Pubblicazioni

Autori:

Bertoldo, Francesco; Zappini, Francesca; Brigo, M.; Moggio, M.; Lucchini, V.; Angelini, C.; Semplicini, C.; Filosto, M.; Ravaglia, S.; Cotelli, S.; Todeschini, A.; Scarpelli, Mauro; Pancheri, Serena; Tonin, Paola

Titolo:

Prevalence of asymptomatic vertebral fractures in late-onset Pompe disease.

Anno:

2015

Tipologia prodotto:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Lingua:

Inglese

Formato:

A Stampa

Referee:

Sì

Nome rivista:

The Journal of Clinical Endocrinology and Metabolism

ISSN Rivista:

0021-972X

N° Volume:

100

Numero o Fascicolo:

2

Intervallo pagine:

401-406

Parole chiave:

Pompe disease; bone mass impairment; asymptomatic vertebral fractures; patients

Breve descrizione dei contenuti:

CONTEXT: Bone fragility and low bone mass have been reported in small case series of patients with Pompe disease with severely reduced muscle strength or immobilization.OBJECTIVE: Our objective was to determine the prevalence of morphometric vertebral fractures and to evaluate bone mass in adults with late-onset Pompe disease.DESIGN: We conducted a multicenter cross-sectional observational study from August 2012 to December 2013.STUDY SETTING: All subjects were outpatients referred to University Referral Centers.PATIENTS: patients included 22 late-onset Pompe disease patients with progressive proximal myopathy and minimal respiratory involvement without other diseases affecting bone mass.MAIN OUTCOME MEASURE: The prevalence of morphometric vertebral fractures was systematically assessed by semiquantitative analysis of lateral spine x-rays (T4-L5).RESULTS: A high prevalence of morphometric vertebral fractures was found. At least 1 vertebral fracture was present in 17 of 22 patients (77%). All vertebral fractures were asymptomatic. Bone mineral density was normal in 36.5% of the patients,whereas 36.5% were osteopenic and 27% were osteoporotic in at least 1 site. Fracture prevalence was independent of muscular and respiratory functional parameters and of genotype.CONCLUSIONS: Our data show for the first time that asymptomatic and atraumatic vertebral fractures occur frequently in late-onset Pompe disease patients without a significant impairment of bone mass. Screening for asymptomatic vertebral fractures should be routinely performed in Pompe disease irrespective of the disease severity. Fracture risk should be confirmed in longitudinal studies

Pagina Web:

http://dx.doi.org/10.1210/jc.2014-2763

Id prodotto:

85928

Handle IRIS:

11562/826766

depositato il:

11 febbraio 2015

ultima modifica:

15 novembre 2022

Citazione bibliografica:

Bertoldo, Francesco; Zappini, Francesca; Brigo, M.; Moggio, M.; Lucchini, V.; Angelini, C.; Semplicini, C.; Filosto, M.; Ravaglia, S.; Cotelli, S.; Todeschini, A.; Scarpelli, Mauro; Pancheri, Serena; Tonin, Paola, Prevalence of asymptomatic vertebral fractures in late-onset Pompe disease. «The Journal of Clinical Endocrinology and Metabolism» , vol. 100 , n. 2 , 2015 , pp. 401-406

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