuals with heritable connective tissue disorders. METHODS: Two readers blinded to diagnosis measured anterior-posterior and transverse dural sac diameter using L1-L5 magnetic resonance images of 71 patients with idiopathic bronchiectasis, 72 control subjects without lung disease, 29 patients with cystic fibrosis, and 24 patients with Marfan syndrome. We compared groups by pairwise analysis of means, using Tukey's method to adjust for multiple comparisons. Dural sac diameter association with phenotypic and clinical features was also tested. MEASUREMENTS AND MAIN RESULTS: The L1-L5 (average) anterior-posterior dural sac diameter of the idiopathic bronchiectasis group was larger than those of the control group (P < 0.001) and the cystic fibrosis group (P = 0.002). There was a strong correlation between increased dural sac size and the presence of pulmonary nontuberculous mycobacterial infection (P = 0.007) and long fingers (P = 0.003). A trend toward larger dural sac diameter was seen in those with scoliosis (P = 0.130) and those with a family history of idiopathic bronchiectasis (P = 0.149). CONCLUSIONS: Individuals with idiopathic bronchiectasis have an enlarged dural sac diameter, which is associated with pulmonary nontuberculous mycobacterial infection, long fingers, and family history of idiopathic bronchiectasis. These findings support our hypothesis that "idiopathic" bronchiectasis development reflects complex genetic variation in heritable connective tissue and associated transforming growth factor-β-related pathway genes.

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Published In

Ann Am Thorac Soc

DOI

EISSN

2325-6621

Publication Date

October 2016

Volume

13

Issue

10

Start / End Page

1712 / 1720

Location

United States

Related Subject Headings

  • Young Adult
  • Spirometry
  • Nontuberculous Mycobacteria
  • Mycobacterium Infections, Nontuberculous
  • Middle Aged
  • Marfan Syndrome
  • Male
  • Magnetic Resonance Imaging
  • Lumbosacral Region
  • Linear Models
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Daniels, M. L. A., Birchard, K. R., Lowe, J. R., Patrone, M. V., Noone, P. G., & Knowles, M. R. (2016). Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants. Ann Am Thorac Soc, 13(10), 1712–1720. https://doi.org/10.1513/AnnalsATS.201603-161OC
Daniels, M Leigh Anne, Katherine R. Birchard, Jared R. Lowe, Michael V. Patrone, Peadar G. Noone, and Michael R. Knowles. “Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants.Ann Am Thorac Soc 13, no. 10 (October 2016): 1712–20. https://doi.org/10.1513/AnnalsATS.201603-161OC.
Daniels MLA, Birchard KR, Lowe JR, Patrone MV, Noone PG, Knowles MR. Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants. Ann Am Thorac Soc. 2016 Oct;13(10):1712–20.
Daniels, M. Leigh Anne, et al. “Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants.Ann Am Thorac Soc, vol. 13, no. 10, Oct. 2016, pp. 1712–20. Pubmed, doi:10.1513/AnnalsATS.201603-161OC.
Daniels MLA, Birchard KR, Lowe JR, Patrone MV, Noone PG, Knowles MR. Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants. Ann Am Thorac Soc. 2016 Oct;13(10):1712–1720.

Published In

Ann Am Thorac Soc

DOI

EISSN

2325-6621

Publication Date

October 2016

Volume

13

Issue

10

Start / End Page

1712 / 1720

Location

United States

Related Subject Headings

  • Young Adult
  • Spirometry
  • Nontuberculous Mycobacteria
  • Mycobacterium Infections, Nontuberculous
  • Middle Aged
  • Marfan Syndrome
  • Male
  • Magnetic Resonance Imaging
  • Lumbosacral Region
  • Linear Models