Evidence map›Paper›PMID 29225784›Full record

ReviewF1000Research2017

Diagnosing alpha-1 antitrypsin deficiency: the first step in precision medicine.

Craig P Hersh

Open access · goldAbstract readReview
In one paragraph

Review in F1000Research, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
0.6field-weighted citation impact, top 29% of its field
1 · What the graph read from 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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed, 12 citations in OpenAlex.

  1. Review
  2. Article
  3. [Research progress of hepatocyte transplantation treatment for alpha-1 antitrypsin deficiency].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2020
    Article
  4. Alpha-1 Antitrypsin Deficiency: a Rare Disease?Current allergy and asthma reports · 2020
    Review
  5. Pharmacogenomics of chronic obstructive pulmonary disease.Expert review of respiratory medicine · 2019
    Review
  6. Alpha-1 Antitrypsin Deficiency as an Incidental Finding in Clinical Genetic Testing.American journal of respiratory and critical care medicine · 2019
    Article
  7. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

1 author at 1 institution in 1 country.

Craig P HershChanning Division of Network Medicine, Brigham and Women's Hospital, 181 Longwood Ave., Boston, MA, 02115, USA.
Harvard University · US

Funding

Systems Biology, Bioinformatics, and BiostatisticsP01HL105339 · NHLBI · BRIGHAM AND WOMEN'S HOSPITAL · PI SILVERMAN, EDWIN K · 2011 to 2015
$12.2M
SYSTEMS GENOMICS OF THE ASTHMA-COPD OVERLAP SYNDROMER01HL130512 · NHLBI · BRIGHAM AND WOMEN'S HOSPITAL · PI HERSH, CRAIG P · 2016 to 2019
$3.5M
INTEGRATIVE GENOMICS OF CLINICAL SUBTYPES IN COPDGENER01HL125583 · NHLBI · BRIGHAM AND WOMEN'S HOSPITAL · PI HERSH, CRAIG P · 2015 to 2018
$3.5M
NHLBI NIH HHS P01 HL105339NHLBI NIH HHS R01 HL125583NHLBI NIH HHS R01 HL130512
6 · The paper itself

Abstract

Severe alpha-1 antitrypsin (AAT) deficiency is one of the most common serious genetic diseases in adults of European descent. Individuals with AAT deficiency have a greatly increased risk for emphysema and liver disease. Other manifestations include bronchiectasis, necrotizing panniculitis and granulomatosis with polyangiitis. Despite the frequency and potential severity, AAT deficiency remains under-recognized, and there is often a delay in diagnosis. This review will focus on three recent updates that should serve to encourage testing and diagnosis of AAT deficiency: first, the publication of a randomized clinical trial demonstrating the efficacy of intravenous augmentation therapy in slowing the progression of emphysema in AAT deficiency; second, the mounting evidence showing an increased risk of lung disease in heterozygous PI MZ genotype carriers; last, the recent publication of a clinical practice guideline, outlining diagnosis and management. Though it has been recognized for more than fifty years, AAT deficiency exemplifies the modern paradigm of precision medicine, with a diagnostic test that identifies a genetic subtype of a heterogeneous disease, leading to a targeted treatment.

Indexed as

alpha-1 antitrypsin deficiencyCOPDdiagnosistesting

Identifiers

PMID29225784
PMCPMC5710307
OpenAlexW2770646197

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

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.