Evidence map›Paper›PMID 38174586›Full record

ArticleThe Journal of clinical endocrinology and metabolism2024

Incidence and Prevalence of Fibrous Dysplasia/McCune-Albright Syndrome: A Nationwide Registry-Based Study in Denmark.

Maartje E Meier, Emese Vágó, Bo Abrahamsen, Olaf M Dekkers, Erzsébet Horváth-Puhó, Lars Rejnmark, Natasha M Appelman-Dijkstra

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In one paragraph

Article in The Journal of clinical endocrinology and metabolism, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
20citing papers in PubMed, 1 pooled it
–field-weighted citation impact
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

20 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Comparison of traditional and new treatments for fibrous dysplasia: a systematic review and meta-analysis.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2026
    Pooled it
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  4. Fibrous dysplasia: clinical and pathologic characteristics, surgical approaches, and outcomes in a large Italian cohort.Journal of orthopaedics and traumatology : official journal of the Italian Society of Orthopaedics and Traumatology · 2026
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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

7 authors.

Maartje E MeierDepartment of Orthopedic Surgery, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands.ORCID 0000-0002-6258-2531
Emese VágóDepartment of Clinical Epidemiology, Aarhus University, 8200 Aarhus N, Denmark.
Bo AbrahamsenOPEN Patient Data Explorative Network, University of Southern Denmark, 5000 Odense C, Denmark.
Olaf M DekkersDepartment of Clinical Epidemiology, Aarhus University, 8200 Aarhus N, Denmark.ORCID 0000-0002-1333-7580
Erzsébet Horváth-PuhóDepartment of Clinical Epidemiology, Aarhus University, 8200 Aarhus N, Denmark.
Lars RejnmarkDepartment of Endocrinology and Internal Medicine, Aarhus University Hospital, 8200 Aarhus N, Denmark.
Natasha M Appelman-DijkstraDepartment of Internal Medicine, Division of Endocrinology, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

contextFibrous dysplasia/McCune-Albright syndrome (FD/MAS) is a rare genetic disorder. Incidence and prevalence are not well-studied. Epidemiological research is complicated by the rarity of FD/MAS, absence of registries, heterogeneous presentation, and possibly asymptomatic phenotype. FD/MAS may present with FGF23-mediated hypophosphatemia, of which the epidemiology is also unclear.

objectiveEvaluate incidence and prevalence of FD/MAS and FD/MAS-related hypophosphatemia.

methodsThis cohort study based on the nationwide Danish National Patient Registry from 1995-2018, included patients identified by ICD-10 codes M85.0 (monostotic FD [MFD]) and Q78.1 (polyostotic FD [PFD]/MAS). Incidence rates and prevalence were calculated and stratified by sex, age, calendar period, and diagnosis code. Cases were screened for FD-associated hypophosphatemia by diagnosis code E.83 (disorder of mineral metabolism) and dispatched vitamin D analogues.

resultsA total of 408 patients were identified, 269 with MFD (66%), 139 with PFD/MAS (34%), comparable between sexes. Incidence of FD/MAS demonstrated increasing secular trend with a rate of 3.6 per 1 000 000 person-years (95% CI: 2.9, 4.5) in 2015-2018. Incidence peaked between age 11 and 20. Prevalence of FD/MAS increased over time to 61.0 (95% CI: 54.6, 67.4) per 1 000 000 persons in 2018. The incidence rate of MFD was 1.5-fold that of PFD/MAS in the first decade, rising to 2.5-fold in the last decade. No FD/MAS cases were registered with diagnosis code or treatment for hypophosphatemia.

conclusionFD/MAS is rare, diagnosis peaks during adolescence without sex predominance, and MFD is most prevalent. Hypophosphatemia may be underdiagnosed and undertreated, or it may be underregistered, comparing this study to literature.

Indexed as

Fibrous Dysplasia, PolyostoticRegistriesAdolescentAdultAgedChildChild, PreschoolCohort StudiesDenmarkFemaleFibroblast Growth Factor-23HumansHypophosphatemiaIncidenceInfantMaleFGF23 protein, humanFibroblast Growth Factor-23epidemiologyfibrous dysplasia/McCune-Albright syndromeincidenceprevalencerare bone disorderregistry

Identifiers

PMID38174586
PMCPMC11099484

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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.