Sleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction
<strong>Background</strong> Cystic fibrosis (CF) transmembrane regulator (CFTR) protein dysfunction causes CF. Improving survival allows detection of increasingly subtle disease manifestations. CFTR dysfunction in the central nervous system (CNS) may disturb circadian rhythm and thus sl...
| Những tác giả chính: | , , , , , |
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| Định dạng: | Journal article |
| Ngôn ngữ: | English |
| Được phát hành: |
Elsevier
2017
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| _version_ | 1826282991864774656 |
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| author | Jensen, J Jones, C Kartsonaki, C Packer, K Adler, F Liou, T |
| author_facet | Jensen, J Jones, C Kartsonaki, C Packer, K Adler, F Liou, T |
| author_sort | Jensen, J |
| collection | OXFORD |
| description | <strong>Background</strong> Cystic fibrosis (CF) transmembrane regulator (CFTR) protein dysfunction causes CF. Improving survival allows detection of increasingly subtle disease manifestations. CFTR dysfunction in the central nervous system (CNS) may disturb circadian rhythm and thus sleep phase. We studied sleep in adults to better understand potential CNS CFTR dysfunction. <strong>Methods</strong> We recruited participants from April 2012 through April 2015 and administered the Munich Chronotype Questionnaire (MCTQ). We compared free-day sleep measurements between CF and non-CF participants and investigated associations with CF survival predictors. <strong>Results</strong> We recruited 23 female and 22 male adults with CF aged 18 to 46 years and 26 female and 22 male volunteers aged 18 to 45 years. Compared with volunteers without CF, patients with CF had delayed sleep onset (0.612 h; P = .015), midsleep (1.11 h; P < .001), and wake (1.15 h; P < .001) times and prolonged sleep latency (7.21 min; P = .05) and duration (0.489 h; P = .05). Every hour delay in sleep onset was associated with shorter sleep duration by 0.29 h in patients with CF and 0.75 h in subjects without CF (P = .007) and longer sleep latency by 7.51 min in patients with CF and 1.6 min in volunteers without CF (P = .035). Among patients with CF, FEV1 % predicted, prior acute pulmonary exacerbations, and weight were independent of all free-day sleep measurements. <strong>Conclusions</strong> CF in adults is associated with marked delays in sleep phase consistent with circadian rhythm phase delays. Independence from disease characteristics predictive of survival suggests that sleep phase delay is a primary manifestation of CFTR dysfunction in the CNS. |
| first_indexed | 2024-03-07T00:52:11Z |
| format | Journal article |
| id | oxford-uuid:86c5abab-b1c9-4f17-a576-e6abe5db2a29 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T00:52:11Z |
| publishDate | 2017 |
| publisher | Elsevier |
| record_format | dspace |
| spelling | oxford-uuid:86c5abab-b1c9-4f17-a576-e6abe5db2a292022-03-26T22:06:17ZSleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunctionJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:86c5abab-b1c9-4f17-a576-e6abe5db2a29EnglishSymplectic Elements at OxfordElsevier2017Jensen, JJones, CKartsonaki, CPacker, KAdler, FLiou, T <strong>Background</strong> Cystic fibrosis (CF) transmembrane regulator (CFTR) protein dysfunction causes CF. Improving survival allows detection of increasingly subtle disease manifestations. CFTR dysfunction in the central nervous system (CNS) may disturb circadian rhythm and thus sleep phase. We studied sleep in adults to better understand potential CNS CFTR dysfunction. <strong>Methods</strong> We recruited participants from April 2012 through April 2015 and administered the Munich Chronotype Questionnaire (MCTQ). We compared free-day sleep measurements between CF and non-CF participants and investigated associations with CF survival predictors. <strong>Results</strong> We recruited 23 female and 22 male adults with CF aged 18 to 46 years and 26 female and 22 male volunteers aged 18 to 45 years. Compared with volunteers without CF, patients with CF had delayed sleep onset (0.612 h; P = .015), midsleep (1.11 h; P < .001), and wake (1.15 h; P < .001) times and prolonged sleep latency (7.21 min; P = .05) and duration (0.489 h; P = .05). Every hour delay in sleep onset was associated with shorter sleep duration by 0.29 h in patients with CF and 0.75 h in subjects without CF (P = .007) and longer sleep latency by 7.51 min in patients with CF and 1.6 min in volunteers without CF (P = .035). Among patients with CF, FEV1 % predicted, prior acute pulmonary exacerbations, and weight were independent of all free-day sleep measurements. <strong>Conclusions</strong> CF in adults is associated with marked delays in sleep phase consistent with circadian rhythm phase delays. Independence from disease characteristics predictive of survival suggests that sleep phase delay is a primary manifestation of CFTR dysfunction in the CNS. |
| spellingShingle | Jensen, J Jones, C Kartsonaki, C Packer, K Adler, F Liou, T Sleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction |
| title | Sleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction |
| title_full | Sleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction |
| title_fullStr | Sleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction |
| title_full_unstemmed | Sleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction |
| title_short | Sleep phase delay in cystic fibrosis: a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction |
| title_sort | sleep phase delay in cystic fibrosis a potential new manifestation of cystic fibrosis transmembrane regulator dysfunction |
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