Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review

International Journal of Chronic Obstructive Pulmonary Disease
Ross G EdgarAlice M Turner

Abstract

Alpha-1 antitrypsin deficiency (AATD) is a rare genetic condition predisposing individuals to chronic obstructive pulmonary disease (COPD). The treatment is generally extrapolated from COPD unrelated to AATD; however, most COPD trials exclude AATD patients; thus, this study sought to systematically review AATD-specific literature to assist evidence-based patient management. Standard review methodology was used with meta-analysis and narrative synthesis (PROSPERO-CRD42015019354). Eligible studies were those of any treatment used in severe AATD. Randomized controlled trials (RCTs) were the primary focus; however, case series and uncontrolled studies were eligible. All studies had ≥10 participants receiving treatment or usual care, with baseline and follow-up data (>3 months). Risk of bias was assessed appropriately according to study methodology. In all, 7,296 studies were retrieved from searches; 52 trials with 5,632 participants met the inclusion criteria, of which 26 studies involved alpha-1 antitrypsin augmentation and 17 concerned surgical treatments (largely transplantation). Studies were grouped into four management themes: COPD medical, COPD surgical, AATD specific, and other treatments. Computed tomography (CT) density, ...Continue Reading

Citations

Feb 16, 2018·Alimentary Pharmacology & Therapeutics·S A TownsendA M Turner
Oct 27, 2017·European Respiratory Review : an Official Journal of the European Respiratory Society·Haitham S Al Ashry, Charlie Strange
Jun 11, 2019·Drugs & Aging·Diana CrossleyElizabeth Sapey
Feb 8, 2020·Respirology : Official Journal of the Asian Pacific Society of Respirology·Jack DummerAnne E Holland
Sep 26, 2019·Expert Opinion on Investigational Drugs·Anita Pye, Alice M Turner
Feb 14, 2020·European Respiratory Review : an Official Journal of the European Respiratory Society·Mario CazzolaMaria Gabriella Matera
Mar 14, 2019·ERJ Open Research·Ildikó HorváthN Gerard McElvaney
Nov 23, 2019·The European Respiratory Journal·Miriam Barrecheguren, Marc Miravitlles
Oct 22, 2020·American Journal of Clinical Pathology·Josiah D MurrayMelissa R Snyder
Dec 4, 2020·Thorax·Mark QuinnRavi Mahadeva
Dec 15, 2020·International Journal of Chronic Obstructive Pulmonary Disease·Kylie B R BelchamberElizabeth Sapey
Dec 29, 2020·Therapeutics and Clinical Risk Management·Mark QuinnAlice M Turner
Nov 14, 2020·Experimental Biology and Medicine·Uday K Baliga, David A Dean
Mar 20, 2018·Free Radical Biology & Medicine·David Z SilbersteinKaren A McDonald
May 21, 2018·Experimental Eye Research·Gustavo OrtizJuan E Gallo
Aug 20, 2021·Therapeutic Advances in Chronic Disease·Igor Barjaktarevic, Michael Campos

❮ Previous
Next ❯

Clinical Trials Mentioned

NCT01217671
NCT00242385
NCT01213043
NCT01983241

Software Mentioned

Ovid
ZETOC
EXACTLE

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.