Evidence map›Paper›PMID 34758173›Full record

ArticleExperimental dermatology2022

Oral supplementation of inorganic pyrophosphate in pseudoxanthoma elasticum.

Eszter Kozák, Krisztina Fülöp, Natália Tőkési, Nidhi Rao, Qiaoli Li, Sharon F Terry, Jouni Uitto, Xiaoming Zhang, Cyrus Becker, András Váradi and 1 more

Open access · greenAbstract read
In one paragraph

Article in Experimental dermatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.

0numbers the graph read from it
0cells of the map it votes in
19citing papers in PubMed
2.9field-weighted citation impact, top 8% 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

19 citing papers in PubMed, 28 citations in OpenAlex.

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  16. Weighing the Evidence for the Roles of Plasma Versus Local Pyrophosphate in Ectopic Calcification Disorders.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2023
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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

11 authors at 4 institutions in 2 countries.

Eszter KozákInstitute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences Centre of Excellence, Budapest, Hungary.ORCID 0000-0001-9283-3752
Krisztina FülöpInstitute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences Centre of Excellence, Budapest, Hungary.ORCID 0000-0002-7453-6014
Natália TőkésiInstitute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences Centre of Excellence, Budapest, Hungary.ORCID 0000-0002-7620-5104
Nidhi RaoInstitute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences Centre of Excellence, Budapest, Hungary.ORCID 0000-0002-8159-7819
Qiaoli LiDepartment of Dermatology and Cutaneous Biology, PXE International Center of Excellence in Research and Clinical Care, Sidney Kimmel Medicine College, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.ORCID 0000-0002-8495-7103
Sharon F TerryPXE International, Washington, District of Columbia, USA.ORCID 0000-0002-0452-9329
Jouni UittoDepartment of Dermatology and Cutaneous Biology, PXE International Center of Excellence in Research and Clinical Care, Sidney Kimmel Medicine College, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.ORCID 0000-0003-4639-807X
Xiaoming ZhangTheratrophix, Sunnyvale, California, USA.
Cyrus BeckerTheratrophix, Sunnyvale, California, USA.ORCID 0000-0001-7868-464X
András VáradiInstitute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences Centre of Excellence, Budapest, Hungary.
Viola PomoziInstitute of Enzymology, Research Centre for Natural Sciences, Hungarian Academy of Sciences Centre of Excellence, Budapest, Hungary.ORCID 0000-0002-5435-4993
Institute of Molecular Life Sciences · HUMaxygen (United States) · USThomas Jefferson University · USPeterson Institute for International Economics · US

Funding

Novel Treatments for PXER01AR072695 · NIAMS · THOMAS JEFFERSON UNIVERSITY · PI LI, QIAOLI, VAN DE WETERING, KOEN · 2018 to 2022
$1.7M
NIAMS NIH HHS R01 AR072695
6 · The paper itself

Abstract

Pseudoxanthoma elasticum (PXE; OMIM 264800) is a rare heritable multisystem disorder, characterized by ectopic mineralization affecting elastic fibres in the skin, eyes and the cardiovascular system. Skin findings often lead to early diagnosis of PXE, but currently, no specific treatment exists to counteract the progression of symptoms. PXE belongs to a group of Mendelian calcification disorders linked to pyrophosphate metabolism, which also includes generalized arterial calcification of infancy (GACI) and arterial calcification due to CD73 deficiency (ACDC). Inactivating mutations in ABCC6, ENPP1 and NT5E are the genetic cause of these diseases, respectively, and all of them result in reduced inorganic pyrophosphate (PP

Indexed as

Pseudoxanthoma ElasticumVascular CalcificationAnimalsDietary SupplementsDiphosphatesHumansMiceMutationPhosphoric Diester HydrolasesPyrophosphatasesDiphosphatesdiphosphoric acidPhosphoric Diester HydrolasesPyrophosphatasesectopic mineralizationinvestigational therapypathological calcificationpseudoxanthoma elasticumpyrophosphate

Identifiers

PMID34758173
PMCPMC8977233
OpenAlexW3213743700

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.