ArticleOrphanet journal of rare diseases2012
Evidence for decline in the incidence of cystic fibrosis: a 35-year observational study in Brittany, France.
Article in Orphanet journal of rare diseases, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis 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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 59 citations in OpenAlex.
- Systematic review: cystic fibrosis in the SARS-CoV-2/COVID-19 pandemic.BMC pulmonary medicine · 2021Pooled it
- Article
- Contemporary cystic fibrosis incidence rates in Canada and the United States.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023Article
- Cystic fibrosis prevalence in the United States and participation in the Cystic Fibrosis Foundation Patient Registry in 2020.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023Article
- Modeling of Respiratory Diseases Evolving with Fibrosis from Organoids Derived from Human Pluripotent Stem Cells.International journal of molecular sciences · 2023Review
- The Prognosis of ART Is Not Altered in Cystic Fibrosis Women: A Case-Report Study.Frontiers in endocrinology · 2022Article
- Choline in cystic fibrosis: relations to pancreas insufficiency, enterohepatic cycle, PEMT and intestinal microbiota.European journal of nutrition · 2021Review
- Cystic Fibrosis.Pediatrics in review · 2021Review
- Comprehensive Analysis of Chemical Structures That Have Been Tested as CFTR Activating Substances in a Publicly Available Database CandActCFTR.Frontiers in pharmacology · 2021Article
- [Results of the neonatal screening on Western Andalusia after a decade of experience.]Revista espanola de salud publica · 2020Article
- Review
- Newborn Screening for CF across the Globe-International journal of neonatal screening · 2020Review
- Evaluating sequence data quality from the Swift Accel-Amplicon CFTR Panel.Scientific data · 2020Article
- The future of cystic fibrosis care: a global perspective.The Lancet. Respiratory medicine · 2020Review
- Aberrant MicroRNAomics in Pulmonary Complications: Implications in Lung Health and Diseases.Molecular therapy. Nucleic acids · 2019Review
- Human Cytomegalovirus Compromises Development of Cerebral Organoids.Journal of virology · 2019Article
- Current Practices and Potential Nanotechnology Perspectives for Pain Related to Cystic Fibrosis.Journal of clinical medicine · 2019Review
- Cystic Fibrosis and Pseudomonas aeruginosa: the Host-Microbe Interface.Clinical microbiology reviews · 2019Review
- Article
- Mixed Communities of Mucoid and NonmucoidmBio · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundCystic fibrosis (CF) is an autosomal recessive disorder whose incidence has long been estimated as 1/2500 live births in Caucasians. Expanding implementation of newborn screening (NBS) programs now allows a better monitoring of the disease incidence, what is essential to make reliable predictions for disease management. This study assessed time trends in the birth incidence of CF over a long period (35 years: 1975-2009) in an area where CF is frequent (Brittany, France) and where NBS has been implemented for more than 20 years.
methodsThis study enrolled CF patients born in Brittany between January 1st 1975 and December 31st 2009 (n = 483). Time trends in incidence were examined using Poisson regression and mainly expressed using the average percent change (APC).
resultsThe average number of patients born each year declined from 18.6 in the late 1970's (period 1975-79) to 11.6 nowadays (period 2005-09). The corresponding incidence rates dropped from 1/1983 to 1/3268, which represented a decline close to 40% between these two periods (APC = -39.3%, 95% CI = -55.8% to -16.7%, p = 0.0020). A clear breakpoint in incidence rate was observed at the end of the 1980's (p < 0.0001). However, the incidence rate has remained quite stable since that time (annual APC = -1.0%, 95% CI = -3.0% to 1.1%, p = 0.3516).
conclusionsThis study provides an accurate picture of the evolution of the incidence of a genetic disease over a long period and highlights how it is influenced by the health policies implemented. We observed a 40% drop in incidence in our area which seems consecutive to the availability of prenatal diagnosis.
Indexed as
Identifiers
What OpenQuestion holds
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.