Evidence map›Paper›PMID 25342923›Full record

ReviewJournal of blood medicine2014

Optimal management of hereditary hemorrhagic telangiectasia.

Neetika Garg, Monica Khunger, Arjun Gupta, Nilay Kumar

Open access · goldAbstract readReview
In one paragraph

Review in Journal of blood medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 48 papers.

0numbers the graph read from it
0cells of the map it votes in
48citing papers in PubMed
4.1field-weighted citation impact, top 5% 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

48 citing papers in PubMed, 108 citations in OpenAlex.

  1. Article
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  12. AThe Journal of international medical research · 2023
    Article
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  15. A challenging case of anemia, respiratory failure and seizures.Acta bio-medica : Atenei Parmensis · 2022
    Article
  16. Article
  17. Cardiac and Hemodynamic Manifestations of Hereditary Hemorrhagic Telangiectasia.The International journal of angiology : official publication of the International College of Angiology, Inc · 2022
    Review
  18. Review
  19. Childhood stroke.Nature reviews. Disease primers · 2022
    Review
  20. 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

4 authors at 4 institutions in 2 countries.

Neetika GargDepartment of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA.
Monica KhungerDepartment of Medicine, All India Institute of Medical Sciences, New Delhi, India.
Arjun GuptaDepartment of Medicine, UT Southwestern Medical Center, Dallas, TX, USA.
Nilay KumarDepartment of Medicine, Cambridge Health Alliance/Harvard Medical School, Cambridge, MA, USA.
All India Institute of Medical Sciences · INBeth Israel Deaconess Medical Center · USHarvard University · USSouthwestern Medical Center · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hereditary hemorrhagic telangiectasia (HHT), also known by the eponym Osler-Weber-Rendu syndrome, is a group of related disorders inherited in an autosomal dominant fashion and characterized by the development of arteriovenous malformations (AVM) in the skin, mucous membranes, and/or internal organs such as brain, lungs, and liver. Its prevalence is currently estimated at one in 5,000 to 8,000. Most cases are due to mutations in the endoglin (HHT1) or ACVRLK1 (HHT2) genes. Telangiectasias in nasal and gastrointestinal mucosa generally present with recurrent/chronic bleeding and iron deficiency anemia. Larger AVMs occur in lungs (~40%-60% of affected individuals), liver (~40%-70%), brain (~10%), and spine (~1%). Due to the devastating and potentially fatal complications of some of these lesions (for example, strokes and brain abscesses with pulmonary AVMs), presymptomatic screening and treatment are of utmost importance. However, due to the rarity of this condition, many providers lack an appreciation for the whole gamut of its manifestations and complications, age-dependent penetrance, and marked intrafamilial variation. As a result, HHT remains frequently underdiagnosed and many families do not receive the appropriate screening and treatments. This article provides an overview of the clinical features of HHT, discusses the clinical and genetic diagnostic strategies, and presents an up-to-date review of literature and detailed considerations regarding screening for visceral AVMs, preventive modalities, and treatment options.

Indexed as

arteriovenous malformationsbevacizumabembolizationepistaxisguidelinesreviewscreening

Identifiers

PMID25342923
PMCPMC4206399
OpenAlexW1993385143

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
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.