Evidence map›Paper›PMID 33741007›Full record

ArticleJournal of orthopaedic surgery and research2021

Hip pathologies in mucopolysaccharidosis type III.

Sandra Rafaela Breyer, Eik Vettorazzi, Leonie Schmitz, Amit Gulati, Katharina Maria von Cossel, Alexander Spiro, Martin Rupprecht, Ralf Stuecker, Nicole Maria Muschol

Open access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of orthopaedic surgery and research, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact, top 97% 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

1 citing paper in PubMed, 1 citations in OpenAlex.

  1. 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

9 authors at 1 institution in 1 country.

Sandra Rafaela BreyerDepartment of Pediatric Orthopedics, Children's Hospital Altona, Bleickenallee 38, 22763, Hamburg, Germany. sandra.r.breyer@gmail.com.ORCID http://orcid.org/0000-0002-1122-0266
Eik VettorazziDepartment of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Leonie SchmitzDepartment of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Amit GulatiDepartment of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Katharina Maria von CosselInternational Center for Lysosomal Disorders, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Alexander SpiroDepartment of Pediatric Orthopedics, Children's Hospital Altona, Bleickenallee 38, 22763, Hamburg, Germany.
Martin RupprechtDepartment of Pediatric Orthopedics, Children's Hospital Altona, Bleickenallee 38, 22763, Hamburg, Germany.
Ralf StueckerDepartment of Pediatric Orthopedics, Children's Hospital Altona, Bleickenallee 38, 22763, Hamburg, Germany.
Nicole Maria MuscholInternational Center for Lysosomal Disorders, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Universität Hamburg · DE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundMucopolysaccharidosis type III (MPS III) comprises a group of rare lysosomal storage diseases. Although musculoskeletal symptoms are less pronounced than in other MPS subtypes, pathologies of hip and spine have been reported in MPS III patients. The purpose of this study was to describe hip pathologies and influencing parameters in MPS III patients.

methodsA retrospective chart review was performed for 101 MPS III patients. Thirty-two patients met the inclusion criteria of enzymatically or genetically confirmed diagnosis and anteroposterior radiograph of the hips. Modified Ficat classification, Wiberg's center-edge angle, and Reimer's migration percentage were measured.

resultsThe mean age at data assessment was 11.0 years (SD 5.7). Osteonecrosis of the femoral head was observed in 17/32 patients. No statistically significant association was found between these changes and age, sex, or MPS III subtype. Patients with a severe phenotype showed significantly higher rates of osteonecrosis (14/17) than patients with an intermediate phenotype. Hip dysplasia was present in 9/32 patients and was significantly associated with osteonecrosis of the femoral head (p = 0.04).

conclusionsThe present study demonstrates a high rate of hip pathologies in MPS III patients. Hip dysplasia and severe phenotype were significantly correlated with osteonecrosis of the femoral head. Therefore, radiographs of the hips are highly recommended in baseline and follow-up assessments of MPS III patients.

trial registrationRetrospectively registered.

Indexed as

AdolescentAdultChildChild, PreschoolFemaleFemur Head NecrosisHipHip DislocationHumansMaleMucopolysaccharidosesRadiographyRetrospective StudiesYoung AdultDysostosis multiplexFemoral headHip dysplasiaMPSMucopolysaccharidosis type IIIOsteonecrosisPainSanfilippo syndromeSkeletal diseaseSkeletal dysplasia

Identifiers

PMID33741007
PMCPMC7977579
OpenAlexW3137283397

What OpenQuestion holds

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LicenceCC BY
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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.