Evidence map›Paper›PMID 34807420›Full record

ReviewAdvances in experimental medicine and biology2021

Pathophysiology and Pathogenesis of Marfan Syndrome.

Sanford M Zeigler, Brandon Sloan, Jeffrey A Jones

Open access · greenAbstract readReview
In one paragraph

Review in Advances in experimental medicine and biology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 59 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
59citing papers in PubMed, 2 pooled it
76.5field-weighted citation impact, top 1% 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

59 citing papers in PubMed, 2 syntheses or guidelines pooled it, 105 citations in OpenAlex.

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  16. Surgical Intervention in Pediatric Marfan Syndrome: A Multiinstitutional Study.World journal for pediatric & congenital heart surgery · 2025
    Article
  17. Review
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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

3 authors at 2 institutions in 1 country.

Sanford M ZeiglerDivision of Cardiothoracic Surgery, Department of Surgery, Medical University of South Carolina, Charleston, SC, USA.
Brandon SloanDivision of Cardiothoracic Surgery, Department of Surgery, Medical University of South Carolina, Charleston, SC, USA.
Jeffrey A JonesDivision of Cardiothoracic Surgery, Medical University of South Carolina and Ralph H. Johnson Veterans Affairs Medical Center, Charleston, SC, USA. jonesja@musc.edu.
Medical University of South Carolina · USRalph H. Johnson VA Medical Center · US

Funding

BLRD VA I01 BX000904
6 · The paper itself

Abstract

Marfan syndrome (MFS) is a systemic connective tissue disorder that is inherited in an autosomal dominant pattern with variable penetrance. While clinically this disease manifests in many different ways, the most life-threatening manifestations are related to cardiovascular complications including mitral valve prolapse, aortic insufficiency, dilatation of the aortic root, and aortic dissection. In the past 30 years, research efforts have not only identified the genetic locus responsible but have begun to elucidate the molecular pathogenesis underlying this disorder, allowing for the development of seemingly rational therapeutic strategies for treating affected individuals. In spite of these advancements, the cardiovascular complications still remain as the most life-threatening clinical manifestations. The present chapter will focus on the pathophysiology and clinical treatment of Marfan syndrome, providing an updated overview of the recent advancements in molecular genetics research and clinical trials, with an emphasis on how this information can focus future efforts toward finding betters ways to detect, diagnose, and treat this devastating condition.

Indexed as

Aortic DissectionMarfan SyndromeAortaFibrillin-1HumansTransforming Growth Factor betaFibrillin-1Transforming Growth Factor betaAneurysmAngiotensin receptor blockers (ARBs)AortaCollagenEndoglinExtracellular matrixExtracellular signal related kinase (ERK)Fbn1C1039G/+FibrillinGenetic testingGhent nosologyLoeys-Dietz syndrome (LDS)LosartanMarfan syndrome (MFS)MetalloproteinasemgR/mgRMitogen-activated protein kinase (MAPK)Mitral valveSMADTGF-β receptorThoracic aortic aneurysm and dissection syndromeTransforming growth factor-β (TGF-β)β-Blockers

Identifiers

PMID34807420
PMCPMC8915437
OpenAlexW3216611866

What OpenQuestion holds

Textmetadata
LicenceTDM
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.