TY - JOUR
T1 - Serum Interleukin-6 Levels and Pulmonary Function in Ataxia-Telangiectasia
AU - McGrath-Morrow, Sharon A.
AU - Collaco, Joseph M.
AU - Detrick, Barbara
AU - Lederman, Howard M.
N1 - Publisher Copyright:
© 2016 Elsevier Inc.
PY - 2016/4/1
Y1 - 2016/4/1
N2 - Objective To evaluate the potential link between systemic inflammation and impaired lung function in people with ataxia-telangiectasia (A-T), we hypothesized that serum levels of interleukin (IL)-6, a proinflammatory cytokine, would correlate inversely with lung function in subjects with A-T. Study design Consecutive subjects with A-T were recruited from the Johns Hopkins Outpatient A-T Clinical Center. Serum levels of IL-6 and 8 were measured by enzyme-linked immunosorbent assay. Spirometry was performed in subjects ≥6 years of age on the same day that serum was obtained for measurements of cytokines. Results Approximately 80% of subjects had elevated serum IL-6 levels (>1.0 pg/mL). No association was found between elevated IL-6 and age. Elevated IL-8 levels were found in 23.6% of subjects, and all subjects with elevated IL-8 levels had elevated IL-6 levels. Subjects with elevated IL-6 levels (mean: 6.14 ± 7.47 pg/mL) had significantly lower mean percent forced vital capacity (FVC%, 50.5% ± 17.8%) compared with subjects with normal serum IL-6 levels (FVC% of 66.2 ± 16.1, P =.018). Greater IL-6 levels were associated with lower FVC% even after adjustment for receiving gamma globulin therapy (P =.024) and supplemental nutrition (P =.055). Conclusions An association was found between elevated serum IL-6 levels and lower lung function in subjects with A-T. In addition, subjects with both elevated IL-6 and IL-8 had the lowest mean lung function. These findings indicate that markers for systemic inflammation may be useful in identifying individuals with A-T at increased risk for lower lung function and may help in assessing response to therapy.
AB - Objective To evaluate the potential link between systemic inflammation and impaired lung function in people with ataxia-telangiectasia (A-T), we hypothesized that serum levels of interleukin (IL)-6, a proinflammatory cytokine, would correlate inversely with lung function in subjects with A-T. Study design Consecutive subjects with A-T were recruited from the Johns Hopkins Outpatient A-T Clinical Center. Serum levels of IL-6 and 8 were measured by enzyme-linked immunosorbent assay. Spirometry was performed in subjects ≥6 years of age on the same day that serum was obtained for measurements of cytokines. Results Approximately 80% of subjects had elevated serum IL-6 levels (>1.0 pg/mL). No association was found between elevated IL-6 and age. Elevated IL-8 levels were found in 23.6% of subjects, and all subjects with elevated IL-8 levels had elevated IL-6 levels. Subjects with elevated IL-6 levels (mean: 6.14 ± 7.47 pg/mL) had significantly lower mean percent forced vital capacity (FVC%, 50.5% ± 17.8%) compared with subjects with normal serum IL-6 levels (FVC% of 66.2 ± 16.1, P =.018). Greater IL-6 levels were associated with lower FVC% even after adjustment for receiving gamma globulin therapy (P =.024) and supplemental nutrition (P =.055). Conclusions An association was found between elevated serum IL-6 levels and lower lung function in subjects with A-T. In addition, subjects with both elevated IL-6 and IL-8 had the lowest mean lung function. These findings indicate that markers for systemic inflammation may be useful in identifying individuals with A-T at increased risk for lower lung function and may help in assessing response to therapy.
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U2 - 10.1016/j.jpeds.2016.01.002
DO - 10.1016/j.jpeds.2016.01.002
M3 - Article
C2 - 26851119
AN - SCOPUS:84956690017
SN - 0022-3476
VL - 171
SP - 256-261.e1
JO - Journal of Pediatrics
JF - Journal of Pediatrics
ER -