Evidence map›Paper›PMID 26116954›Full record

ArticleMolecular genetics and metabolism2015

Non-invasive pulmonary function test on Morquio patients.

Francyne Kubaski, Shunji Tomatsu, Pravin Patel, Tsutomu Shimada, Li Xie, Eriko Yasuda, Robert Mason, William G Mackenzie, Mary Theroux, Michael B Bober and 3 more

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

Article in Molecular genetics and metabolism, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04112602 (Respiratory Impairment in Mucopolysaccharidosis Patients), which is not on this map. Cited by 9 papers.

0numbers the graph read from it
0cells of the map it votes in
9citing papers in PubMed
1.1field-weighted citation impact, top 23% 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.

NCT04112602 completednot on this mapstarted 2019, after this paper: background citation

Respiratory Impairment in Mucopolysaccharidosis Patients: Impact of Glycosaminoglycans (GAG) on the Proteases/Antiproteases Balance

TypeobservationalSponsorUniversity Hospital, ToursRan2019 to 2020Enrolled27ConditionsMucopolysaccharidosesArmsSputum, Tracheal aspiration
3 · Its place in the literature

Who cites it

9 citing papers in PubMed, 15 citations in OpenAlex.

  1. Natural History of Morquio A Syndrome.Journal of inherited metabolic disease · 2026
    Review
  2. Review
  3. Article
  4. Article
  5. Review
  6. Article
  7. Review
  8. Review
  9. Non-invasive pulmonary function test on Morquio patients.Journal of rare diseases research & treatment · 2017
    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 3 institutions in 2 countries.

Francyne KubaskiNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Department of Biological Sciences, University of Delaware, Newark, DE, USA.
Shunji TomatsuNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Department of Pediatrics, Gifu University, Gifu, Japan. Electronic address: stomatsu@nemours.org.
Pravin PatelNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Tsutomu ShimadaNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Li XieNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Eriko YasudaNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Robert MasonNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Department of Biological Sciences, University of Delaware, Newark, DE, USA.
William G MackenzieNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Mary TherouxNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Michael B BoberNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
Helen M OldhamDepartment of Respiratory Care, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA.
Tadao OriiDepartment of Pediatrics, Gifu University, Gifu, Japan.
Thomas H ShafferNemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA; Center for Pediatric Lung Research, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA. Electronic address: tshaffer@nemours.org.
Alfred I. duPont Hospital for Children · USGifu University · JPUniversity of Delaware · US

Funding

Center for Pediatric ResearchP20GM103464 · NIGMS · NEMOURS CHILDREN'S HOSPITAL, DELAWARE · PI SHAFFER, THOMAS H · 2012 to 2014
$5.6M
NIGMS NIH HHS P20 GM103464NIGMS NIH HHS P20GM103464
6 · The paper itself

Abstract

In clinical practice, respiratory function tests are difficult to perform in Morquio syndrome patients due to their characteristic skeletal dysplasia, small body size and lack of cooperation of young patients, where in some cases, conventional spirometry for pulmonary function is too challenging. To establish feasible clinical pulmonary endpoints and determine whether age impacts lung function in Morquio patients non-invasive pulmonary tests and conventional spirometry were evaluated. The non-invasive pulmonary tests: impulse oscillometry system, pneumotachography, and respiratory inductance plethysmography in conjunction with conventional spirometry were evaluated in twenty-two Morquio patients (18 Morquio A and 4 Morquio B) (7 males), ranging from 3 to 40 years of age. Twenty-two patients were compliant with non-invasive tests (100%) with the exception of IOS (81.8%-18 patients). Seventeen patients (77.3%) were compliant with spirometry testing. All subjects had normal vital signs at rest including >95% oxygen saturation, end tidal CO2 (38-44 mmHg), and age-appropriate heart rate (mean=98.3, standard deviation=19) (two patients were deviated). All patients preserved normal values in the impulse oscillometry system, pneumotachography, and respiratory inductance plethysmography, although predicted forced expiratory total (72.8±6.9 SE%) decreased with age and was below normal; phase angle (35.5±16.5°), %rib cage (41.6±12.7%), resonant frequency, and forced expiratory volume in 1 s/forced expiratory volume total (110.0±3.2 SE%) were normal and not significantly impacted by age. The proposed non-invasive pulmonary function tests are able to cover a greater number of patients (young patients and/or wheel-chair bound), thus providing a new diagnostic approach for the assessment of lung function in Morquio syndrome which in many cases may be difficult to evaluate. Morquio patients studied herein demonstrated no clinical or functional signs of restrictive and/or obstructive lung disease.

Indexed as

AdolescentAdultAgingChildChild, PreschoolFemaleHumansLungMaleMucopolysaccharidosis IVRespiratory Function TestsSpirometryVital CapacityYoung AdultImpulse oscillometry systemMorquio syndromeNon-invasive pulmonary function testPneumotachographyRespiratory inductance plethysmography

Identifiers

PMID26116954
PMCPMC4706533
OpenAlexW851407559

What OpenQuestion holds

Textmetadata
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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.