Cited by 685Cystic Fibrosis Research AdvancesGenetic and Kidney Cyst DiseasesNeonatal Respiratory Health Research

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Cystic fibrosis

Open access

Shteinberg, Michal, Haq, Iram J, Polineni, Deepika, Davies, Jane C·2021·The Lancet

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Abstract

Cystic fibrosis is a monogenic disease considered to affect at least 100 000 people worldwide. Mutations in CFTR, the gene encoding the epithelial ion channel that normally transports chloride and bicarbonate, lead to impaired mucus hydration and clearance. Classical cystic fibrosis is thus characterised by chronic pulmonary infection and inflammation, pancreatic exocrine insufficiency, male infertility, and might include several comorbidities such as cystic fibrosis-related diabetes or cystic fibrosis liver disease. This autosomal recessive disease is diagnosed in many regions following newborn screening, whereas in other regions, diagnosis is based on a group of recognised multiorgan clinical manifestations, raised sweat chloride concentrations, or CFTR mutations. Disease that is less easily diagnosed, and in some cases affecting only one organ, can be seen in the context of gene variants leading to residual protein function. Management strategies, including augmenting mucociliary clearance and aggressively treating infections, have gradually improved life expectancy for people with cystic fibrosis. However, restoration of CFTR function via new small molecule modulator drugs is transforming the disease for many patients. Clinical trial pipelines are actively exploring many other approaches, which will be increasingly needed as survival improves and as the population of adults with cystic fibrosis increases. Here, we present the current understanding of CFTR mutations, protein function, and disease pathophysiology, consider strengths and limitations of current management strategies, and look to the future of multidisciplinary care for those with cystic fibrosis.

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Authors: Shteinberg, M., Haq, I. J., Polineni, D., & Davies, J. C.
Affiliations: Technion – Israel Institute of Technology (IL)
Newcastle upon Tyne Hospitals NHS Foundation Trust (GB)
University of Kansas Medical Center (US)
Guy's and St Thomas' NHS Foundation Trust (GB)
ORCID: Shteinberg, Michal
Haq, Iram J
Polineni, Deepika
Davies, Jane C
Venue: The Lancet
Publisher: Elsevier BV
Published: 2021-06
Volume: 397
Issue: 10290
Pages: 2195-2211
ISSN: 0099-5355 (ISSN-L)
0140-6736
DOI: 10.1016/s0140-6736(20)32542-3
OpenAlex: W4205612199
Cited by: 682
References: 195
Citations by year: 2026: 96 · 2025: 132 · 2024: 147 · 2023: 160 · 2022: 131 · 2021: 14
FWCI: 18.21
Type: journal article
Language: en
Open access: green
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
Locations: The Lancet (publishedVersion)
PubMed (publishedVersion)
Newcastle University ePrints (Newcastle Univesity)
Funders: National Institute for Health and Care Research (NIHR201402, CL-2018-01-002)
Cystic Fibrosis Trust (SRC 014)
Academy of Medical Sciences (SGL024\1027)
Topics: Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Neonatal Respiratory Health Research
Keywords: cystic fibrosis, CFTR mutations, CFTR function, mucus hydration, chronic lung infection, exocrine pancreatic insufficiency, male infertility, cystic fibrosis-related diabetes, cystic fibrosis liver disease, newborn screening, sweat test, CFTR modulator therapy, mucociliary clearance, disease pathophysiology, multidisciplinary care
MeSH terms: Bicarbonates, Chlorides, Cystic Fibrosis, Humans, Life Expectancy, Mucociliary Clearance, Mutation, Exocrine Pancreatic Insufficiency, Genetic Therapy, Cystic Fibrosis Transmembrane Conductance Regulator, Disease Management

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