Evidence map›Paper›PMID 28851540›Full record

ArticleBone2018

Mast cell inhibition as a therapeutic approach in fibrodysplasia ossificans progressiva (FOP).

Tracy A Brennan, Carter M Lindborg, Christian R Bergbauer, Haitao Wang, Frederick S Kaplan, Robert J Pignolo

Abstract read
In one paragraph

Article in Bone, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.

0numbers the graph read from it
0cells of the map it votes in
22citing papers in PubMed
–field-weighted citation impact
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

22 citing papers in PubMed.

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  11. Modeling theeLife · 2021
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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

6 authors.

Tracy A BrennanDepartment of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States. Electronic address: tabrenna@verizon.net.
Carter M LindborgDepartment of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States. Electronic address: cml274@georgetown.edu.
Christian R BergbauerDepartment of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States.
Haitao WangDepartment of Orthopaedic Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States; The Center for Research in FOP and Related Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States.
Frederick S KaplanDepartment of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States; Department of Orthopaedic Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States; The Center for Research in FOP and Related Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States. Electronic address: frederick.kaplan@uphs.upenn.edu.
Robert J PignoloDepartment of Medicine, Mayo Clinic School of Medicine, Mayo Clinic, Rochester, MN, United States. Electronic address: pignolo.robert@mayo.edu.

Funding

Overall: Resource-based Center for Musculoskeletal Disorders Research (Overall Application)P30AR069619 · NIAMS · UNIVERSITY OF PENNSYLVANIA · PI LOUIS J SOSLOWSKY · 2016 to 2026
$9.1M
The Role of Hyaluronic Acid Receptors in Tendon HealingP30AR050950 · NIAMS · UNIVERSITY OF PENNSYLVANIA · PI PIGNOLO, ROBERT JOHN · 2006 to 2015
$6.2M
The Cellular and Molecular Basis of FOP LesionsR01AR041916 · NIAMS · UNIVERSITY OF PENNSYLVANIA · PI KAPLAN, FREDERICK SAMUEL, SHORE, EILEEN M · 1994 to 2014
$3.8M
NIAMS NIH HHS P30 AR050950NIAMS NIH HHS P30 AR069619NIAMS NIH HHS R01 AR041916
6 · The paper itself

Abstract

backgroundEpisodic flare-ups of fibrodysplasia ossificans progressiva (FOP) are characterized clinically by severe, often posttraumatic, connective tissue swelling and intramuscular edema, followed histologically by an intense and highly angiogenic fibroproliferative reaction. This early inflammatory and angiogenic fibroproliferative response is accompanied by the presence of abundant mast cells far in excess of other reported myopathies.

resultsUsing an injury-induced, constitutively-active transgenic mouse model of FOP we show that mast cell inhibition by cromolyn, but not aprepitant, results in a dramatic reduction of heterotopic ossification. Cromolyn, but not aprepitant, significantly decreases the total number of mast cells in FOP lesions. Furthermore, cromolyn specifically diminishes the number of degranulating and resting degranulated mast cells in pre-osseous lesions.

conclusionsThis work demonstrates that consideration of FOP as a type of localized mastocytosis may offer new therapeutic interventions for treatment of this devastating condition.

Indexed as

AnimalsAprepitantCromolyn SodiumDisease Models, AnimalMast CellsMiceMorpholinesMyositis OssificansOssification, HeterotopicAprepitantCromolyn SodiumMorpholinesACVR1CromolynFibrodyplasia ossificans progressiva (FOP)Heterotopic ossificationMast cells

Identifiers

PMID28851540
PMCPMC7805128

What OpenQuestion holds

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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.