ArticleBMC pulmonary medicine2018
Clinical expression of cystic fibrosis in a large cohort of Italian siblings.
Article in BMC pulmonary medicine, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 38 citations in OpenAlex.
- Toward Precision Electrochemical Sensing of CFTR Function in Cystic Fibrosis Models.Analytical chemistry · 2026Article
- Metabolomic profiling of saliva from cystic fibrosis patients.Scientific reports · 2025Article
- Non pathological sweat test, pancreatic insufficiency and Cystic Fibrosis: an unusual case in a child with F508del-duplication of exons 1-3 CFTR genotype.BMC pediatrics · 2024Article
- Liver biochemical indexes and cholesterol metabolism in cystic fibrosis patients with F508del/CFTR variant genotype after elexacaftor/tezacaftor/ivacaftor treatment.Scientific reports · 2024Article
- Cystic fibrosis and CFTR-related disorder with electrolyte imbalance at diagnosis: clinical features and outcome in an Italian cohort.European journal of pediatrics · 2023Article
- Quantitative Evaluation ofGenes · 2023Article
- Lipidomic alterations in human saliva from cystic fibrosis patients.Scientific reports · 2023Article
- One year of treatment with elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis homozygous for the F508del mutation causes a significant increase in liver biochemical indexes.Frontiers in molecular biosciences · 2023Article
- Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index.Italian journal of pediatrics · 2022Review
- Oxylipin profile in saliva from patients with cystic fibrosis reveals a balance between pro-resolving and pro-inflammatory molecules.Scientific reports · 2022Article
- Cystic fibrosis related liver disease and endocrine considerations.Journal of clinical & translational endocrinology · 2022Article
- Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis.Genes · 2021Review
- NGS Gene Panel Analysis Revealed Novel Mutations in Patients with Rare Congenital Diarrheal Disorders.Diagnostics (Basel, Switzerland) · 2021Article
- Lung Microbiome in Cystic Fibrosis.Life (Basel, Switzerland) · 2021Review
- Quantitative Evaluation of CFTR Pre-mRNA Splicing Dependent on the (TG)mTn Poly-Variant Tract.Diagnostics (Basel, Switzerland) · 2021Article
- ExtensiveJournal of clinical medicine · 2020Article
- Impaired Ratio of Unsaturated to Saturated Non-Esterified Fatty Acids in Saliva from Patients with Cystic Fibrosis.Diagnostics (Basel, Switzerland) · 2020Article
- Cystic Fibrosis-Screening Positive Inconclusive Diagnosis: Newborn Screening and Long-Term Follow-Up Permits to Early Identify Patients with CFTR-Related Disorders.Diagnostics (Basel, Switzerland) · 2020Article
- Clinical and Genotypical Features of False-Negative Patients in 26 Years of Cystic Fibrosis Neonatal Screening in Tuscany, Italy.Diagnostics (Basel, Switzerland) · 2020Article
- Salivary Cytokines and Airways Disease Severity in Patients with Cystic Fibrosis.Diagnostics (Basel, Switzerland) · 2020Article
Corrections and comments
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Authors and funding
23 authors at 15 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundA clinical heterogeneity was reported in patients with Cystic Fibrosis (CF) with the same CFTR genotype and between siblings with CF.
methodsWe investigated all clinical aspects in a cohort of 101 pairs of siblings with CF (including 6 triplets) followed since diagnosis.
resultsSevere lung disease had a 22.2% concordance in sib-pairs, occurred early and the FEV
conclusionsPhysicians involved in care of patients with CF and in genetic counseling must be aware of the clinical heterogeneity of CF even in sib-pairs that, at the state of the art, is difficult to explain.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.