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Orphanet journal of rare diseases · 2019

Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium.

СтатьяПоддержка NIH: внешняя программаПоддержка исследования: вне правительства СШАОбзорConsensus StatementНазвание и аннотация: Перевод в очереди
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Javaid Muhammad Kassim, Boyce Alison, Appelman-Dijkstra Natasha, Ong Juling, Defabianis Patrizia, Offiah Amaka, Arundel Paul, Shaw Nick, Pos Valter Dal, Underhil Ann, Portero Deanna, Heral Lisa, Heegaard Anne-Marie, Masi Laura, Monsell Fergal, Stanton Robert, Dijkstra Pieter Durk Sander, Brandi Maria Luisa, Chapurlat Roland, Hamdy Neveen Agnes Therese, Collins Michael Terrence

Аннотация

Fibrous Dysplasia / McCune Albright syndrome (FD/MAS) represents a wide spectrum of diseases due to somatic gain-of-function mutations of the GNAS gene. The mutation leads to overactivity in the target tissues and to a wide phenotype of clinical features that vary in severity and age of onset. The rarity of the disease and its variable presentation to multiple specialities often leads to misdiagnosis and inappropriate variability in investigations and treatments. To address this, our international consortium of clinicians, researchers, and patients' advocates has developed pragmatic clinical guidelines for best clinical practice for the definition, diagnosis, staging, treatment and monitoring for FD/MAS to empower patients and support clinical teams in both general and specialised healthcare settings. With the lack of strong evidence to inform care, the guidelines were developed based on review of published literature, long-standing extensive experience of authors, input from other healthcare professionals involved in the care of FD/MAS patients and feedback from patients and patient groups across the globe. This has led to the formulation of a set of statements to inform healthcare professionals, patients, their families, carers and patient groups of the best practice of care. It is anticipated the implementation of these recommendations will lead to improvement in the care of patients with FD/MAS internationally.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты