Evidence map›Paper›PMID 41921026›Full record

ArticleEndocrine connections2026

Clinical features of Italian adult individuals with X-linked hypophosphatemia: a multicenter retrospective study.

Silvia Carrara, Giampiero I Baroncelli, Gaetano Paride Arcidiacono, Marco Barale, Maria Luisa Brandi, Valentina Camozzi, Elena Castellano, Filomena Cetani, Pasquale Comberiati, Simone Della Valentina and 22 more

Abstract read
In one paragraph

Article in Endocrine connections, 2026. 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
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. FGF23 - A hormone produced by bone and has many faces.Reviews in endocrine & metabolic disorders · 2026
    Review
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

32 authors.

Silvia CarraraDepartment of Medical Biotechnology and Translational Medicine, University of Milan, Milan, Italy.ORCID https://orcid.org/0009-0001-4477-4935
Giampiero I BaroncelliPediatric and Adolescent Endocrinology, Division of Pediatrics, Department of Obstetrics, Gynecology and Pediatrics, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.ORCID https://orcid.org/0000-0001-8465-5087
Gaetano Paride ArcidiaconoClinica Medica 1, Department of Medicine, University of Padua, Padua, Italy.ORCID https://orcid.org/0000-0003-0409-4449
Marco BaraleDivision of Oncological Endocrinology, Department of Medical Sciences, University of Turin, Turin, Italy.ORCID https://orcid.org/0000-0003-3441-2014
Maria Luisa BrandiDonatello Bone Clinic, Villa Donatello Hospital, Florence, Italy.ORCID https://orcid.org/0000-0002-8741-0592
Valentina CamozziEndocrine Unit, University-Hospital of Padua, Padua, Italy.ORCID https://orcid.org/0000-0002-1634-951X
Elena CastellanoDepartment of Endocrinology, Diabetes and Metabolism, Santa Croce and Carle Hospital, Cuneo, Italy.ORCID https://orcid.org/0000-0001-6622-4267
Filomena CetaniUnit of Endocrinology, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.ORCID https://orcid.org/0000-0003-2558-9547
Pasquale ComberiatiPediatric and Adolescent Endocrinology, Division of Pediatrics, Department of Obstetrics, Gynecology and Pediatrics, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.ORCID https://orcid.org/0000-0001-5209-9733
Simone Della ValentinaDepartment of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Cristina Eller-VainicherSC Endocrinologia, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.ORCID https://orcid.org/0000-0002-6805-5804
Nadia Edvige FolignoNephrology and Dialysis Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.ORCID https://orcid.org/0000-0001-9536-9254
Sandro GianniniClinica Medica 1, Department of Medicine, University of Padua, Padua, Italy.ORCID https://orcid.org/0000-0003-0796-9749
Laura GianottiStruttura Complessa di Endocrinologia e Diabetologia Territoriale, ASL Cuneo 1, Cuneo, Italy.ORCID https://orcid.org/0000-0002-4857-2468
Giorgia GrassiSC Endocrinologia, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.ORCID https://orcid.org/0000-0002-7236-0713
Martina LaganàDipartimento di Patologia Umana dell'Adulto e dell'Età Evolutiva "Gaetano Barresi" - Unità di Endocrinologia, University of Messina, Messina, Italy.
Silvia LaiDepartment of Translation and Precision Medicine, Sapienza University of Rome, Rome, Italy.ORCID https://orcid.org/0000-0002-7199-2954
Gemma MarcucciBone Metabolic Diseases Unit - Department of Biomedical, Experimental and Clinical Sciences, University Hospital of Florence, Florence, Italy.ORCID https://orcid.org/0000-0003-0579-0542
Laura MasiMetabolic Bone Diseases Unit, University Hospital of Florence AOUC, Florence, Italy.ORCID https://orcid.org/0000-0002-6467-4165
Francesca PagliaEndocrinology Unit, Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy.ORCID https://orcid.org/0009-0000-5842-1324
Andrea PalermoUnit of Metabolic Bone and Thyroid Disorders, Fondazione Policlinico Campus Bio-Medico, Rome, Italy.ORCID https://orcid.org/0000-0002-1143-4926
Adolfo Marco PerrottaDepartment of Translation and Precision Medicine, Sapienza University of Rome, Rome, Italy.ORCID https://orcid.org/0000-0002-0423-4256
Francesca PigliaruEndocrinology Unit, Azienda Ospedaliera-Universitaria di Cagliari, Cagliari, Italy.ORCID https://orcid.org/0000-0002-3899-0391
Massimo ProcopioDivision of Endocrinology, Diabetes and Metabolism, Department of Medical Sciences, University of Turin, Turin, Italy.ORCID https://orcid.org/0009-0000-0751-3798
Vincenzo RochiraEndocrinology Unit, Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy.ORCID https://orcid.org/0000-0001-8169-0696
Silverio RotondiDepartment of Translation and Precision Medicine, Sapienza University of Rome, Rome, Italy.ORCID https://orcid.org/0000-0003-4553-1756
Rosaria Maddalena RuggeriDipartimento di Patologia Umana dell'Adulto e dell'Età Evolutiva "Gaetano Barresi" - Unità di Endocrinologia, University of Messina, Messina, Italy.ORCID https://orcid.org/0000-0001-8899-684X
Marco Onofrio TorresClinica Medica 1, Department of Medicine, University of Padua, Padua, Italy.ORCID https://orcid.org/0009-0004-1426-1452
Silvia VaiBone Metabolism Diseases and Diabetes Unit, IRCCS Istituto Auxologico Italiano, Milan, Italy.
Giuseppe VezzoliNephrology and Dialysis Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.ORCID https://orcid.org/0000-0003-4481-5693
Marta ZampognaDepartment of Medical Biotechnology and Translational Medicine, University of Milan, Milan, Italy.
Sabrina CorbettaBone Metabolism Diseases and Diabetes Unit, IRCCS Istituto Auxologico Italiano, Milan, Italy.ORCID https://orcid.org/0000-0001-8140-3175

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Objective: X-linked hypophosphatemia (XLH) is the most common congenital phosphate disorder affecting individuals throughout the lifespan. We investigated the skeletal burden, the cardiovascular involvement, the diagnostic performance and the therapeutic management in a cohort of Italian adults with XLH. Design: Cross-sectional study involving 15 Italian tertiary centers. Methods: Retrospective study. Results: In total, 170 adults (110 females and 60 males), aged 44.6 ± 14.6 (19-83) years, were identified. i) Skeletal deformities were detected in 87.1% of individuals, fractures/pseudofractures in 44.7%, osteophytosis in 65.4% and enthesopathies in 57.6%. Dental disease affected 72.4% of individuals. The skeletal burden was heavier in males than in females. ii) Hypertension occurred in 14.7% of individuals and was associated with elevated plasma intact FGF23 levels; dyslipidemia, diabetes and cerebrovascular events occurred in very few individuals. iii) FGF23 levels were measured in 30.0% of individuals; they were >30 pg/mL (nv 23-95) in nearly all individuals but overtly elevated in 58.8%. Genetic analysis has been performed in 86.5% of the cohort, and PHEX mutations were identified in 95.2% of the individuals without evidence of genotype/phenotype correlation. iv) 44.2% of individuals were on conventional therapy, 32.5% were on burosumab, and 23.3% were untreated. Individuals having received diagnosis in the adulthood (n = 14) were neither medically nor surgically treated during their childhood. Conclusion: The burden of XLH disease in adulthood is determined by skeletal manifestations and dental disease and may be more severe in males. Additionally, cardiometabolic impairment may not be common. The disease burden impacts most of the individuals, beyond those presenting the criteria for burosumab reimbursement. Significance statement: Data from a consistent cohort of adults with XLH highlighted that skeletal and dental disease-related complications significantly affect XLH individuals during adulthood and aging. Skeletal features associated with aging occur earlier in adults with XLH, being more evident when untreated or poorly treated with conventional therapy. The disease burden impacts most of individuals, beyond those presenting the criteria for burosumab reimbursement. The study contributes in increasing awareness toward adult XLH individuals and provides data for implementing the disease management and the health policy planning.

Indexed as

adultsFGF23hypophosphatemiaskeletal deformitiesX-linked osteomalacia

Identifiers

PMID41921026
PMCPMC13130878

What Socratic holds

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LicenceCC BY-NC
Read underepoch 390

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 Socratic graph.