Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry

International Journal of Environmental Research and Public Health
Anna ZolinRita Padoan

Abstract

Data collected in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database were used to investigate whether risk factors for death in childhood and adolescents CF patients have different impact in countries of different income. In this way, it is possible to recognize where interventions could improve the quality of care and survival in these patients. We matched deceased and alive patients by age, country, year of follow-up. Multivariable logistic models were developed. In the years of this study, the ECFSPR collected information on 24,416 patients younger than 18 years: 7830 patients were from countries with low/middle income and 16,586 from countries with high income; among these the dead are 102 and 107 (p < 0.001), respectively. The use of oxygen, forced expiratory volume in one second (FEV₁) below 40% and BMI standard deviation score (SDS) below -2 represent risk factors for death. However, some patients from countries with high income remain alive even if their values of FEV₁% and BMI-SDS were low, and some deceased patients from countries with high income had high values of FEV₁% (>60%). Evaluation of mortality in pediatric age may reflect the availability of resources for CF diagnosis and treatment in so...Continue Reading

References

Feb 1, 1993·Pediatric Pulmonology·X WangB G Ferris
May 15, 1996·American Journal of Epidemiology·M Corey, V Farewell
Jan 5, 1999·American Journal of Respiratory and Critical Care Medicine·J L HankinsonK B Fedan
Jul 16, 2002·American Journal of Epidemiology·Hui-Chuan LaiPhilip M Farrell
Jul 21, 2009·The Journal of Pediatrics·Stephen M StickUNKNOWN Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)
Mar 23, 2010·Lancet·Jonathan McCormickUNKNOWN European Registry Working Group
Jul 8, 2010·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Isabelle Sermet-GaudelusUNKNOWN European Cystic Finrosis Society (ECFS), Neonatal Screening Working Group
Nov 3, 2010·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Gita MehtaUNKNOWN European Registry Working Group
Jan 5, 2011·Pediatric Pulmonology·Jessica E PittmanMichael R Knowles
Apr 20, 2013·The European Respiratory Journal·Eitan KeremUNKNOWN ECFS Patient Registry Steering Group
Dec 18, 2013·Pediatric Pulmonology·Neil B Sweezey, Felix Ratjen
Jun 9, 2014·Orphanet Journal of Rare Diseases·Laura VivianiHanne Vebert Olesen
Aug 26, 2014·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Gianfranco AlicandroCarla Colombo
Aug 20, 2015·Annals of the American Thoracic Society·Don B SandersUNKNOWN EPIC Study Group
Apr 14, 2016·Clinical Nutrition : Official Journal of the European Society of Parenteral and Enteral Nutrition·Dominique TurckMichael Wilschanski
Jun 9, 2016·Expert Review of Respiratory Medicine·Gabriela R Oates, Michael S Schechter
Jan 4, 2017·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Jürg BarbenKevin W Southern
Apr 25, 2017·Pediatric Pulmonology·Susanna A McColleyMichael W Konstan
Apr 26, 2018·ERJ Open Research·Gloria TridelloBaroukh M Assael

❮ Previous
Next ❯

Citations

Feb 6, 2020·Journal of Palliative Medicine·Elisabeth P DellonDio Kavalieratos
Jan 10, 2019·International Journal of Environmental Research and Public Health·Ana Villaverde-HuesoVerónica Alonso-Ferreira
Oct 24, 2020·International Journal of Environmental Research and Public Health·Marta RachelSabina Galiniak
Aug 4, 2020·Pediatrics International : Official Journal of the Japan Pediatric Society·Ayse Ayzit KilincHaluk Cokugras
Dec 31, 2021·Pediatric Pulmonology·Marco ZampoliBrenda M Morrow

❮ Previous
Next ❯

Methods Mentioned

BETA
bronchoalveolar lavage

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

The European Respiratory Journal
Eitan KeremECFS Patient Registry Steering Group
Canadian Oncology Nursing Journal = Revue Canadienne De Nursing Oncologique
Savitri Singh-Carlson
The European Respiratory Journal
K De Boeckmembers of the Belgian CF Registry
© 2022 Meta ULC. All rights reserved