ReviewCellular and molecular life sciences : CMLS2017
Cystic fibrosis: a clinical view.
Review in Cellular and molecular life sciences : CMLS, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05599958 (Clinical and Genetic Profile of Pediatric Patients With Cystic Fibrosis in Sohag.), which is not on this map. Cited by 135 papers, 3 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Clinical and Genetic Profile of Pediatric Patients With Cystic Fibrosis in Sohag.
Who cites it
135 citing papers in PubMed, 3 syntheses or guidelines pooled it, 251 citations in OpenAlex.
- CRISPR for cystic fibrosis: Advances and insights from a systematic review.Molecular therapy : the journal of the American Society of Gene Therapy · 2025Pooled it
- Effectiveness of Probiotics, Prebiotics, and Symbiotic Supplementation in Cystic Fibrosis Patients: A Systematic Review and Meta-Analysis of Clinical Trials.Medicina (Kaunas, Lithuania) · 2025Pooled it
- Pooled it
- Male reproductive phenotype in cystic fibrosis: comparison of existing animal models.Lab animal · 2026Review
- Solid Lipid Nanoparticles Based Dry Powder Inhalers for Enhanced Drug Delivery Against Lung Diseases.AAPS PharmSciTech · 2026Review
- SOLAR: a noninterventional study of outcomes over the long term of people with cystic fibrosis (CF) aged ≥6 years treated with elexacaftor/tezacaftor/ivacaftor using data from the French CF Registry.ERJ open research · 2026Article
- Investigating the Association Between Cystic Fibrosis and Colorectal Neoplasia: A Matched Case-Control Study.JGH open : an open access journal of gastroenterology and hepatology · 2026Article
- Telehealth by Home Monitoring and Video Consultation for Children With Cystic Fibrosis: Qualitative and Quantitative Study.JMIR formative research · 2026Article
- Human iPSC-derived macrophages for studying intrinsic and extrinsic factors in cystic fibrosis.EXO : beyond the cell · 2026Article
- Cystic fibrosis transmembrane conductance regulator modulator therapy and lived experiences in South Africa: A mixed-methods study.The South African journal of physiotherapy · 2026Article
- Beyond the matrix: rethinking antibiotic tolerance in CF biofilms using 3D models.NPJ biofilms and microbiomes · 2025Article
- Human induced pluripotent stem cells for in vitro modeling of impaired mucociliary clearance in cystic fibrosis lung disease.Stem cell research & therapy · 2025Article
- Cyclodextrin-Based Nanotransporters as a Versatile Tool to Manage Oxidative Stress-Induced Lung Diseases.Antioxidants (Basel, Switzerland) · 2025Review
- Article
- Impact of Modulator Therapy on the Chronic Colonization of Lower Respiratory Tract Pathogens in Children: Data From Cystic Fibrosis Registry of Turkey.Pediatric pulmonology · 2025Article
- Anti-infectives in Pediatric Patients with Cystic Fibrosis: A Comprehensive Review of Population Pharmacokinetic Analyses.Clinical pharmacokinetics · 2025Review
- Intact spermatogenesis in an azoospermic patient with AZFa (sY84 and sY86) microdeletion and a homozygous TG12-5T variant in CFTR.Basic and clinical andrology · 2025Article
- Article
- miRNA-target gene network analysis in siblings with cystic fibrosis and phenotypic variability.Turkish journal of medical sciences · 2025Article
- IL-6 trans-signaling in cystic fibrosis bronchial cells potentiates TNF-α-driven ICAM-1 expression.Frontiers in cell and developmental biology · 2025Article
75 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude. Classical treatment includes pancreatic enzyme replacement, respiratory physiotherapy, mucolitics, and aggressive antibiotic therapy. A significant proportion of patients with severe symptoms still requires lung or, less frequently, liver transplantation. The great number of mutations and their diverse effects on the CFTR protein account only partially for CF clinical variability, and modifier genes have a role in modulating the clinical expression of the disease. Despite the increasing understanding of CFTR functioning, several aspects of CF need still to be clarified, e.g., the worse outcome in females, the risk of malignancies, the pathophysiology, and best treatment of comorbidities, such as CF-related diabetes or CF-related bone disorder. Research is focusing on new drugs restoring CFTR function, some already available and with good clinical impact, others showing promising preliminary results that need to be confirmed in phase III clinical trials.
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Registered trials
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.