Registry of Multiple Osteochondromas

NCT ID: NCT04133285

Last Updated: 2025-11-20

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Total Enrollment

10000 participants

Study Classification

OBSERVATIONAL

Study Start Date

2013-06-28

Study Completion Date

2032-12-31

Brief Summary

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REM is a retrospective and prospective registry, finalized for care and research purposes. It is articulated in main sections - strongly related and mutually dependent on each other - corresponding to different data domains: personal information, clinical data, genetic data, genealogical data, surgeries, etc.

This approach has been developed to corroborate and integrate data from different sources evaluating several aspects of diseases and to correlate genetic background and phenotypic outcomes, in order to better investigate disease pathophysiology. Due to legal requirements, institutional directives and organizational issues, we are unable to include individuals residing outside Italy in the registry at this time. We are currently engaged in the preparation of a recruitment process for individuals residing outside Italy.

Detailed Description

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The traditional method of collecting patient information is often chaotic, inconvenient, and sometimes even unsafe, particularly when dealing with rare diseases. In 2013, the need to simplify the diagnostic process and to overcome the difficulties of data storage and analysis, led to the suggestion of implementing the Registry of Multiple Osteochondromas (REM).

The REM relies on an IT platform named Genotype-phenotype Data Integration platform - GeDI. This solution was developed through a collaboration between Rare Skeletal Disease Department and a local software company (Dilaxia S.p.A.) and is General Data Protection Regulation (GDPR)-compliant, multi-client and web-accessible. It has been designed according to current medical informatics standards, including the Orphanet code, the International Classification of Diseases (ICD), and the Human Genome Variants Society, aiming to follow Findability, Accessibility, Interoperability, Reusability (FAIR) principles. GeDI is continuously being implemented to improve the management of people with Multiple Osteochondromas and to assist researchers in analyzing the information collected. REM is divided into the following main sections:

* Personal data: it comprises general information, birth details and residence data;
* Patient data: including the patients internal code, the hospital code and other patient details;
* Diagnostic Process: the diagnosis, the status (affected, suspected, etc.), age at diagnosis, comorbidities, allergies, etc.;
* Genogram: a tool for designing the family transmission of the disease, alongside information on the disease status of all relatives included;
* Clinical events: it records a long list of signs and symptoms of Multiple Osteochondromas as well as several additional items to describe the disease
* Genetic Analysis and Alteration: including analytical technique, sample information, analysis duration, etc. This section also comprises detailed information on any detected pathological variants (e.g. gene, international reference, DNA change, protein change, genomic position, etc.);
* Visits: this section includes visit type (genetic, orthopedic, rehabilitation, pediatric, etc.), the date of the visit, prescriptions, imaging, etc.;
* Treatments: this section comprises information of a wide range of treatments including pharmacological, devices, supplements, and other treatments such as psychological, nutritional, etc;
* Surgeries: this section contains information on the type of surgeries, the age of the patients, the site/localization of the procedures, etc.
* Documents: this repository allow us to store all types of documents (radiological reports, imaging, consents, clinical reports, etc.);
* Consents: this section provides a comprehensive overview of all consents collected, including the collection date;
* Samples: this section includes information on the samples, like the type, date of collection, etc.
* PROs: this section collects information on patients reported outcomes such as the quality of life or ABC scale.

Conditions

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Multiple Osteochondromas

Study Design

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Observational Model Type

COHORT

Study Time Perspective

OTHER

Study Groups

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Multiple Osteochondromas patients

Patients affected by Multiple Osteochondromas. The Registry will include also data on fetuses (prenatal).

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* All Multiple Osteochondromas patients, including prenatal diagnosis of Multiple Osteochondromas

Exclusion Criteria

* Any condition unrelated to Multiple Osteochondromas
Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Luca Sangiorgi

OTHER

Sponsor Role lead

Responsible Party

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Luca Sangiorgi

Head of Department of Rare Skeletal Disorders

Responsibility Role SPONSOR_INVESTIGATOR

Principal Investigators

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Luca Sangiorgi, MD, PhD, MS

Role: PRINCIPAL_INVESTIGATOR

Istituto Ortopedico Rizzoli

Locations

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Irccs Istituto Ortopedico Rizzoli

Bologna, Emilia-Romagna, Italy

Site Status RECRUITING

Countries

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Italy

Central Contacts

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Marina Mordenti, PhD

Role: CONTACT

+39 05 6366062

Marcella Lanza, MSc

Role: CONTACT

+39 05 6366169

Facility Contacts

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Marina Mordenti, PhD

Role: primary

+39 051 6366062

Marcella Lanza, PhD

Role: backup

+39 051 6366169

References

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Mordenti M, Ferrari E, Pedrini E, Fabbri N, Campanacci L, Muselli M, Sangiorgi L. Validation of a new multiple osteochondromas classification through Switching Neural Networks. Am J Med Genet A. 2013 Mar;161A(3):556-60. doi: 10.1002/ajmg.a.35819. Epub 2013 Feb 8.

Reference Type BACKGROUND
PMID: 23401177 (View on PubMed)

Pedrini E, Jennes I, Tremosini M, Milanesi A, Mordenti M, Parra A, Sgariglia F, Zuntini M, Campanacci L, Fabbri N, Pignotti E, Wuyts W, Sangiorgi L. Genotype-phenotype correlation study in 529 patients with multiple hereditary exostoses: identification of "protective" and "risk" factors. J Bone Joint Surg Am. 2011 Dec 21;93(24):2294-302. doi: 10.2106/JBJS.J.00949.

Reference Type BACKGROUND
PMID: 22258776 (View on PubMed)

Schmale GA, Conrad EU 3rd, Raskind WH. The natural history of hereditary multiple exostoses. J Bone Joint Surg Am. 1994 Jul;76(7):986-92. doi: 10.2106/00004623-199407000-00005.

Reference Type BACKGROUND
PMID: 8027127 (View on PubMed)

Pacifici M. Hereditary Multiple Exostoses: New Insights into Pathogenesis, Clinical Complications, and Potential Treatments. Curr Osteoporos Rep. 2017 Jun;15(3):142-152. doi: 10.1007/s11914-017-0355-2.

Reference Type BACKGROUND
PMID: 28466453 (View on PubMed)

Vink GR, White SJ, Gabelic S, Hogendoorn PC, Breuning MH, Bakker E. Mutation screening of EXT1 and EXT2 by direct sequence analysis and MLPA in patients with multiple osteochondromas: splice site mutations and exonic deletions account for more than half of the mutations. Eur J Hum Genet. 2005 Apr;13(4):470-4. doi: 10.1038/sj.ejhg.5201343.

Reference Type BACKGROUND
PMID: 15586175 (View on PubMed)

White SJ, Vink GR, Kriek M, Wuyts W, Schouten J, Bakker B, Breuning MH, den Dunnen JT. Two-color multiplex ligation-dependent probe amplification: detecting genomic rearrangements in hereditary multiple exostoses. Hum Mutat. 2004 Jul;24(1):86-92. doi: 10.1002/humu.20054.

Reference Type BACKGROUND
PMID: 15221792 (View on PubMed)

Wuyts W, Van Hul W. Molecular basis of multiple exostoses: mutations in the EXT1 and EXT2 genes. Hum Mutat. 2000;15(3):220-7. doi: 10.1002/(SICI)1098-1004(200003)15:33.0.CO;2-K.

Reference Type BACKGROUND
PMID: 10679937 (View on PubMed)

Darilek S, Wicklund C, Novy D, Scott A, Gambello M, Johnston D, Hecht J. Hereditary multiple exostosis and pain. J Pediatr Orthop. 2005 May-Jun;25(3):369-76. doi: 10.1097/01.bpo.0000150813.18673.ad.

Reference Type BACKGROUND
PMID: 15832158 (View on PubMed)

Cacciari E, Milani S, Balsamo A, Spada E, Bona G, Cavallo L, Cerutti F, Gargantini L, Greggio N, Tonini G, Cicognani A. Italian cross-sectional growth charts for height, weight and BMI (2 to 20 yr). J Endocrinol Invest. 2006 Jul-Aug;29(7):581-93. doi: 10.1007/BF03344156.

Reference Type BACKGROUND
PMID: 16957405 (View on PubMed)

Cacciari E, Milani S, Balsamo A, Dammacco F, De Luca F, Chiarelli F, Pasquino AM, Tonini G, Vanelli M. Italian cross-sectional growth charts for height, weight and BMI (6-20 y). Eur J Clin Nutr. 2002 Feb;56(2):171-80. doi: 10.1038/sj.ejcn.1601314.

Reference Type BACKGROUND
PMID: 11857051 (View on PubMed)

Porter DE, Lonie L, Fraser M, Dobson-Stone C, Porter JR, Monaco AP, Simpson AH. Severity of disease and risk of malignant change in hereditary multiple exostoses. A genotype-phenotype study. J Bone Joint Surg Br. 2004 Sep;86(7):1041-6. doi: 10.1302/0301-620x.86b7.14815.

Reference Type BACKGROUND
PMID: 15446535 (View on PubMed)

Goud AL, de Lange J, Scholtes VA, Bulstra SK, Ham SJ. Pain, physical and social functioning, and quality of life in individuals with multiple hereditary exostoses in The Netherlands: a national cohort study. J Bone Joint Surg Am. 2012 Jun 6;94(11):1013-20. doi: 10.2106/JBJS.K.00406.

Reference Type BACKGROUND
PMID: 22637207 (View on PubMed)

D'Ambrosi R, Ragone V, Caldarini C, Serra N, Usuelli FG, Facchini RM. The impact of hereditary multiple exostoses on quality of life, satisfaction, global health status, and pain. Arch Orthop Trauma Surg. 2017 Feb;137(2):209-215. doi: 10.1007/s00402-016-2608-4. Epub 2016 Dec 8.

Reference Type BACKGROUND
PMID: 27933382 (View on PubMed)

Related Links

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https://www.ior.it/en/curarsi-al-rizzoli/registries-rare-hereditary-diseases

Institutional webpage of Registries For Rare Hereditary Diseases

Other Identifiers

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21629/2013

Identifier Type: -

Identifier Source: org_study_id

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