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| Item Type: | Article |
|---|---|
| Title: | A longitudinal multi-omic dataset of pediatric cystic fibrosis patients receiving lumacaftor/ivacaftor therapy: clinical, microbiome, inflammatory and metabolomic measurements collected over 24 months |
| Creators: |
Knoll, Rebecca Luise  ORCID: https://orcid.org/0000-0002-3496-345X, Rossow, Virginia, Rössler, Juliane, Hilbert, Katja, Jarquín-Díaz, Víctor Hugo ORCID: https://orcid.org/0000-0003-3758-1091, Bartolomaeus, Theda Ulrike Patricia ORCID: https://orcid.org/0000-0002-5156-7184, Nitsche, Oliver ORCID: https://orcid.org/0000-0001-8795-6504, Essex, Morgan ORCID: https://orcid.org/0000-0001-8758-7497, Löber, Ulrike ORCID: https://orcid.org/0000-0001-7468-9531, Meng, Chen, Kleigrewe, Karin ORCID: https://orcid.org/0000-0002-7718-0252, Gehring, Stephan, Forslund-Startceva, Sofia Kirke ORCID: https://orcid.org/0000-0003-4285-6993 and Poplawska, Krystyna
|
| Abstract: | This article describes a longitudinal multiomic dataset gen- erated within a prospective phase IV pilot study of eight children with cystic fibrosis homozygous for the F508del mutation who initiated lumacaftor/ivacaftor therapy. Partic- ipants were followed for up to 24 months with repeated col- lection of clinical metadata, anthropometric measurements, sweat chloride concentrations, lung function assessments, in- flammatory markers, conventional microbiology results, stool samples, respiratory samples, and serum metabolomics. The resulting dataset links host phenotypes, microbiome compo- sition, inflammatory parameters, and metabolomic measure- ments across multiple body sites and time points. Micro- biome data were generated from stool, sputum and throat swab samples using 16S rRNA gene sequencing, while serum metabolomics was assessed using untargeted mass spectrom- etry. The dataset is publicly available through SRA, MassIVE and GitHub repositories and may support future studies of longitudinal host–microbiome interactions, biomarker discov- ery, methodological benchmarking and comparative analyses across CFTR modulator eras. |
| Keywords: | Pediatric Cystic Fibrosis, Lumacaftor/Ivacaftor, Deep Phenotyping, Microbiome, Metabolome |
| Source: | Data in Brief |
| ISSN: | 2352-3409 |
| Publisher: | Elsevier |
| Volume: | 69 |
| Page Range: | 113237 |
| Number of Pages: | 1 |
| Date: | August 2026 |
| Official Publication: | https://doi.org/10.1016/j.dib.2026.113237 |
| PubMed: | View item in PubMed |
| Related to: |
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