Evidence map›Paper›PMID 32183856›Full record

ArticleOrphanet journal of rare diseases2020

Abnormally increased carotid intima media-thickness and elasticity in patients with Morquio A disease.

Raymond Y Wang, Kyle D Rudser, Donald R Dengel, Nicholas Evanoff, Julia Steinberger, Nina Movsesyan, Robert Garrett, Katherine Christensen, Deborah Boylan, Stephen R Braddock and 3 more

Registry-linked trialOpen access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05063435 (Cardiovascular Structure and Function in the Mucopolysaccharidoses), which is not on this 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 34% 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.

NCT05063435 active not recruitingnot on this mapstarted 2021, after this paper: background citation

Cardiovascular Structure and Function in the Mucopolysaccharidoses

TypeobservationalSponsorChildren's Hospital of Orange CountyRan2021 to 2026Enrolled30ConditionsMucopolysaccharidoses, Carotid Disease, Cardiac Disease, InflammationArmsCarotid ultrasonography, Echocardiography, transthoracic, Venipuncture
3 · Its place in the literature

Who cites it

7 citing papers in PubMed, 6 citations in OpenAlex.

  1. Natural History of Morquio A Syndrome.Journal of inherited metabolic disease · 2026
    Review
  2. Article
  3. Article
  4. Editorial: Cardiac issues in adults with mucopolysaccharidosis.Frontiers in cardiovascular medicine · 2022
    Article
  5. Article
  6. 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

13 authors at 7 institutions in 2 countries.

Raymond Y WangDivision of Metabolic Disorders, CHOC Children's Specialists, Orange, CA, USA. rawang@choc.org.ORCID 0000-0001-6494-7613
Kyle D RudserDivision of Biostatistics, School of Public Health, University of Minnesota, Minneapolis, MN, USA.
Donald R DengelSchool of Kinesiology, College of Education and Human Development, University of Minnesota, Minneapolis, MN, USA.
Nicholas EvanoffSchool of Kinesiology, College of Education and Human Development, University of Minnesota, Minneapolis, MN, USA.
Julia SteinbergerDepartment of Pediatrics, University of Minnesota Medical School, Minneapolis, MN, USA.
Nina MovsesyanResearch Institute, CHOC Children's Hospital, Orange, CA, USA.
Robert GarrettDepartment of Radiology, School of Medicine, Saint Louis University, Saint Louis, MO, USA.
Katherine ChristensenDepartment of Pediatrics, Doisy Research Center, School of Medicine, Saint Louis University, St. Louis, MO, USA.
Deborah BoylanSSM Cardinal Glennon Children's Hospital, Saint Louis, MO, USA.
Stephen R BraddockDepartment of Pediatrics, Doisy Research Center, School of Medicine, Saint Louis University, St. Louis, MO, USA.
Marwan ShinawiDepartment of Pediatrics, Division of Genetics and Genomic Medicine, Washington University School of Medicine, Saint Louis, MO, USA.
Qi GanDepartment of Pediatrics, Doisy Research Center, School of Medicine, Saint Louis University, St. Louis, MO, USA.
Adriana M MontañoDepartment of Pediatrics, Doisy Research Center, School of Medicine, Saint Louis University, St. Louis, MO, USA. adriana.montano@health.slu.edu.ORCID 0000-0002-3971-5789
Saint Louis University · USUniversity of Minnesota · USChildren's Hospital of Orange County · USCardinal Glennon Children’s Medical Center · USSaint Louis University · ESUniversity of Minnesota Medical Center · USWashington University in St. Louis · US

Funding

University of Minnesota Clinical and Translational Science Institute (UMN CTSI)UL1TR002494 · NCATS · UNIVERSITY OF MINNESOTA · PI BLAZAR, BRUCE R, WEISDORF, DANIEL J · 2018 to 2022
$34.9M
Cardiovascular Risk and Insulin Resistance: Age 7-40R01DK072124 · NIDDK · UNIVERSITY OF MINNESOTA · PI STEINBERGER, JULIA · 2007 to 2011
$3.0M
Metabolic Syndrome in Childhood Cancer SurvivorsR01CA113930 · NCI · UNIVERSITY OF MINNESOTA · PI STEINBERGER, JULIA · 2006 to 2010
$2.5M
NCATS NIH HHS UL1 TR002494NCI NIH HHS R01 CA113930NIDDK NIH HHS R01 DK072124
6 · The paper itself

Abstract

backgroundCardiovascular disease frequently causes morbidity and mortality in mucopolysaccharidoses (MPS); however, cardiovascular anatomy and dysfunction in MPS IVA (Morquio A disease) is not well described. Consequently, the study aimed to compare carotid artery structure and elasticity of MPS IVA patients with other MPS patients and healthy control subjects, and quantitate frequency of MPS IVA cardiac structural and functional abnormalities.

methodsProspective, multi-center echocardiogram and carotid ultrasound evaluations of 12 Morquio A patients were compared with other MPS and healthy control subjects. Average differences between groups were adjusted for age, sex, and height with robust variance estimation for confidence intervals and P-values.

resultsMorquio A patients demonstrated significantly higher (P < 0.001) adjusted carotid intima-media thickness (cIMT), mean (SD) of 0.56 mm (0.03) compared to control subjects, 0.44 mm (0.04). The Morquio A cohort had significantly greater adjusted carotid elasticity (carotid cross-sectional compliance + 43%, P < 0.001; carotid incremental elastic modulus - 33%, P = 0.003) than control subjects and other MPS patients. Aortic root dilatation was noted in 56% of the Morquio A cohort, which also had highly prevalent mitral (73%) and aortic (82%) valve thickening, though hemodynamically significant valve dysfunction was less frequent (9%).

conclusionsIncreased carotid elasticity in Morquio A patients is an unexpected contrast to the reduced elasticity observed in other MPS. These Morquio A cIMT findings corroborate MPS IVA arterial post-mortem reports and are consistent with cIMT of other MPS. Aortic root dilatation in Morquio A indicates arterial elastin dysfunction, but their carotid hyperelasticity indicates other vascular intima/media components, such as proteoglycans, may also influence artery function. Studying MPS I and IVA model systems may uniquely illuminate the function of glycosaminoglycan-bearing proteoglycans in arterial health.

Indexed as

Carotid Intima-Media ThicknessMucopolysaccharidosis IVCross-Sectional StudiesElasticityHumansProspective StudiesCardiovascular diseaseCarotid ultrasoundIntima media thicknessMorquio A diseaseMucopolysaccharidosis IVA

Identifiers

PMID32183856
PMCPMC7079365
OpenAlexW3012186471

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Registered trials

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.